**Orbital Cisticercosis. A Challenger for Neurologists, Ophthalmologists, Neuro-Opthalmologists, and General Practitioners**

Humberto Foyaca Sibat, María Carolina Salazar Campos and Lourdes de Fátima Ibañez Valdés

Additional information is available at the end of the chapter

http://dx.doi.org/10.5772/52515

## **1. Introduction**

112 Novel Aspects on Cysticercosis and Neurocysticercosis

2006;74(5):850-5.

expressed in heat inactivated M13 filamentous phage proves effective against naturally

[121] Assana E, Kyngdon CT, Gauci CG, Geerts S, Dorny P, De Deken R, Anderson GA, Zoli AP, Lightowlers MW. Elimination of *Taenia solium* transmission to pigs in a field trial of

[122] Garcia HH, Gonzalez AE, Gilman RH, Moulton LH, Verastegui M, Rodriguez S, Gavidia C, Tsang VC; Cysticercosis Working Group in Peru. Combined human and porcine mass chemotherapy for the control of *Taenia solium*. Am J Trop Med Hyg.

[123] Garcia HH, Gonzalez AE, Rodriguez S, Gonzalvez G, Llanos-Zavalaga F, Tsang VC, Gilman RH; Grupo de Trabajo en Cisticercosis en Perú. Epidemiology and control of

[124] Prichard RK, Basáñez MG, Boatin BA, McCarthy JS, García HH, Yang GJ, Sripa B, Lustigman S. A research agenda for helminth diseases of humans: intervention for

[125] Mexican Health Secretary. Modificación a la Norma Oficial Mexicana NOM-021-SSA2- 1994, para la prevención y control del complejo taeniosis/cisticercosis en el primer nivel

http://www.salud.gob.mx/unidades/cdi/nom/m021ssa294.html. (accessed 12 June 2012) [126] Flisser A, Correa D. Neurocysticercosis may no longer be a public health problem in

[127] de Aluja A, Morales Soto J, Sciutto E. A Programme to Control Taeniosis-Cysticercosis (*Taenia solium*) in Mexico, Current Topics in Tropical Medicine, Dr. Alfonso Rodriguez-Morales (Ed.), ISBN: 978-953-51-0274-8, InTech, 2012. Available from: http://www.intechopen.com/books/current-topics-in-tropical-medicine/a-program-to-

[128] Willingham AL 3rd, Harrison LJ, Fèvre EM, Parkhouse ME; Cysticercosis Working Group in Europe. Inaugural meeting of the Cysticercosis Working Group in Europe.

[129] Garcia HH, Gonzalez AE, Rodriguez S, Gonzalvez G, Llanos-Zavalaga F, Tsang VC, Gilman RH; Grupo de Trabajo en Cisticercosis en Perú. Epidemiology and control of

cysticercosis in Peru. Rev Peru Med Exp Salud Publica. 2010;27(4):592-7.

cysticercosis in Peru. Rev Peru Med Exp Salud Publica. 2010;27(4):592-7.

control and elimination. PLoS Negl Trop Dis. 2012;6(4):e1549.

de atención médica. Diario Oficial de la Federación, 2004.

control-taeniosis-cysticercosis-taenia-solium-in-mexico

Mexico. PLoS Negl Trop Dis 2010;4: e831

Emerg Infect Dis. 2008;14(12):e2.

acquired *Taenia solium* porcine cysticercosis. Vaccine. 2008;26(23):2899-905.

the TSOL18 vaccine in Cameroon. Int J Parasitol. 2010;40(5):515-9.

Cysticercosis is a disease closely related to poverty in general and in particular with a poor personal hygiene and food, socio-cultural factors, environmental, education for health in the community, and also very closely related to the hygienic and sanitary conditions of each region. This is a preventable and an eradicable disease [1] that currently affects more than 50 million people in the entire world, of which 400 thousand live in Latin America [2] is internationally accepted that the onset of epilepsy, intracranial hypertension or headache of unknown cause in a person originating, visitor or in contact with another person from an area where *T solium* is endemic suggests considering the diagnosis of NCC. The patient affected by the NCC can remain asymptomatic for several months or years, and its diagnosis can be confirmed by accident when a CT scan is carried out in search of another diagnosis. [3,4] According to WHO figures confirmed by Roman and collaborators [5] around 50 thousand people die each year as a result of the NCC and the epilepsy secondary to the NCC (ESNCC).

*Taenia solium* produces two different diseases: taeniasis (Te) and/or cysticercosis (CC). When the parasite is an adult expels its mature proglotides and eggs with the stool to the environment; the subsequent ingestion of these eggs of pork cause porcine cysticercosis (PCC). When a person swallows raw pork meat or not well cooked and infected with cysticerci (CT) then develops a new adult parasite. The human then becomes an accidental intermediate host. These oncosphere (primary larvae) penetrate the intestinal mucosa and enter the circulatory system. The Hematogenous spread of neural, muscular, and ocular tissues occurs. Within these tissues, the oncosphere develop into secondary larvae (i.e., the cysticerci).

© 2013 Foyaca Sibat et al., licensee InTech. This is an open access chapter distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. © 2013 Foyaca Sibat et al., licensee InTech. This is a paper distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

When man eats the eggs of *T. solium* acquires CC which can be found in any tissue including heart, liver, lungs, pancreas, spleen, kidneys, and peritoneal cavity among others. (See Chapter 3) [6-32] As has been mentioned, the location of Ct (larval stage) in the CNS and the eye (considering the retina as an extension of the CNS) is called NCC and is considered the neurological disease more important of parasitic origin in man and the main cause of epilepsy late onset. [33] In the brain NCC is located mainly at the level of gray substance cortical or at the level of the union between the gray and white matter because it is the best blood supplied area. [34] The CC is considered is a serious problem for public health in several developing countries where social conditions, economic and cultural levels favor the maintenance of this zoonotic disease and it is seen as a growing community problem in those developed countries with a high rate of immigrants from endemic areas. WHO includes the NCC between neglected diseases or forgotten that cause a significant impact on the economy in several regions of the world. It affects 4% of the population in endemic areas [35,36] where the personal and food hygiene habits and sometimes the religious tendencies determine the incidence and prevalence of the disease, for example: Islamism. Islam was not the first religion to prohibit the consumption of pork, before that Judaism made it perhaps less severe restrictions; however some cases have been reported in the Jewish community in New York. [37]

Orbital Cisticercosis. A Challenger for Neurologists,

Ophthalmologists, Neuro-Opthalmologists, and General Practitioners 115

Iv-Ip (6.98 % ), Sa-Ip (3.49 %) and Sa-Iv (2.32 %) reported by Amaral et al. [46] In a hospitalbased study conducted in Mexico is reported a rate of up to 8.6 cases of NCC per 100 hospitalized, and in the series of necrosis of up to 2 453 per 100 000 inhabitants and pointed out that 43.3 % of the cases were asymptomatic [35, 47] and 80% were autopsy findings. [48] Official statistics report an annual average of 500 cases of CC, with a national rate raw 0.6 per 100,000 inhabitants. [49-51] this report does not include the cases that were not attended by professional health personnel or that were not included in the study for other reasons, and does not include the extra-parenchymal presentations or even other modalities of CC. In Brazil, the prevalence in autopsies varies from 0.12 to 9% and in a series of clinical cases it varies from 0.03 to 7.5 %; in studies sero-epidemiological of 0.6 to 5.2 %. [52] In Colombia there is not much information on the CC, only some work in the departments of Antioquia and Santander that reported 1 to 2% of affectation to the population and 2% in Ecuador. [53, 54] On the other hand it has been estimated that 50 million people are infected by *T solium* in the world, of which 350 thousand live in Latin America [55] especially in those countries that do not have an adequate health infrastructure and proper health education program. [56] The main risk factors associated with the CC are the presence of peoples infected by *T solium* in the community with inadequate primary health care system, without proper health education and personal and food hygiene and without a sustainable supply of safe and clean water and where free-range pigs are able to ingest contaminated human feces [57-62] and the use of infected feces as an agricultural fertilizer. [63] The NCC has become a public

health problem in different countries in Africa, Asia and Latin America [56, 64-68]

sphenoid sinus.

The orbits are two bony cavities occupied by the eyes and associated muscles, nerves, blood vessels, fat, and much of the lacrimal apparatus. Each orbit is shaped like a pear or a foursided pyramid, with its apex situated posteriorly and its base anteriorly. The orbit is related to its superior side to the anterior cranial fossa and usually to the frontal sinus, laterally to the temporal fossa in (anterior) and to the middle cranial fossa (posterior), on its inferior side of the maxillary sinus, and medially to the ethmoid bone and the anterior extent of the

Orbital cysticercosis is caused by the infestation of the larval form of the pork tapeworm Taenia solium in the orbital cavity affecting the eyeball (ocular cysticercosis) or the extra ocular structures found within the orbital cavity such as: extra ocular muscles, nerves, ganglions, and fatty tissue. Orbital cysticercosis may cause significant visual loss, especially

Ocular cysticercosis may be extraocular (in the sub-conjunctival or orbital tissues) or intraocular (in the vitreous, sub-retinal space, or anterior chamber). [70-72] Ocular manifestations of cysticercosis vary from asymptomatic to painful blind eye and may be associated with neurological symptoms such as headache, fits, diplopia, and restriction of

According to some authors the most common location of the Ocular CC is the sub retinal region (35 %), followed by the vitreous cysticercosis (22 %), conjunctival cysticercosis (22 %),

if the cyst is located intraocularly or is compressing the optic nerve. [70]

the ocular movements, nystagmus and papilloedema.

Due to globalization a growing number of uncontrolled migrants from endemic areas of Latin America came to the USA every day therefore a significantly increasing number of cases of NCC are gradually diagnosed in the country, especially in the southwest, including Texas and California. [4, 6-8,33, 37, 38-41] It suffices to mention that a total of 1494 patients with NCC were confirmed between 1980 and 2004 of which 66% suffered from epilepsy, 16% had an obstructive hydrocephalus and 15% headache due to intraparenchymal NCC [IpNCC] (91%), intraventricular (6 %) or subarachnoid (2 %) either because travelled to endemic areas, were of Hispanic origin or had any contact with carriers of the parasite. [42] In Latin America has been described the existence of the NCC in 18 countries with an estimated 350,000 patients infected by the complex TE/CC. In 2008, Pawlowski [43] stipulated that in the world would have 2.5 million owning approximate mind of disabled people of *Taenia solium*, at least 20 million with CC, which causes on average 50.000 deaths a year. In his recent book, Hotez [2] reported that in the United States were confirmed between one and two thousand cases of NCC each year and considering the growing Latino population could have more than 41,000 Hispanics suffer from NCC in that country. [2,43,44] Neurocysticercosis is also virtually endemic in certain countries in Europe such as Romania, Poland and even Portugal. In Spain the growing strength of the migration of individuals coming from endemic areas has conditioned an increase in the frequency of this entity. [45]

Other less frequent locations of *T solium* in the CNS include: intra ventricular (Iv), subarachnoid (Sa), medullary (Me), intraocular (Io) and intraspinal (Ip). Some authors report a list of frequencies for these locations and some combinations among of them such as: IpNCC (55.23 % ), IvNCC (15,69 %) % ), SaNCC (11.63 % ), MeNCC (3.4 % ), IoNCC (0.58 % ), IpNCC (0.09 %) and the frequency of different combinations in the following manner: Iv-Ip (6.98 % ), Sa-Ip (3.49 %) and Sa-Iv (2.32 %) reported by Amaral et al. [46] In a hospitalbased study conducted in Mexico is reported a rate of up to 8.6 cases of NCC per 100 hospitalized, and in the series of necrosis of up to 2 453 per 100 000 inhabitants and pointed out that 43.3 % of the cases were asymptomatic [35, 47] and 80% were autopsy findings. [48] Official statistics report an annual average of 500 cases of CC, with a national rate raw 0.6 per 100,000 inhabitants. [49-51] this report does not include the cases that were not attended by professional health personnel or that were not included in the study for other reasons, and does not include the extra-parenchymal presentations or even other modalities of CC. In Brazil, the prevalence in autopsies varies from 0.12 to 9% and in a series of clinical cases it varies from 0.03 to 7.5 %; in studies sero-epidemiological of 0.6 to 5.2 %. [52] In Colombia there is not much information on the CC, only some work in the departments of Antioquia and Santander that reported 1 to 2% of affectation to the population and 2% in Ecuador. [53, 54] On the other hand it has been estimated that 50 million people are infected by *T solium* in the world, of which 350 thousand live in Latin America [55] especially in those countries that do not have an adequate health infrastructure and proper health education program. [56] The main risk factors associated with the CC are the presence of peoples infected by *T solium* in the community with inadequate primary health care system, without proper health education and personal and food hygiene and without a sustainable supply of safe and clean water and where free-range pigs are able to ingest contaminated human feces [57-62] and the use of infected feces as an agricultural fertilizer. [63] The NCC has become a public health problem in different countries in Africa, Asia and Latin America [56, 64-68]

114 Novel Aspects on Cysticercosis and Neurocysticercosis

New York. [37]

entity. [45]

When man eats the eggs of *T. solium* acquires CC which can be found in any tissue including heart, liver, lungs, pancreas, spleen, kidneys, and peritoneal cavity among others. (See Chapter 3) [6-32] As has been mentioned, the location of Ct (larval stage) in the CNS and the eye (considering the retina as an extension of the CNS) is called NCC and is considered the neurological disease more important of parasitic origin in man and the main cause of epilepsy late onset. [33] In the brain NCC is located mainly at the level of gray substance cortical or at the level of the union between the gray and white matter because it is the best blood supplied area. [34] The CC is considered is a serious problem for public health in several developing countries where social conditions, economic and cultural levels favor the maintenance of this zoonotic disease and it is seen as a growing community problem in those developed countries with a high rate of immigrants from endemic areas. WHO includes the NCC between neglected diseases or forgotten that cause a significant impact on the economy in several regions of the world. It affects 4% of the population in endemic areas [35,36] where the personal and food hygiene habits and sometimes the religious tendencies determine the incidence and prevalence of the disease, for example: Islamism. Islam was not the first religion to prohibit the consumption of pork, before that Judaism made it perhaps less severe restrictions; however some cases have been reported in the Jewish community in

Due to globalization a growing number of uncontrolled migrants from endemic areas of Latin America came to the USA every day therefore a significantly increasing number of cases of NCC are gradually diagnosed in the country, especially in the southwest, including Texas and California. [4, 6-8,33, 37, 38-41] It suffices to mention that a total of 1494 patients with NCC were confirmed between 1980 and 2004 of which 66% suffered from epilepsy, 16% had an obstructive hydrocephalus and 15% headache due to intraparenchymal NCC [IpNCC] (91%), intraventricular (6 %) or subarachnoid (2 %) either because travelled to endemic areas, were of Hispanic origin or had any contact with carriers of the parasite. [42] In Latin America has been described the existence of the NCC in 18 countries with an estimated 350,000 patients infected by the complex TE/CC. In 2008, Pawlowski [43] stipulated that in the world would have 2.5 million owning approximate mind of disabled people of *Taenia solium*, at least 20 million with CC, which causes on average 50.000 deaths a year. In his recent book, Hotez [2] reported that in the United States were confirmed between one and two thousand cases of NCC each year and considering the growing Latino population could have more than 41,000 Hispanics suffer from NCC in that country. [2,43,44] Neurocysticercosis is also virtually endemic in certain countries in Europe such as Romania, Poland and even Portugal. In Spain the growing strength of the migration of individuals coming from endemic areas has conditioned an increase in the frequency of this

Other less frequent locations of *T solium* in the CNS include: intra ventricular (Iv), subarachnoid (Sa), medullary (Me), intraocular (Io) and intraspinal (Ip). Some authors report a list of frequencies for these locations and some combinations among of them such as: IpNCC (55.23 % ), IvNCC (15,69 %) % ), SaNCC (11.63 % ), MeNCC (3.4 % ), IoNCC (0.58 % ), IpNCC (0.09 %) and the frequency of different combinations in the following manner: The orbits are two bony cavities occupied by the eyes and associated muscles, nerves, blood vessels, fat, and much of the lacrimal apparatus. Each orbit is shaped like a pear or a foursided pyramid, with its apex situated posteriorly and its base anteriorly. The orbit is related to its superior side to the anterior cranial fossa and usually to the frontal sinus, laterally to the temporal fossa in (anterior) and to the middle cranial fossa (posterior), on its inferior side of the maxillary sinus, and medially to the ethmoid bone and the anterior extent of the sphenoid sinus.

Orbital cysticercosis is caused by the infestation of the larval form of the pork tapeworm Taenia solium in the orbital cavity affecting the eyeball (ocular cysticercosis) or the extra ocular structures found within the orbital cavity such as: extra ocular muscles, nerves, ganglions, and fatty tissue. Orbital cysticercosis may cause significant visual loss, especially if the cyst is located intraocularly or is compressing the optic nerve. [70]

Ocular cysticercosis may be extraocular (in the sub-conjunctival or orbital tissues) or intraocular (in the vitreous, sub-retinal space, or anterior chamber). [70-72] Ocular manifestations of cysticercosis vary from asymptomatic to painful blind eye and may be associated with neurological symptoms such as headache, fits, diplopia, and restriction of the ocular movements, nystagmus and papilloedema.

According to some authors the most common location of the Ocular CC is the sub retinal region (35 %), followed by the vitreous cysticercosis (22 %), conjunctival cysticercosis (22 %),

and anterior segment cysticercosis. (5 %) [73] While involvement of other regions (e.g., extraocular muscles, optic nerve) are relatively less common. [70]

Orbital Cisticercosis. A Challenger for Neurologists,

Ophthalmologists, Neuro-Opthalmologists, and General Practitioners 117

Most of patient reported at that time were from non-endemic areas for cysticercosis, this information was very important in the diagnostic process but at the present moment cysticercosis can be found in almost all countries. The next report came from India. It was a 69-year-old male who presented with pulsatile proptosis owing to metastasis of an asymptomatic renal cell carcinoma [121] this case was an example of the great challenger. In 2007, another three patients: two males of 67 and 58 years old males and a 23-years-oldfemale from India presenting iris mass, orbital metastasis were reported. [122] At this time, a total of 71 patients was reported in the international medical literature. This report suggested that patients presenting with atypical orbital or ocular masses, the possibility of renal cell carcinoma metastasis should be considered, especially if there is a history of previous renal disorder. Incisional biopsy with histopathological evaluation may be an important means to diagnose this condition and facilitate appropriate therapy. Other author reported similar findings. [123-126] Based on this review of the medical literature we can conclude that differential diagnosis between orbital metastasis and orbital cysticercosis it is not difficult considering that only three patients presenting proptosis of the eyes as the first clinical manifestation of RCC have been reported to the medical

Patients with hepatic cell carcinoma presenting primary symptoms of metastatic disease are rare and the retro-orbital mass as the initial manifestation of disease is also very uncommon as well. [127] Nevertheless, this an uncommon pathological disorder can also affect intracranial structures mainly in elderly patients or presenting as rapid onset of proptosis and visual loss (only three patients reported), or painful proptosis as the most common clinical sign of hepatocellular carcinoma metastatic to the orbit (ten patients reported). [128- 132] In conclusion, hepatocellular carcinoma is a rare source of metastasis to the orbit, and only 14 histopathologically proven cases have been reported in the international medical literature. Proptosis is the most common presenting feature in the reviewed reports and a majority of patients have occult primary tumors at presentation and the diagnosis of the

The age of the patients, clinical features of the orbital lesion, presence of other metastatic lesions, epidemiological features of cysticercosis, CT scans, and ultrasonographic studies are

In the previous chapter we reviewed some aspects on orbital cysticercosis according to their different locations within and outside of the eyeball without exceeding the limits of orbit, mainly those clinical features of the ocular cysticercosis associated with damage of other

It may be asymptomatic in the early stages. As the parasite grows, it causes a painless,

literature. [125]

orbital mass is based on histopathology. [131]

**2.1. Intraocular cysticercosis** 

elements that allow you to make a certain diagnosis.

progressive loss of vision related to the area of location.

organs as part of the disseminated cysticercosis at the same time.

**2. Vitreous cysticercosis and anterior chamber cysticercosis** 

Sub retinal cysticercosis (SrCC), vitreous cysticercosis (VCC) and anterior segment cysticercosis (AsCC) are more common in India. While either eye may be affected, bilateral involvement is rare [74] and multiple cysts may develop in the same eye. [75] Ocular manifestations may be devastating as the cysticercus enlarges. The cysticercus may lead to blindness in 3-5 years. [76] Decreased vision, pain, and recurrent redness of the involved eye are common symptoms of intraocular cysticercosis. [70]

Some authors consider that intraocular cysticercosis is predominant in the Western countries, whereas extraocular is more common in the Indian population attributed to geographic and environmental factors. [77, 79] However, other affirm that intraocular involvement is more common in India compared with Western countries. [78,] and involvement of conjunctiva is most commonly reported in India [80-99], whereas involvement of posterior segment of the eye is most commonly seen in Western countries. [100-109] compared with conjunctival region but it also can be seen in India. [110-112]

Orbital cysticercosis should be suspected in patients who have lived in an endemic area and who develop uveitis, leucocoria or Neuro-Ophthalmological signs, also in the presence of sub conjunctival cysts or lids nodules. Cysts deep within the orbit are best treated conservatively with a 4-week regimen of oral albendazole (15 mg/kg/d) in conjunction with oral steroids (1.5 mg/kg/d) in a tapering dose over a 1-month period. Treatment may increase inflammation as the cyst involutes, leading to worsening clinical status. Thus, concomitant administration of corticosteroids is recommended to avert an inflammatory response. [113,114]

In 2010, Rath et al., Studied 93 patients presenting orbital cysticercosis with mean age of 13 years and they concluded that orbital cysticercosis is a common clinical condition in the developing world which typically affects young individuals and has a wide spectrum of clinical manifestations; B-scan ultrasonography and computed tomography scan are useful in confirming the diagnosis and despite resolution of cysticercosis with medical management, a significant proportion of patients may have residual functional deficits. [77, 115,116]

## **1.1. Is orbital metastasis another challenger for differential diagnosis?**

It may be, therefore our readership should remember that renal cell carcinoma (RCC) is a tumor of middle-aged men that metastasizes to the lung, liver, and bone. When this tumor spreads to the orbit, the orbital metastasis is likely to be the first indication of the cancer and up to 1993 only a few cases were reported. [117] In 1994, Schmidt et al [118] reported a 69 years-old-male patient with similar condition. Konya et al [119] reported two cases: a 44 and 59-years-old- male patients with proptosis and a tongue tumor due to RCC. Another patient was: 39-year-old black man with sickle cell trait, presented with a rapidly progressive painful proptosis of the left eye due to metastasis from renal medullary carcinoma. [120] Most of patient reported at that time were from non-endemic areas for cysticercosis, this information was very important in the diagnostic process but at the present moment cysticercosis can be found in almost all countries. The next report came from India. It was a 69-year-old male who presented with pulsatile proptosis owing to metastasis of an asymptomatic renal cell carcinoma [121] this case was an example of the great challenger. In 2007, another three patients: two males of 67 and 58 years old males and a 23-years-oldfemale from India presenting iris mass, orbital metastasis were reported. [122] At this time, a total of 71 patients was reported in the international medical literature. This report suggested that patients presenting with atypical orbital or ocular masses, the possibility of renal cell carcinoma metastasis should be considered, especially if there is a history of previous renal disorder. Incisional biopsy with histopathological evaluation may be an important means to diagnose this condition and facilitate appropriate therapy. Other author reported similar findings. [123-126] Based on this review of the medical literature we can conclude that differential diagnosis between orbital metastasis and orbital cysticercosis it is not difficult considering that only three patients presenting proptosis of the eyes as the first clinical manifestation of RCC have been reported to the medical literature. [125]

Patients with hepatic cell carcinoma presenting primary symptoms of metastatic disease are rare and the retro-orbital mass as the initial manifestation of disease is also very uncommon as well. [127] Nevertheless, this an uncommon pathological disorder can also affect intracranial structures mainly in elderly patients or presenting as rapid onset of proptosis and visual loss (only three patients reported), or painful proptosis as the most common clinical sign of hepatocellular carcinoma metastatic to the orbit (ten patients reported). [128- 132] In conclusion, hepatocellular carcinoma is a rare source of metastasis to the orbit, and only 14 histopathologically proven cases have been reported in the international medical literature. Proptosis is the most common presenting feature in the reviewed reports and a majority of patients have occult primary tumors at presentation and the diagnosis of the orbital mass is based on histopathology. [131]

The age of the patients, clinical features of the orbital lesion, presence of other metastatic lesions, epidemiological features of cysticercosis, CT scans, and ultrasonographic studies are elements that allow you to make a certain diagnosis.

In the previous chapter we reviewed some aspects on orbital cysticercosis according to their different locations within and outside of the eyeball without exceeding the limits of orbit, mainly those clinical features of the ocular cysticercosis associated with damage of other organs as part of the disseminated cysticercosis at the same time.

## **2. Vitreous cysticercosis and anterior chamber cysticercosis**

## **2.1. Intraocular cysticercosis**

116 Novel Aspects on Cysticercosis and Neurocysticercosis

response. [113,114]

115,116]

and anterior segment cysticercosis. (5 %) [73] While involvement of other regions (e.g.,

Sub retinal cysticercosis (SrCC), vitreous cysticercosis (VCC) and anterior segment cysticercosis (AsCC) are more common in India. While either eye may be affected, bilateral involvement is rare [74] and multiple cysts may develop in the same eye. [75] Ocular manifestations may be devastating as the cysticercus enlarges. The cysticercus may lead to blindness in 3-5 years. [76] Decreased vision, pain, and recurrent redness of the involved eye

Some authors consider that intraocular cysticercosis is predominant in the Western countries, whereas extraocular is more common in the Indian population attributed to geographic and environmental factors. [77, 79] However, other affirm that intraocular involvement is more common in India compared with Western countries. [78,] and involvement of conjunctiva is most commonly reported in India [80-99], whereas involvement of posterior segment of the eye is most commonly seen in Western countries.

[100-109] compared with conjunctival region but it also can be seen in India. [110-112]

Orbital cysticercosis should be suspected in patients who have lived in an endemic area and who develop uveitis, leucocoria or Neuro-Ophthalmological signs, also in the presence of sub conjunctival cysts or lids nodules. Cysts deep within the orbit are best treated conservatively with a 4-week regimen of oral albendazole (15 mg/kg/d) in conjunction with oral steroids (1.5 mg/kg/d) in a tapering dose over a 1-month period. Treatment may increase inflammation as the cyst involutes, leading to worsening clinical status. Thus, concomitant administration of corticosteroids is recommended to avert an inflammatory

In 2010, Rath et al., Studied 93 patients presenting orbital cysticercosis with mean age of 13 years and they concluded that orbital cysticercosis is a common clinical condition in the developing world which typically affects young individuals and has a wide spectrum of clinical manifestations; B-scan ultrasonography and computed tomography scan are useful in confirming the diagnosis and despite resolution of cysticercosis with medical management, a significant proportion of patients may have residual functional deficits. [77,

It may be, therefore our readership should remember that renal cell carcinoma (RCC) is a tumor of middle-aged men that metastasizes to the lung, liver, and bone. When this tumor spreads to the orbit, the orbital metastasis is likely to be the first indication of the cancer and up to 1993 only a few cases were reported. [117] In 1994, Schmidt et al [118] reported a 69 years-old-male patient with similar condition. Konya et al [119] reported two cases: a 44 and 59-years-old- male patients with proptosis and a tongue tumor due to RCC. Another patient was: 39-year-old black man with sickle cell trait, presented with a rapidly progressive painful proptosis of the left eye due to metastasis from renal medullary carcinoma. [120]

**1.1. Is orbital metastasis another challenger for differential diagnosis?** 

extraocular muscles, optic nerve) are relatively less common. [70]

are common symptoms of intraocular cysticercosis. [70]

It may be asymptomatic in the early stages. As the parasite grows, it causes a painless, progressive loss of vision related to the area of location.

Patients` complaints about an intermittent roundish, dark mobile mass (intravitreal location) or may experience visual field defects (sub retinal or CNS location).

Orbital Cisticercosis. A Challenger for Neurologists,

Ophthalmologists, Neuro-Opthalmologists, and General Practitioners 119

products released from the cyst, and the patient presents a blind painful eye. [136,142.143]. Only a few cases about cysticercosis resulting in glaucoma due to pupillary block have been

It can be recognized through clear media, the translucent cyst with a dense white spot formed by the invaginated scolex and the undulating movements are typical. We can see the scolex returning rapidly to the cyst when exposed to the ophthalmoscope or slit lamp light. [144,145] The frequency of different location of intraocular cysticercosis can be seen in the

**Figure 2.** Frequency of intraocular cysticercosis in Western countries and India

After migration into the vitreous, a chorioretinal scar may develop in this area.

Diagnostic paracentesis of the anterior chamber cysticercosis can show more than 50% of leukocytes in the aqueous humor with eosinophils . ELISA IgG serology test for cysticercosis and Western blot contribute to confirm the diagnosis although some cross reaction can be found in patients presenting Diphyllobothriosis, Taeniasis, Cysticercosis, Echinococcosis

Are of limited value in diagnosing intraocular cysticercosis. FBC, serum chemistries and

reported.[72]

figure below.

*Laboratory tests:* 

*Stool samples:* 

*Blood tests:* 

(Hydatidosis), Coenuriosis or Sparganosis.

May not show any proglottids or eggs of T. solium.

ESR may all be normal; eosinophilia is uncommon.

**2.2. Intravitreal cysticercosis** 

Intraocular lesions caused by cysticercosis most commonly occur in the vitreous or subretinal space, but subchoroidal, sub hyaliod, and into the anterior chamber, also occur. [133-138] Lens involvement also can occur but it is extremely rare. (139)

A cyst in the anterior chamber can be manifested as an acute anterior uveitis, sometimes is so severe that is difficult to differentiate a cyst from a dislocated lens and always there is a cataract formation. The parasite can be seen moving freely into the cavity. (See figure 1)

When the parasite is freely mobile it may contribute to the absence of reaction in the anterior chamber. The portal of entry of the parasite into the anterior chamber has not been well documented although some author refers that the cyst may enter the anterior chamber either from the posterior ciliary arteries or from the angle. [154].

**Figure 1.** Anterior chamber cysticercosis with freely mobile cysticercus into the aqueous humor.

In the posterior segment, parasites gain access presumably through the posterior ciliary arteries. From this location, they usually pass through a rent in the retina into the vitreous. In rare cases the parasite may pass from the vitreous, through the pupil, into the anterior chamber. Infestation of the ocular adnexa is probably through the anterior ciliary arteries. [101-134,140]

The intraocular location of the cyst can cause retinal detachment, macular scarring, retinal vasculitis and vitritis [141] and usually affects young patients unilaterally. The cyst may be in either the vitreous cavity or the sub retinal space, and visual acuity at presentation is often poor. [142]

Most patients presenting intraocular cysticercosis can tolerate it well while the cysticercus is alive, however when the parasite dies, there is a marked inflammatory response to toxic products released from the cyst, and the patient presents a blind painful eye. [136,142.143]. Only a few cases about cysticercosis resulting in glaucoma due to pupillary block have been reported.[72]

## **2.2. Intravitreal cysticercosis**

118 Novel Aspects on Cysticercosis and Neurocysticercosis

Patients` complaints about an intermittent roundish, dark mobile mass (intravitreal location)

Intraocular lesions caused by cysticercosis most commonly occur in the vitreous or subretinal space, but subchoroidal, sub hyaliod, and into the anterior chamber, also occur.

A cyst in the anterior chamber can be manifested as an acute anterior uveitis, sometimes is so severe that is difficult to differentiate a cyst from a dislocated lens and always there is a cataract formation. The parasite can be seen moving freely into the cavity. (See figure 1)

When the parasite is freely mobile it may contribute to the absence of reaction in the anterior chamber. The portal of entry of the parasite into the anterior chamber has not been well documented although some author refers that the cyst may enter the anterior chamber either

**Figure 1.** Anterior chamber cysticercosis with freely mobile cysticercus into the aqueous humor.

In the posterior segment, parasites gain access presumably through the posterior ciliary arteries. From this location, they usually pass through a rent in the retina into the vitreous. In rare cases the parasite may pass from the vitreous, through the pupil, into the anterior chamber. Infestation of the ocular adnexa is probably through the anterior ciliary arteries.

The intraocular location of the cyst can cause retinal detachment, macular scarring, retinal vasculitis and vitritis [141] and usually affects young patients unilaterally. The cyst may be in either the vitreous cavity or the sub retinal space, and visual acuity at presentation is

Most patients presenting intraocular cysticercosis can tolerate it well while the cysticercus is alive, however when the parasite dies, there is a marked inflammatory response to toxic

or may experience visual field defects (sub retinal or CNS location).

[133-138] Lens involvement also can occur but it is extremely rare. (139)

from the posterior ciliary arteries or from the angle. [154].

[101-134,140]

often poor. [142]

It can be recognized through clear media, the translucent cyst with a dense white spot formed by the invaginated scolex and the undulating movements are typical. We can see the scolex returning rapidly to the cyst when exposed to the ophthalmoscope or slit lamp light. [144,145] The frequency of different location of intraocular cysticercosis can be seen in the figure below.

**Figure 2.** Frequency of intraocular cysticercosis in Western countries and India

After migration into the vitreous, a chorioretinal scar may develop in this area.

#### *Laboratory tests:*

Diagnostic paracentesis of the anterior chamber cysticercosis can show more than 50% of leukocytes in the aqueous humor with eosinophils . ELISA IgG serology test for cysticercosis and Western blot contribute to confirm the diagnosis although some cross reaction can be found in patients presenting Diphyllobothriosis, Taeniasis, Cysticercosis, Echinococcosis (Hydatidosis), Coenuriosis or Sparganosis.

#### *Stool samples:*

May not show any proglottids or eggs of T. solium.

#### *Blood tests:*

Are of limited value in diagnosing intraocular cysticercosis. FBC, serum chemistries and ESR may all be normal; eosinophilia is uncommon.

*EITB* (enzyme-linked immuno-electro transfer blot assay) has been shown to have greater than 98% sensitivity and specificity in patients with systemic disease, and can be completed with the determination of circulating antibody and antigen in the aqueous humor.

Orbital Cisticercosis. A Challenger for Neurologists,

Ophthalmologists, Neuro-Opthalmologists, and General Practitioners 121

general these cysts are around 2x2 mm in size, grayish-white in color and are freely mobile (See figure 2), changing its shape by protruding and retracting its scolex from and into the cyst cavity; sometimes the parasite is not neither attached to the iris, cornea or other intraocular anatomical structure and usually there are not an associated conjunctival or ciliary congestion and not abnormalities of the retina, macula vitreous or optic disc with preservation of the visual acuity but it can be associated with iridocyclitis [150-152] and secondary glaucoma [153] and as a rule the parasite is attached to the iris and only

Some author reported a frequency of ocular involvement in cysticercosis around 13 to 46% [155,156] and its associated intraocular inflammation may also cause cataract formation [154] and iris atrophy. We found some differences and similarities compared with South African's

Most of studies done in India and Western countries confirmed that orbital cysticercosis with ocular involvement is an uncommon pathological process that may cause severe damage of the eyes and an important involvement of the visual acuity. [80-84, 93, 142, 150, 156-158] The pathogenesis of ocular injury due to an intense inflammatory reaction secondary to intravitreous cysticercosis has been documented throughout an experimental animal model for intravitreous cysticercosis using New Zealand rabbits and Taenia

In 1964, Proctor [160] estimated a 1% incidence in South Africa of infestation by tapeworm cysts in 7 597 autopsies. Pammenter and Rossouw in 1984, [161] reported the results of serological tests in 79 patients with systemic cysticercosis in South Africa, from this study they confirmed that in isolated ocular cysticercosis serological tests are usually negative:

occasionally to the corneal endothelium or lens capsule.

**Figure 3.** Intravitreous cysticercus moving freely

**2.3. Ocular cysticercosis in South Africa** 

patients that we will comment below.

crassiceps cysticerci since 1996. [159]

In ocular cysticercosis, serologic tests are helpful if positive, but false-negative tests may be reported, and their negativity does not rule out the disease. [143,146]

#### *Imagenology*

Fluorescein Angiography is useful in delineating the sub-retinal cyst located in the periphery of the retina. Plain X-rays of soft tissue or skull often reveal calcified cysticerci. The enhanced CT scan is valuable for identifying and following the evolution of orbital cysticercosis, which may appear as solid, cystic or calcified nodules.

MRI provides detailed images of living and degenerating cysticerci. [141]

Ultrasonography is an effective and economical alternative to MRI and CT for the detection of the intra-ocular cysticerci. It is a real time, dynamic examination, also allows direct visualization of the movements of the parasite when it is intraocular. U/S is effective for the detection of the cysts in the orbit and muscles too.

A cysticercus cyst with the scolex attached to the inner wall composes the "hanging drop sign." This sign was seen in cases studied using simultaneous A-and B-scan ultrasonography. This appearance was consistent and reproducible, regardless of whether the cyst was intraocular or extraocular in location. Serial B-scan ocular ultrasonography or CT scans of the orbit helps to follow the resolution of the cyst, which is recognized by the disappearance of the scolex. [15]

Optical Coherence Tomography (OCT) exam is seen as hyporeflective area due to the presence of fluid in the cyst cavity. Sometimes the height of the cyst obscured the visualization of the scolex. In children, intraocular cysticercosis can simulate retinoblastoma mainly in the inflammatory response.

#### *Therapeutic aspects*

Medical treatment for intraocular cysticercosis is not advisable while that Albendazole or Praziquantel (PZQ), in conjunction with corticosteroids can be used for extraocular presentations with very good results.

Surgical treatment is used to remove the cysts from the adnexa, anterior and posterior segment. Removal of the cyst is mandatory to remove the source of the toxins causing inflammation and early removal has been advocated by many authors. [144,147] Reviews of treated cases suggest that early removal of the organism is associated with preservation of visual function. [137] If possible, ocular cysts should be removed before systemic treatment is undertaken to prevent damage from the dying process of the parasite. Freely mobile live cysticercus in the anterior chamber associated to NCC in patients complete asymptomatic has been reported in the medical literature. [148] or may be attached by inflammatory membranes to surrounding structures, such as the cornea, lens capsule, or iris. [149] In general these cysts are around 2x2 mm in size, grayish-white in color and are freely mobile (See figure 2), changing its shape by protruding and retracting its scolex from and into the cyst cavity; sometimes the parasite is not neither attached to the iris, cornea or other intraocular anatomical structure and usually there are not an associated conjunctival or ciliary congestion and not abnormalities of the retina, macula vitreous or optic disc with preservation of the visual acuity but it can be associated with iridocyclitis [150-152] and secondary glaucoma [153] and as a rule the parasite is attached to the iris and only occasionally to the corneal endothelium or lens capsule.

**Figure 3.** Intravitreous cysticercus moving freely

120 Novel Aspects on Cysticercosis and Neurocysticercosis

*Imagenology* 

*EITB* (enzyme-linked immuno-electro transfer blot assay) has been shown to have greater than 98% sensitivity and specificity in patients with systemic disease, and can be completed

In ocular cysticercosis, serologic tests are helpful if positive, but false-negative tests may be

Fluorescein Angiography is useful in delineating the sub-retinal cyst located in the periphery of the retina. Plain X-rays of soft tissue or skull often reveal calcified cysticerci. The enhanced CT scan is valuable for identifying and following the evolution of orbital

Ultrasonography is an effective and economical alternative to MRI and CT for the detection of the intra-ocular cysticerci. It is a real time, dynamic examination, also allows direct visualization of the movements of the parasite when it is intraocular. U/S is effective for the

A cysticercus cyst with the scolex attached to the inner wall composes the "hanging drop sign." This sign was seen in cases studied using simultaneous A-and B-scan ultrasonography. This appearance was consistent and reproducible, regardless of whether the cyst was intraocular or extraocular in location. Serial B-scan ocular ultrasonography or CT scans of the orbit helps to follow the resolution of the cyst, which is recognized by the

Optical Coherence Tomography (OCT) exam is seen as hyporeflective area due to the presence of fluid in the cyst cavity. Sometimes the height of the cyst obscured the visualization of the scolex. In children, intraocular cysticercosis can simulate retinoblastoma

Medical treatment for intraocular cysticercosis is not advisable while that Albendazole or Praziquantel (PZQ), in conjunction with corticosteroids can be used for extraocular

Surgical treatment is used to remove the cysts from the adnexa, anterior and posterior segment. Removal of the cyst is mandatory to remove the source of the toxins causing inflammation and early removal has been advocated by many authors. [144,147] Reviews of treated cases suggest that early removal of the organism is associated with preservation of visual function. [137] If possible, ocular cysts should be removed before systemic treatment is undertaken to prevent damage from the dying process of the parasite. Freely mobile live cysticercus in the anterior chamber associated to NCC in patients complete asymptomatic has been reported in the medical literature. [148] or may be attached by inflammatory membranes to surrounding structures, such as the cornea, lens capsule, or iris. [149] In

with the determination of circulating antibody and antigen in the aqueous humor.

reported, and their negativity does not rule out the disease. [143,146]

cysticercosis, which may appear as solid, cystic or calcified nodules.

detection of the cysts in the orbit and muscles too.

disappearance of the scolex. [15]

mainly in the inflammatory response.

presentations with very good results.

*Therapeutic aspects* 

MRI provides detailed images of living and degenerating cysticerci. [141]

## **2.3. Ocular cysticercosis in South Africa**

Some author reported a frequency of ocular involvement in cysticercosis around 13 to 46% [155,156] and its associated intraocular inflammation may also cause cataract formation [154] and iris atrophy. We found some differences and similarities compared with South African's patients that we will comment below.

Most of studies done in India and Western countries confirmed that orbital cysticercosis with ocular involvement is an uncommon pathological process that may cause severe damage of the eyes and an important involvement of the visual acuity. [80-84, 93, 142, 150, 156-158] The pathogenesis of ocular injury due to an intense inflammatory reaction secondary to intravitreous cysticercosis has been documented throughout an experimental animal model for intravitreous cysticercosis using New Zealand rabbits and Taenia crassiceps cysticerci since 1996. [159]

In 1964, Proctor [160] estimated a 1% incidence in South Africa of infestation by tapeworm cysts in 7 597 autopsies. Pammenter and Rossouw in 1984, [161] reported the results of serological tests in 79 patients with systemic cysticercosis in South Africa, from this study they confirmed that in isolated ocular cysticercosis serological tests are usually negative:

45,5% ELISA positive, the indirect haemagglutination test 33,3% positive, and the fluorescent antibody test 48,9% positive. Almost always these tests were positive in cases of systemic cysticercosis in our region. This experience was confirmed also by other. [162, 164].

Orbital Cisticercosis. A Challenger for Neurologists,

Ophthalmologists, Neuro-Opthalmologists, and General Practitioners 123

In individuals with uveitis, the perioperative corticosteroid administration is recommended. Although surgical excision of orbital cysts was considered the ideal treatment modality, deep orbital dissection and difficulty in completely excising the cyst because of the surrounding inflammatory response, increase the chances of postoperative complications

Destruction of the larvae in situ of photocoagulation [170] cryotherapy, and diathermy has been attempted with some success. However, as intraocular cysticercosis may lead to severe inflammation following larval death, early surgical removal of the cyst is the treatment of

In years the 70s and 80s systemic cysticercosis was very common in rural areas of South Africa but ocular cysticercosis was not associated with any other systemic features and CT suggested that some patients with ocular cysticercosis had neurocysticercosis as well, although none was reported with accompanying systemic physical signs or symptoms. All patients had severe reactions to the cyst, whereas in the majority of reported cases there was

The treatment of anterior chamber cysticercosis is essentially surgical. The different modalities used to remove the cyst include paracentesis, extraction with capsule forceps, cryo-extraction, erysiphake extraction, and viscoexpression. [15,78] Viscoexpression allows removal of an intact cyst through a small limbal incision. In the treatment of intraocular cysticercosis, the most common surgical approach is removal through the pars plana route following vitrectomy. [168] Sub retinal cyst anterior to the equator may be removed transsclerally, whereas sub retinal cysts posterior to the equator and intravitreal cysts are best removed trans-vitreally. [137] Ideally, the cyst should be removed in toto. Complete surgical removal of the intact cyst results in good functional recovery in eyes with intraocular

The macular region is the site of choice for the subretinal cysticercus to lodge, maybe because of the high vascularization of this area but it can be seen in another subretinal area as well. In early stages, subretinal cysticercosis can appear as an acute central retinitis with retinal edema and subretinal exudates. The subretinal organism eventually develops into a cyst and the parasite and its movements can be recognized through the thin muscular layers. The observation of the cyst is more difficult when it is located in the periphery of the retina because scleral depression has to be used and the movements of the parasite cannot

Reportedly 35% of the cysts are in the subretinal space, 22% in the vitreous, 22% in the subconjunctival space, 5% in the anterior segment and 1% in the orbit. Among the

intraocular cysts, 60% are found intravitreally and 40% are subretinal. [137,153]

**3. Subretinal cysticercosis and optic nerve neurocysticercosis** 

(e.g., decreased vision, diplopia). [110, 169]

choice. [137]

a little ocular reaction. [107]

cysticercosis. [110, 136,137, 167]

**3.1. Subretinal cysticercosis** 

be observed clearly. (See figure 4 and 5)

In 1987, Welsh et al [106] studied thirteen cases of ocular cysticercosis at King Edward VIII Hospital over an 8-year period from 1976 to 1984. All patients came from rural areas, a reflection on the low standards of hygiene. At that time, adolescent females ingested tapeworm eggs for slimming purposes, but none of this report was under that category. From their review of the medical literature they found that up to 1952, 1 216 cases of ocular cysticercosis were reported and when studied their patients most of them were occasional and came to South Africa from India. Of the 13 patients with ocular cysts nine were found in the vitreous, three in the anterior chamber and one under the conjunctiva of the eye. The majority of patients were female and all were Zulus living in areas of KwaZulu Natal, most of them complained of loss of vision for an average of 3 months. The only exception was one patient with the sub-conjunctival cyst who had waited 2 years before seeking treatment, since the lump had not affected his vision. Today all types of cysticercosis in KwaZulu Natal practically disappeared and patients with cysticercosis can be seen only at the former Transkei or another province but emigrated from this region (currently region C and D) of the Eastern Cape Province which does not mean that cysticercosis is going to disappear in the next decade how we explained in the previous chapter.

All patients from Welsh' series [107] presenting vitreous cysticercosis complained of severe loss of vision, and there was always a marked vitreoretinal reaction and the patients with cysts in the anterior chamber showed severe anterior chamber reactions with anterior uveitis. The vision was reduced in all patients to where hand movements could not be seen or to perception of light being absent or to complete blindness, except for the patient with the subconjunctival cyst . In this series of patients because of the severity of the lesions, surgical removal, although partly successful, did not improve vision and the distribution of the cysts in the various structures of the eye was similar to cases reported in the literature. We would like to highlight that around 70's and 80's years several cases presenting bilateral involvement of the eyes and/or multifocal intra-ocular cysts were most commonly seen. [74,104,107,164,165]

Medical treatment for intraocular cysticercosis is not recommended based on South African's experiences with praziquantel (isquinoline pyrazine) [106] and another report from the same period of time. [166] Surgical removal is strongly recommended as the best treatment because complications associated with the cyst were minimal. Removal of vitreous cysts by pars plana vitrectomy is the method of choice. [110,167]

Removal of a cyst from the anterior chamber is relatively simple excepting cases pre-existing vitreoretinal-uveal reaction. [110, 168] The treatment of anterior chamber cysticercosis is essentially surgical. The different modalities used to remove the cyst include paracentesis, extraction with capsule forceps, cryo-extraction, diathermia, and viscoexpression. [78, 110,136, 168, 169]

In individuals with uveitis, the perioperative corticosteroid administration is recommended. Although surgical excision of orbital cysts was considered the ideal treatment modality, deep orbital dissection and difficulty in completely excising the cyst because of the surrounding inflammatory response, increase the chances of postoperative complications (e.g., decreased vision, diplopia). [110, 169]

Destruction of the larvae in situ of photocoagulation [170] cryotherapy, and diathermy has been attempted with some success. However, as intraocular cysticercosis may lead to severe inflammation following larval death, early surgical removal of the cyst is the treatment of choice. [137]

In years the 70s and 80s systemic cysticercosis was very common in rural areas of South Africa but ocular cysticercosis was not associated with any other systemic features and CT suggested that some patients with ocular cysticercosis had neurocysticercosis as well, although none was reported with accompanying systemic physical signs or symptoms. All patients had severe reactions to the cyst, whereas in the majority of reported cases there was a little ocular reaction. [107]

The treatment of anterior chamber cysticercosis is essentially surgical. The different modalities used to remove the cyst include paracentesis, extraction with capsule forceps, cryo-extraction, erysiphake extraction, and viscoexpression. [15,78] Viscoexpression allows removal of an intact cyst through a small limbal incision. In the treatment of intraocular cysticercosis, the most common surgical approach is removal through the pars plana route following vitrectomy. [168] Sub retinal cyst anterior to the equator may be removed transsclerally, whereas sub retinal cysts posterior to the equator and intravitreal cysts are best removed trans-vitreally. [137] Ideally, the cyst should be removed in toto. Complete surgical removal of the intact cyst results in good functional recovery in eyes with intraocular cysticercosis. [110, 136,137, 167]

## **3. Subretinal cysticercosis and optic nerve neurocysticercosis**

## **3.1. Subretinal cysticercosis**

122 Novel Aspects on Cysticercosis and Neurocysticercosis

the next decade how we explained in the previous chapter.

[74,104,107,164,165]

110,136, 168, 169]

45,5% ELISA positive, the indirect haemagglutination test 33,3% positive, and the fluorescent antibody test 48,9% positive. Almost always these tests were positive in cases of systemic cysticercosis in our region. This experience was confirmed also by other. [162, 164]. In 1987, Welsh et al [106] studied thirteen cases of ocular cysticercosis at King Edward VIII Hospital over an 8-year period from 1976 to 1984. All patients came from rural areas, a reflection on the low standards of hygiene. At that time, adolescent females ingested tapeworm eggs for slimming purposes, but none of this report was under that category. From their review of the medical literature they found that up to 1952, 1 216 cases of ocular cysticercosis were reported and when studied their patients most of them were occasional and came to South Africa from India. Of the 13 patients with ocular cysts nine were found in the vitreous, three in the anterior chamber and one under the conjunctiva of the eye. The majority of patients were female and all were Zulus living in areas of KwaZulu Natal, most of them complained of loss of vision for an average of 3 months. The only exception was one patient with the sub-conjunctival cyst who had waited 2 years before seeking treatment, since the lump had not affected his vision. Today all types of cysticercosis in KwaZulu Natal practically disappeared and patients with cysticercosis can be seen only at the former Transkei or another province but emigrated from this region (currently region C and D) of the Eastern Cape Province which does not mean that cysticercosis is going to disappear in

All patients from Welsh' series [107] presenting vitreous cysticercosis complained of severe loss of vision, and there was always a marked vitreoretinal reaction and the patients with cysts in the anterior chamber showed severe anterior chamber reactions with anterior uveitis. The vision was reduced in all patients to where hand movements could not be seen or to perception of light being absent or to complete blindness, except for the patient with the subconjunctival cyst . In this series of patients because of the severity of the lesions, surgical removal, although partly successful, did not improve vision and the distribution of the cysts in the various structures of the eye was similar to cases reported in the literature. We would like to highlight that around 70's and 80's years several cases presenting bilateral involvement of the eyes and/or multifocal intra-ocular cysts were most commonly seen.

Medical treatment for intraocular cysticercosis is not recommended based on South African's experiences with praziquantel (isquinoline pyrazine) [106] and another report from the same period of time. [166] Surgical removal is strongly recommended as the best treatment because complications associated with the cyst were minimal. Removal of

Removal of a cyst from the anterior chamber is relatively simple excepting cases pre-existing vitreoretinal-uveal reaction. [110, 168] The treatment of anterior chamber cysticercosis is essentially surgical. The different modalities used to remove the cyst include paracentesis, extraction with capsule forceps, cryo-extraction, diathermia, and viscoexpression. [78,

vitreous cysts by pars plana vitrectomy is the method of choice. [110,167]

The macular region is the site of choice for the subretinal cysticercus to lodge, maybe because of the high vascularization of this area but it can be seen in another subretinal area as well. In early stages, subretinal cysticercosis can appear as an acute central retinitis with retinal edema and subretinal exudates. The subretinal organism eventually develops into a cyst and the parasite and its movements can be recognized through the thin muscular layers. The observation of the cyst is more difficult when it is located in the periphery of the retina because scleral depression has to be used and the movements of the parasite cannot be observed clearly. (See figure 4 and 5)

Reportedly 35% of the cysts are in the subretinal space, 22% in the vitreous, 22% in the subconjunctival space, 5% in the anterior segment and 1% in the orbit. Among the intraocular cysts, 60% are found intravitreally and 40% are subretinal. [137,153]

When the parasite dies, an intraocular inflammation develops. Signs of acute anterior uveitis are present and severe opacification of the vitreous is observed. There are reports of dead and degenerating cysticerci causing a severe inflammatory reaction and ultimately loss of the eye. The cysts are known to release toxic products that cause severe inflammation mimicking endophthalmitis and even intraocular tumor. [171] The differential diagnosis with other parasitic infections and tumors has to be done. [144, 172, 173]

Orbital Cisticercosis. A Challenger for Neurologists,

Ophthalmologists, Neuro-Opthalmologists, and General Practitioners 125

Seems to be that the viable cysts can evoke less inflammatory response, [170] while the degenerating cyst rapidly increases in size due to osmotic regulation and causes compression of the surrounding tissues and release of antigens into the surrounding tissue

After the pars plana vitrectomy approach for removal of intravitreal cysts has developed, the prognosis of these eyes has improved. Another technique in conjunction with endolaser, diathermy, cryocoagulation, photocoagulation, and internal tamponade according to the retinal damage had been advocated for the treatment of cysticercosis. However, these methods will give rise to severe intraocular inflammation due to the toxins released from

[137, 172, 174, 175] If the cyst is ruptured, care should be taken to remove all of the residual debris to prevent severe postoperative inflammation. Without treatment for intraocular

Some report confirmed that the cyst usually presents in the sub-retinal space or vitreous cavity, where it can be observed ophthalmoscopically. The site of entry into the eye is most probably the choroidal vasculature from here the parasite can shift its location from subretinal space into the vitreous through a retinal break which could seal spontaneously, this passage incites inflammation, leaving behind a chorioretinal scar or even moves from

At this point, is good to remember what we explained before about cysts moving freely in the posterior chamber: apart from uveitis cysticercus may also lead to retinal hemorrhages, proliferative vitreoretinopathy, retinal detachment, disc edema, cyclitic membrane

In places where specialized retinal services are not available, external approach for the removal of sub retinal cyst is used. One of us (MCSC) has been used this surgical technique

**3.2. Approach to subretinal cyst removal using a conventional surgery technique** 

The conventional serial approach is a well-established surgical procedure for the treatment of subretinal cysts. This technique was the only one performed for many years to remove intraocular cysticercus (See figure 6) until advanced modern and easy methods were

The conventional method is more time consuming but it can be done in places where other

Number one priority is localizing the cyst by indirect ophthalmoscopy at the pre-operatory

2. To perform a limbal peritomy by grasping the conjunctiva and Tenon`s capsule at the level of the limbus with a toothed forceps and with a conjunctival scissors. The

inducing inflammatory response. [106, 156,170, 173]

parasites, the eyes go to the blindness in a short period of time.

anterior to posterior chamber and vice versa. [106,176-1978]

in places where other surgical approaches cannot be applied.

developed as the pars plana vitrectomy and internal tamponades.

1. To retract the lids using a wire lid retractor or sutures.

the dead larval tissues.

formation, and phthisis. [177,178]

facilities are not available.

stage and then to proceed as follows:

**Figure 4.** Shows subretinal cysticercosis seen by fundoscopy.

**Figure 5.** Another view of subretinal cysticercosis seen by fundoscopy (left & center) and picture of the fundus after surgical treatment on the right.

Seems to be that the viable cysts can evoke less inflammatory response, [170] while the degenerating cyst rapidly increases in size due to osmotic regulation and causes compression of the surrounding tissues and release of antigens into the surrounding tissue inducing inflammatory response. [106, 156,170, 173]

124 Novel Aspects on Cysticercosis and Neurocysticercosis

When the parasite dies, an intraocular inflammation develops. Signs of acute anterior uveitis are present and severe opacification of the vitreous is observed. There are reports of dead and degenerating cysticerci causing a severe inflammatory reaction and ultimately loss of the eye. The cysts are known to release toxic products that cause severe inflammation mimicking endophthalmitis and even intraocular tumor. [171] The differential diagnosis

with other parasitic infections and tumors has to be done. [144, 172, 173]

**Figure 4.** Shows subretinal cysticercosis seen by fundoscopy.

fundus after surgical treatment on the right.

**Figure 5.** Another view of subretinal cysticercosis seen by fundoscopy (left & center) and picture of the

After the pars plana vitrectomy approach for removal of intravitreal cysts has developed, the prognosis of these eyes has improved. Another technique in conjunction with endolaser, diathermy, cryocoagulation, photocoagulation, and internal tamponade according to the retinal damage had been advocated for the treatment of cysticercosis. However, these methods will give rise to severe intraocular inflammation due to the toxins released from the dead larval tissues.

[137, 172, 174, 175] If the cyst is ruptured, care should be taken to remove all of the residual debris to prevent severe postoperative inflammation. Without treatment for intraocular parasites, the eyes go to the blindness in a short period of time.

Some report confirmed that the cyst usually presents in the sub-retinal space or vitreous cavity, where it can be observed ophthalmoscopically. The site of entry into the eye is most probably the choroidal vasculature from here the parasite can shift its location from subretinal space into the vitreous through a retinal break which could seal spontaneously, this passage incites inflammation, leaving behind a chorioretinal scar or even moves from anterior to posterior chamber and vice versa. [106,176-1978]

At this point, is good to remember what we explained before about cysts moving freely in the posterior chamber: apart from uveitis cysticercus may also lead to retinal hemorrhages, proliferative vitreoretinopathy, retinal detachment, disc edema, cyclitic membrane formation, and phthisis. [177,178]

In places where specialized retinal services are not available, external approach for the removal of sub retinal cyst is used. One of us (MCSC) has been used this surgical technique in places where other surgical approaches cannot be applied.

## **3.2. Approach to subretinal cyst removal using a conventional surgery technique**

The conventional serial approach is a well-established surgical procedure for the treatment of subretinal cysts. This technique was the only one performed for many years to remove intraocular cysticercus (See figure 6) until advanced modern and easy methods were developed as the pars plana vitrectomy and internal tamponades.

The conventional method is more time consuming but it can be done in places where other facilities are not available.

Number one priority is localizing the cyst by indirect ophthalmoscopy at the pre-operatory stage and then to proceed as follows:


extension varies according to the size of the cyst. Radial relaxing incisions is made through the conjunctiva and Tenon`s capsule in oblique meridians to prevent tearing of the conjunctiva during the surgery and avoid further complications as a symblepharon formation.

Orbital Cisticercosis. A Challenger for Neurologists,

Ophthalmologists, Neuro-Opthalmologists, and General Practitioners 127

11. In case the cyst burst during the extraction a thorough irrigation has to be done.

transvitreally. [112]

obtained. [178]

surgical removal of the cysticercus.

were reported by others. [178-182]

Neurocysticercosis. (NCC)

**3.3. Optic nerve neurocysticercosis** 

The cysticercosis does not affect the spinal nerves or cranial nerves.

Subretinal cyst anterior to the equator may be removed trans sclerally, whereas subretinal cysts posterior to the equator and intravitreal cysts are best removed

Under conventional surgical procedures by sclerotomy exactly over the subretinal *cyst*  localized by scleral depression under indirect ophthalmoscopy, other authors also obtained successful surgical removal of subretinal cysticerci including patients with an associated exudative retina detachment with extensive gliosis, fixed folds, focal retinitis, and multiple subretinal precipitates specially near to the cyst but fatal results in patients presenting multifocal intraocular cysticercosis and severe NCC (even in the non-pork eaters) is

In the treatment of intraocular cysticercosis, the most common surgical approach is removal through the pars plana route following vitrectomy. Ideally, the cyst should be removed in toto. Complete surgical removal of the intact cyst results in good functional recovery in eyes with intraocular cysticercosis. [29] Systemic corticosteroids are used before and after

Patnaik and Kalsi [178] also reported a progressive pre-retinal gliosis with the passage of time with the evagination of the scolex against the retina, the latter is perforated, often in more than one point. They comment that the cyst through one of these perforations can escape into the vitreous cavity and the detached retina continues to shrink with increasing gliosis and vitreous becomes progressively hazy which becomes totally impenetrable with massive exudation then the parasite cannot be located. It may be presumed that the sudden advent of massive inflammation is associated with rupture of the cyst or death of the parasite. The eye is lost with massive inflammation. Even the successful removal of the subretinal cyst if not conducted early enough may not prevent the progressive deterioration of the retina and vitreous probably because of continued inflammation. The same results

The optic (second cranial) nerve is the nerve of sight, and it extends from the eye to the optic chiasm. Developmentally, it may be considered as a tract between the retina (a derivative of the brain) and the brain. The nerve fibers, which arise in the retina, converge on the optic disc, pierce the layers of the eye, and receive myelin sheaths. The optic nerve, itself, is surrounded by meningeal sheaths continuous with those of the brain, and also by the subarachnoid space. As is known the optic nerve originates from the axonal extensions of the ganglion cells of the retina which is an extension of the central nervous system. Therefore this type of presentation of cysticercosis should be grouped within the category of


**Figure 6.** External approach to subretinal cysticercosis.


11. In case the cyst burst during the extraction a thorough irrigation has to be done.

Subretinal cyst anterior to the equator may be removed trans sclerally, whereas subretinal cysts posterior to the equator and intravitreal cysts are best removed transvitreally. [112]

Under conventional surgical procedures by sclerotomy exactly over the subretinal *cyst*  localized by scleral depression under indirect ophthalmoscopy, other authors also obtained successful surgical removal of subretinal cysticerci including patients with an associated exudative retina detachment with extensive gliosis, fixed folds, focal retinitis, and multiple subretinal precipitates specially near to the cyst but fatal results in patients presenting multifocal intraocular cysticercosis and severe NCC (even in the non-pork eaters) is obtained. [178]

In the treatment of intraocular cysticercosis, the most common surgical approach is removal through the pars plana route following vitrectomy. Ideally, the cyst should be removed in toto. Complete surgical removal of the intact cyst results in good functional recovery in eyes with intraocular cysticercosis. [29] Systemic corticosteroids are used before and after surgical removal of the cysticercus.

Patnaik and Kalsi [178] also reported a progressive pre-retinal gliosis with the passage of time with the evagination of the scolex against the retina, the latter is perforated, often in more than one point. They comment that the cyst through one of these perforations can escape into the vitreous cavity and the detached retina continues to shrink with increasing gliosis and vitreous becomes progressively hazy which becomes totally impenetrable with massive exudation then the parasite cannot be located. It may be presumed that the sudden advent of massive inflammation is associated with rupture of the cyst or death of the parasite. The eye is lost with massive inflammation. Even the successful removal of the subretinal cyst if not conducted early enough may not prevent the progressive deterioration of the retina and vitreous probably because of continued inflammation. The same results were reported by others. [178-182]

#### **3.3. Optic nerve neurocysticercosis**

126 Novel Aspects on Cysticercosis and Neurocysticercosis

the muscle to do the fixation.

**Figure 6.** External approach to subretinal cysticercosis.

treatment to avoid bleeding trans operators

sutures cannot be placed under the muscles.

9. Sometimes more diathermy is necessary

the conjunctiva with Vicryl is done.

incision.

formation.

extension varies according to the size of the cyst. Radial relaxing incisions is made through the conjunctiva and Tenon`s capsule in oblique meridians to prevent tearing of the conjunctiva during the surgery and avoid further complications as a symblepharon

4. Isolated two or three rectus muscles according to the place the surgeon is going to do the scleral incision. Here is important to highlight do not cut the muscle belly to avoid bleeding. Using a muscle hook passed underside and 4-0 silk suture is passed around

5. Localization of the subretinal cyst is done again with the indirect ophthalmoscope and the assistant help to mark the limits on the sclera where the incision will be made. These

6. The Scleral incision has to be made at right angles to the surface of the sclera to maintain a uniform depth of the undermining. The incision should extend more than two thirds of the scleral thickness to have a floor for the placement of the diathermy

7. 4-0 or 5-0 polyester sutures with spatulated needle to close the scleral flap are used. The sutures are placed 2mm apart in a radial fashion and approximately 1.5 mm from the edge of the scleral flap. Two or three mattress sutures are placed in each quadrant. If the incision is bigger than a single quadrant with 2 sutures in each one is enough. The

8. The scleral lamina is perforated and the cyst is expressed out through a choroidal

10. Scleral sutures are closed, suture of the muscles release and closure of the Tenon`s and

marks can be done with the use of the diathermy or with a surgical pen.

3. Scleral exposure has taken care to avoid damage of the vortex veins.

The cysticercosis does not affect the spinal nerves or cranial nerves.

The optic (second cranial) nerve is the nerve of sight, and it extends from the eye to the optic chiasm. Developmentally, it may be considered as a tract between the retina (a derivative of the brain) and the brain. The nerve fibers, which arise in the retina, converge on the optic disc, pierce the layers of the eye, and receive myelin sheaths. The optic nerve, itself, is surrounded by meningeal sheaths continuous with those of the brain, and also by the subarachnoid space. As is known the optic nerve originates from the axonal extensions of the ganglion cells of the retina which is an extension of the central nervous system. Therefore this type of presentation of cysticercosis should be grouped within the category of Neurocysticercosis. (NCC)

Although other zoonotic parasites (Trematode) have been found in the layers of the retina at longitudinal position [183] the size and shape of cysticercus exceed the thickness of the retina so that its location is below it, and in close relation to the vascular layer of which is nourished. For certain reasons when the larva is leaving the subretinal region only has the option to do so toward the vitreous cavity because the resistance given by the sclera to be crossed.

Orbital Cisticercosis. A Challenger for Neurologists,

Ophthalmologists, Neuro-Opthalmologists, and General Practitioners 129

nerve swelling was detected with a shadow of scolex. Abnormal P-100 values of visual

This condition is often mistaken for optic nerve tumors and on neuroimaging the diagnosis is often delayed or missed. [197] However, imagenology studies based on contrast-enhanced MR imaging study and contrast-enhanced CT usually reveals a ring-enhancing cyst with a mural nodule located in the optic nerve although sometimes it only shows a thickened of the optic nerve with or without ring-enhancing lesion containing an eccentric nodule. [190,193, 197, 198] Ultrasonography can also help to determine the cause of optic neuritis due to the disorganization of the cyst with inflammation of the adjacent optic nerve. [187, 191, 193, 195, 197-201] and these investigations are useful to confirm: response to the treatment and patient's compliance. Other investigation to support the diagnostic process is an enzyme-

Medical therapy in the form of steroids along with albendazol was ineffective. Surgical removal by lateral orbitotomy was done and a complete cyst with scolex was removed, which was confirmed histopathologically as a case of retrobulbar optic nerve cysticercosis. [189] Next patient, a 50-year-old woman with atypical optic neuritis was also treated with oral prednisolone and albendazole, with no improvement in vision [190] While other patients presenting optic nerve cysticercosis including bilateral optic nerve involvement

Ocular and extraocular cysticercosis are a commonly encountered manifestation of the disease caused by the cysticercus cellulosae. Any region of the eye may be afflicted including the subconjunctival space. The conjunctiva is a connection between the eyelids, sclera and cornea. It is the mucous membrane that lines the posterior surface of the eyelids

The host inflammatory response to cysticerci located into the conjunctival depends on the parasite's ability to evade host immunity; therefore, inflammation is restricted to degenerating cysts whose ability to evade host defenses is faltering. Lack of inflammation occurs with both healthy cysticerci (vesicular stage) and those that have involuted (inactive disease). Upon involution, cysts undergo granulomatous change and exhibit calcification. [70] It almost never happens in the conjunctiva region. Subconjunctival cysticercosis usually presents as a painful, yellowish, nodular subconjunctival mass with surrounding conjunctival congestion or may even present as an eyelid nodule or even as subconjuctival abscess from orbital myocysticercosis. [202] Patients presenting lacrimal canalicular

The most common outcome is the spontaneous extrusion of the T solium cysticercus. [90-92, 94-96,98, 203] sometimes they got extruded from the orbit or from subconjunctival space but

(palpebral conjunctiva) and the anterior aspect of the globe (bulbar conjunctiva).

evoked potential can be useful for supporting a differential diagnosis.

linked immunosorbent assay test for cysticercosis. [190, 193, 195, 196]

improved dramatically with prednisone and albendazole. [191, 193,195]

**4. Conjunctival cysticercosis** 

obstruction have been reported as well. [85, 97]

almost always associated with clinical improvement. [90]

The NCC of the optic nerve is extremely uncommon and until today no more than 10 patients have been well documented. [184-200]

As we discussed in the previous chapter orbital cysticercosis as a presentation of disseminating cysticercosis has not been reported in the medical literature excepting one anecdotal case [194]. The first well documented patient presenting optic nerve cysticercosis was from India (1991). Other authors [185] reported a 15-years-old female with progressive deterioration of her visual acuity of seven months duration. Her CT scan revealed a right retrobulbar optic nerve thickened with small area of low attenuation in the thickened portion of the optic nerve. A diagnosis of optic nerve glioma or granulomas was considered and a right frontal craniotomy and extradural frontal orbitomy was performed, they found an intense fibrosis and or deeper incision a sago grain like cyst was identified and excised. Histopathology revealed it to be a cysticercosis. Postoperatively the patient's vision full was completely recovered but there was an oculomotor nerve paresis. Similar patient was not described previously. Five years later, Bousquest et al [186] reported a first case of intraoptic neurocysticercosis in a 12-year-old boy living on Reunion Island (France). They also initially considered a diagnosis of optic nerve tumor and later computerized tomography scans and surgical aspects confirmed a diagnosis of retrobulbar optic nerve cysticercosis recommending a conservative removal using en bloc orbitotomy because of good functional and aesthetic results. Despite a first case was reported before we would like to highlight that Bousquest [186] was the first to use the correct terminology for cysticercosis involving the optic nerve such as: Intraoptic neurocysticercosis.

Other anecdotic report about the occurrence of optic neuritis following oral albendazol therapy for orbital cysticercosis was done in 1998 [187] but this one served to highlight the importance of adding a steroid medication to Albendazole in the treatment of all cases of orbital cysticercosis mainly when cysts are in close proximity to the optic nerve. They confirmed a full visual recovery after resolution of optic neuritis after four weeks treatment with Prednisolone at the dose of 1mg/kg per day.

Another 15-years-old-female presenting cysticercosis of the optic nerve is reported by Gurha et al [188] This patient presented with rapidly diminishing vision in the left eye, headache and papillitis. A magnetic resonance imaging revealed a cystic lesion at the entrance of the optic canal. Surgery performed was a transcranial orbitotomy which included deroofing of the optic canal and removal of the cyst from under the sheath of the optic nerve and the outcome was a remarkable visual recovery. At the same time a 15-year-old boy presented with diminution of vision which rapidly progressed to no perception of light. In the ocular fundus, a neuroretinitis-like picture was seen. On CT-scan and ultrasonography, an optic nerve swelling was detected with a shadow of scolex. Abnormal P-100 values of visual evoked potential can be useful for supporting a differential diagnosis.

This condition is often mistaken for optic nerve tumors and on neuroimaging the diagnosis is often delayed or missed. [197] However, imagenology studies based on contrast-enhanced MR imaging study and contrast-enhanced CT usually reveals a ring-enhancing cyst with a mural nodule located in the optic nerve although sometimes it only shows a thickened of the optic nerve with or without ring-enhancing lesion containing an eccentric nodule. [190,193, 197, 198] Ultrasonography can also help to determine the cause of optic neuritis due to the disorganization of the cyst with inflammation of the adjacent optic nerve. [187, 191, 193, 195, 197-201] and these investigations are useful to confirm: response to the treatment and patient's compliance. Other investigation to support the diagnostic process is an enzymelinked immunosorbent assay test for cysticercosis. [190, 193, 195, 196]

Medical therapy in the form of steroids along with albendazol was ineffective. Surgical removal by lateral orbitotomy was done and a complete cyst with scolex was removed, which was confirmed histopathologically as a case of retrobulbar optic nerve cysticercosis. [189] Next patient, a 50-year-old woman with atypical optic neuritis was also treated with oral prednisolone and albendazole, with no improvement in vision [190] While other patients presenting optic nerve cysticercosis including bilateral optic nerve involvement improved dramatically with prednisone and albendazole. [191, 193,195]

## **4. Conjunctival cysticercosis**

128 Novel Aspects on Cysticercosis and Neurocysticercosis

patients have been well documented. [184-200]

optic nerve such as: Intraoptic neurocysticercosis.

with Prednisolone at the dose of 1mg/kg per day.

crossed.

Although other zoonotic parasites (Trematode) have been found in the layers of the retina at longitudinal position [183] the size and shape of cysticercus exceed the thickness of the retina so that its location is below it, and in close relation to the vascular layer of which is nourished. For certain reasons when the larva is leaving the subretinal region only has the option to do so toward the vitreous cavity because the resistance given by the sclera to be

The NCC of the optic nerve is extremely uncommon and until today no more than 10

As we discussed in the previous chapter orbital cysticercosis as a presentation of disseminating cysticercosis has not been reported in the medical literature excepting one anecdotal case [194]. The first well documented patient presenting optic nerve cysticercosis was from India (1991). Other authors [185] reported a 15-years-old female with progressive deterioration of her visual acuity of seven months duration. Her CT scan revealed a right retrobulbar optic nerve thickened with small area of low attenuation in the thickened portion of the optic nerve. A diagnosis of optic nerve glioma or granulomas was considered and a right frontal craniotomy and extradural frontal orbitomy was performed, they found an intense fibrosis and or deeper incision a sago grain like cyst was identified and excised. Histopathology revealed it to be a cysticercosis. Postoperatively the patient's vision full was completely recovered but there was an oculomotor nerve paresis. Similar patient was not described previously. Five years later, Bousquest et al [186] reported a first case of intraoptic neurocysticercosis in a 12-year-old boy living on Reunion Island (France). They also initially considered a diagnosis of optic nerve tumor and later computerized tomography scans and surgical aspects confirmed a diagnosis of retrobulbar optic nerve cysticercosis recommending a conservative removal using en bloc orbitotomy because of good functional and aesthetic results. Despite a first case was reported before we would like to highlight that Bousquest [186] was the first to use the correct terminology for cysticercosis involving the

Other anecdotic report about the occurrence of optic neuritis following oral albendazol therapy for orbital cysticercosis was done in 1998 [187] but this one served to highlight the importance of adding a steroid medication to Albendazole in the treatment of all cases of orbital cysticercosis mainly when cysts are in close proximity to the optic nerve. They confirmed a full visual recovery after resolution of optic neuritis after four weeks treatment

Another 15-years-old-female presenting cysticercosis of the optic nerve is reported by Gurha et al [188] This patient presented with rapidly diminishing vision in the left eye, headache and papillitis. A magnetic resonance imaging revealed a cystic lesion at the entrance of the optic canal. Surgery performed was a transcranial orbitotomy which included deroofing of the optic canal and removal of the cyst from under the sheath of the optic nerve and the outcome was a remarkable visual recovery. At the same time a 15-year-old boy presented with diminution of vision which rapidly progressed to no perception of light. In the ocular fundus, a neuroretinitis-like picture was seen. On CT-scan and ultrasonography, an optic Ocular and extraocular cysticercosis are a commonly encountered manifestation of the disease caused by the cysticercus cellulosae. Any region of the eye may be afflicted including the subconjunctival space. The conjunctiva is a connection between the eyelids, sclera and cornea. It is the mucous membrane that lines the posterior surface of the eyelids (palpebral conjunctiva) and the anterior aspect of the globe (bulbar conjunctiva).

The host inflammatory response to cysticerci located into the conjunctival depends on the parasite's ability to evade host immunity; therefore, inflammation is restricted to degenerating cysts whose ability to evade host defenses is faltering. Lack of inflammation occurs with both healthy cysticerci (vesicular stage) and those that have involuted (inactive disease). Upon involution, cysts undergo granulomatous change and exhibit calcification. [70] It almost never happens in the conjunctiva region. Subconjunctival cysticercosis usually presents as a painful, yellowish, nodular subconjunctival mass with surrounding conjunctival congestion or may even present as an eyelid nodule or even as subconjuctival abscess from orbital myocysticercosis. [202] Patients presenting lacrimal canalicular obstruction have been reported as well. [85, 97]

The most common outcome is the spontaneous extrusion of the T solium cysticercus. [90-92, 94-96,98, 203] sometimes they got extruded from the orbit or from subconjunctival space but almost always associated with clinical improvement. [90]

Anterior subconjunctival cysts may be treated with excision biopsy .As the cyst is usually adherent to the adjacent muscle, excision may be difficult. Care must be taken to keep the extraocular muscle intact during dissection because an excessive intraoperative dissection of the sub conjunctival cyst may damage the extraocular muscle fibers, leading to postoperative diplopia and strabismus. [89, 99]

Orbital Cisticercosis. A Challenger for Neurologists,

Ophthalmologists, Neuro-Opthalmologists, and General Practitioners 131

The rectus muscles are all approximately 40 mm long and each receives innervation from the undersurface (intraconal space) at the junction of the middle and posterior thirds of the

To achieve a better understanding of this variant of solium tapeworm infestation of the extraocular muscles, we have decided to merge the contents of sub chapter in accordance to the affected muscles after committing a first attention to the more important type of

Before continue, our readership should remember that other causes of thickening of the extraocular muscle such as: fatty infiltration of the muscle can also cause a bulging of the eye anteriorly out of the orbit (Graves-Basedow disease) but in those cases we prefer the medical term: exophthalmos. In other words, we reserve the medical terminology of exophthalmos for those cases of protrusion of the eyes related to endocrine dysfunction.

One of the primary symptoms of extraocular cysticercosis is double vision (diplopia) from misalignment of the visual axes, and the pattern of image separation is the key to diagnosing which particular cranial nerve (and extraocular muscle) is involved. Therefore, at this stage is important to know how to approach these patients. Our first recommendation is to break down between monocular (i.e. present with one eye closed) and binocular diplopia (far more common). For causes of monocular diplopia is important to check the ophthalmological function mainly. If binocular diplopia is present then is important to look for: variations on the quality of diplopia through the day (myasthenia gravis), associated endocrinopathy (Graves-Basedow disease), orbital fracture (most likely to cause inferior oblique palsy), orbital tumours, gaze palsy (progressive supranuclear palsy, brainstem disorders, Wernicke's, Miller-Fisher, cavernous sinus syndrome, etc.). If your patient complains of diplopia on looking in one diagonal direction, it is either the samesided and oriented rectus muscle or the other-sided oppositely-oriented oblique muscle; e.g. diplopia on looking up and right, it is either right superior rectus or left inferior oblique. If your patient complaints of diplopia on looking down and left is either left inferior rectus or right superior oblique. Finally, if you cover up an eye when the patient is complaining of diplopia, the eye that, when covered, takes away the most peripheral

We use the medical terminology of proptosis when there is a protrusion of one or both eyeballs by at least two millimeters but less than 18 mm, which can be congenital, familial,

Apart from extraocular cysticercosis as a cause of proptosis other etiologies include: leukemias, orbital cellulitis, meningioma of the sphenoid wing, dacryoadenitis, mucormycosis, dermoid, orbital fracture of the apex, floor, medial wall or zygomatic bone, Duane syndrome, cavernous sinus syndrome, congenital glaucoma, nasopharyngeal angiofibroma, Hand-Schuller-Christian disease, hemangioma, high altitude cerebral edema

muscle or 26 mm from the insertion

presentation: the proptosis.

image is the problem.

**5.1. Proptosis of the eyes secondary to cysticercosis** 

or due to a pathological condition not secondary to endocrinopathies.

## **5. Extraocular muscle cysticercosis**

Extraocular muscles differ histologically from most other skeletal muscles in that they are made up of two different types of muscle cells. Each muscle cell is composed of groups of myofibrils called sarcomeres. Fast-twitch muscle fibrils generate fast eye movements and are composed of well-defined myofibrils with well-developed sarcomeres while slow-twitch muscle fibrils generate slow or tonic eye movements and are composed of poorly defined myofibrils with poorly developed sarcomeres. Cholinergic motor neurons supply both types of muscle fibers. The innervation to fast-twitch fibrils is thick and heavily myelinated, with a single (en plaque) neuromuscular junction, whereas the innervation to slow-twitch fibrils is thin, with multiple grapelike clusters of neuromuscular junctions. [204] The eye's major blood supply comes from the ophthalmic artery. The lateral muscular branch of the ophthalmic artery supplies the lateral rectus, superior rectus, and superior oblique muscles. The medial muscular branch supplies the inferior rectus, medial rectus, and inferior oblique muscles. Medial and lateral muscular branches of the artery give rise to seven anterior ciliary vessels, which travel with the four rectus muscles to provide circulation for the anterior segment of the eye. Each rectus muscle has two anterior ciliary vessels, except for the lateral rectus muscle, which has 1 vessel. These vessels pass anteriorly to the episclera and supply the anterior segment of the eye, including the sclera, limbus, and conjunctiva. [204]

The extraocular muscles rotate the eyeball around vertical, horizontal and antero-posterior axes. Extraocular muscles other than the medial rectus and lateral rectus have more than one action due to the angle they make with the optical axis of the eye while inserting into the eyeball. The superior and inferior oblique muscles make an angle of 51 degrees with the optical axis.

As we before-cited, orbital cysticercosis is an uncommon presentation of cysticercosis even in endemic countries. In some South African provinces where it was seen before, it does exist at the present moment, and infestation of extraocular muscle remains exceedingly rare; but because of the versatility of the presentation, cysticercosis still should be considered in the differential diagnosis of many orbital disorders, especially in an endemic region and extraocular muscle cysticercosis should be considered in the differential diagnosis of recent acquired motility disorder or proptosis. According to several publications in the international medical literature and based on our experience we fully support that cysticercosis is a pleomorphic disorder whose presentation depends on a combination of inflammatory response, topography of the lesions, degree of parasitic load and sequelae of previous infestation as was established by others as well. [78, 205-208]

The rectus muscles are all approximately 40 mm long and each receives innervation from the undersurface (intraconal space) at the junction of the middle and posterior thirds of the muscle or 26 mm from the insertion

130 Novel Aspects on Cysticercosis and Neurocysticercosis

postoperative diplopia and strabismus. [89, 99]

**5. Extraocular muscle cysticercosis** 

optical axis.

Anterior subconjunctival cysts may be treated with excision biopsy .As the cyst is usually adherent to the adjacent muscle, excision may be difficult. Care must be taken to keep the extraocular muscle intact during dissection because an excessive intraoperative dissection of the sub conjunctival cyst may damage the extraocular muscle fibers, leading to

Extraocular muscles differ histologically from most other skeletal muscles in that they are made up of two different types of muscle cells. Each muscle cell is composed of groups of myofibrils called sarcomeres. Fast-twitch muscle fibrils generate fast eye movements and are composed of well-defined myofibrils with well-developed sarcomeres while slow-twitch muscle fibrils generate slow or tonic eye movements and are composed of poorly defined myofibrils with poorly developed sarcomeres. Cholinergic motor neurons supply both types of muscle fibers. The innervation to fast-twitch fibrils is thick and heavily myelinated, with a single (en plaque) neuromuscular junction, whereas the innervation to slow-twitch fibrils is thin, with multiple grapelike clusters of neuromuscular junctions. [204] The eye's major blood supply comes from the ophthalmic artery. The lateral muscular branch of the ophthalmic artery supplies the lateral rectus, superior rectus, and superior oblique muscles. The medial muscular branch supplies the inferior rectus, medial rectus, and inferior oblique muscles. Medial and lateral muscular branches of the artery give rise to seven anterior ciliary vessels, which travel with the four rectus muscles to provide circulation for the anterior segment of the eye. Each rectus muscle has two anterior ciliary vessels, except for the lateral rectus muscle, which has 1 vessel. These vessels pass anteriorly to the episclera and supply the

anterior segment of the eye, including the sclera, limbus, and conjunctiva. [204]

previous infestation as was established by others as well. [78, 205-208]

The extraocular muscles rotate the eyeball around vertical, horizontal and antero-posterior axes. Extraocular muscles other than the medial rectus and lateral rectus have more than one action due to the angle they make with the optical axis of the eye while inserting into the eyeball. The superior and inferior oblique muscles make an angle of 51 degrees with the

As we before-cited, orbital cysticercosis is an uncommon presentation of cysticercosis even in endemic countries. In some South African provinces where it was seen before, it does exist at the present moment, and infestation of extraocular muscle remains exceedingly rare; but because of the versatility of the presentation, cysticercosis still should be considered in the differential diagnosis of many orbital disorders, especially in an endemic region and extraocular muscle cysticercosis should be considered in the differential diagnosis of recent acquired motility disorder or proptosis. According to several publications in the international medical literature and based on our experience we fully support that cysticercosis is a pleomorphic disorder whose presentation depends on a combination of inflammatory response, topography of the lesions, degree of parasitic load and sequelae of To achieve a better understanding of this variant of solium tapeworm infestation of the extraocular muscles, we have decided to merge the contents of sub chapter in accordance to the affected muscles after committing a first attention to the more important type of presentation: the proptosis.

Before continue, our readership should remember that other causes of thickening of the extraocular muscle such as: fatty infiltration of the muscle can also cause a bulging of the eye anteriorly out of the orbit (Graves-Basedow disease) but in those cases we prefer the medical term: exophthalmos. In other words, we reserve the medical terminology of exophthalmos for those cases of protrusion of the eyes related to endocrine dysfunction.

One of the primary symptoms of extraocular cysticercosis is double vision (diplopia) from misalignment of the visual axes, and the pattern of image separation is the key to diagnosing which particular cranial nerve (and extraocular muscle) is involved. Therefore, at this stage is important to know how to approach these patients. Our first recommendation is to break down between monocular (i.e. present with one eye closed) and binocular diplopia (far more common). For causes of monocular diplopia is important to check the ophthalmological function mainly. If binocular diplopia is present then is important to look for: variations on the quality of diplopia through the day (myasthenia gravis), associated endocrinopathy (Graves-Basedow disease), orbital fracture (most likely to cause inferior oblique palsy), orbital tumours, gaze palsy (progressive supranuclear palsy, brainstem disorders, Wernicke's, Miller-Fisher, cavernous sinus syndrome, etc.). If your patient complains of diplopia on looking in one diagonal direction, it is either the samesided and oriented rectus muscle or the other-sided oppositely-oriented oblique muscle; e.g. diplopia on looking up and right, it is either right superior rectus or left inferior oblique. If your patient complaints of diplopia on looking down and left is either left inferior rectus or right superior oblique. Finally, if you cover up an eye when the patient is complaining of diplopia, the eye that, when covered, takes away the most peripheral image is the problem.

### **5.1. Proptosis of the eyes secondary to cysticercosis**

We use the medical terminology of proptosis when there is a protrusion of one or both eyeballs by at least two millimeters but less than 18 mm, which can be congenital, familial, or due to a pathological condition not secondary to endocrinopathies.

Apart from extraocular cysticercosis as a cause of proptosis other etiologies include: leukemias, orbital cellulitis, meningioma of the sphenoid wing, dacryoadenitis, mucormycosis, dermoid, orbital fracture of the apex, floor, medial wall or zygomatic bone, Duane syndrome, cavernous sinus syndrome, congenital glaucoma, nasopharyngeal angiofibroma, Hand-Schuller-Christian disease, hemangioma, high altitude cerebral edema

(proptosis), Beckwith–Wiedemann syndrome, aortic incompetence: manifests as a pulsatile pseudo proptosis, Wegener's granulomatosis and Pfeiffer syndrome.

Orbital Cisticercosis. A Challenger for Neurologists,

Ophthalmologists, Neuro-Opthalmologists, and General Practitioners 133

palpebrae superioris myocysticercosis include a young patient from an endemic region with a suggestive exposure history, proptosis, mild lid edema or erythema and a palpable cystic eyelid mass. [136] Sekhar et al [78] reported that subconjunctival cyst might be related to

Only six patients (all from India) have been reported in the medical literature, they had between 9 and 23 years of age and most of some of them complained of unilateral ptosis, inflammation of the upper eyelid and restricted ocular motility while another complained of mild upper eyelid discomfort without display or decreased vision and no lid erythema or

There are also other parasitic infestations that can induce ptosis. Echinococcosis [220] and trichuriasis [221] are the two widely mentioned parasitic infestations that can induce ptosis. Therefore neuroimaging studies are useful to distinguish myocysticercosis from other cystic lesions of the extraocular muscles, such as echinocococus granulosus (hydatid cyst), cystic tumors or extraocular myositis with cystic changes. A hydatid cyst lacks a scolex and does not usually demonstrate intense rim enhancement. However, CT may be preferable as intracranial CT can identify cerebral cysticercosis, which was as high as 16.7% in a case series of myocysticercosis. [155] The diagnosis by neuroimagen is important as myocysticercosis responds well to anti-helminthic therapy, while surgical excision is the primary treatment modality for hydatid cysts or tumors. Ultrasound and CT scan appear

In patients presenting fluctuating palpebral ptosis with negative neostigmine and electrophysiological tests (pseudo myasthenia) cysticercosis of midbrain should be investigated because focal encephalitis and edema at the midbrain may cause this

Indeed, ocular cysticercosis is not a rare disease in the developing countries, and ptosis due to cysticercosis is sporadically reported [223] but it should keep it mind during the

The superior rectus muscle is a muscle in the orbit. It is one of the extraocular muscles. It is innervated by the superior division of the oculomotor nerve (Cranial Nerve III). In the primary position (looking straight ahead), the superior rectus muscle's primary function is

Extraocular muscle cysticercosis is the most common site of this parasitic disease when

In some series of patients with extraocular cysticercosis, the superior rectus muscle is the most commonly affected (33.3%) compared other involved muscles. [77] and clinical features usually are painful unilateral ptosis, inflammation of the upper eyelid and some

ptosis and suggested that the surgical removal of the parasite is a good management.

edema plus full extraocular movements in all directions of gaze. [14, 29, 218, 219]

comparable in ability to detect the scolex. [29, 136]

differential diagnosis of unilateral eyelid ptosis.

**5.3. Superior rectus muscle cysticercosis** 

elevation, although it also contributes to intorsion and adduction.

symptomatology. [222]

involving the orbit. [224]

Proptosis due to cysticercosis has been reported also in children, usually presenting progressive painful protrusion of the eye with redness, lacrimination and diminution of vision [206] but it is an uncommon cause of proptosis in all age groups with reported incidence varying from 0 to 20%, [208-213] some authors say that the commonest cause of proptosis is medial rectus cysticercosis [208] and the frequency of proptosis among other signs of orbital cysticercosis is around 20-30% preceded by ocular motility restriction (60- 64%) and followed by diplopia (35-38%) and palpebral ptosis (12-16%). [77]

## **5.2. Levator palpebrae superioris muscle cysticercosis and palpebral ptosis**

The Levator palpebrae superioris originates on the lesser wing of the sphenoid bone, just above the optic foramen. It broadens and becomes the Levator aponeurosis. This portion inserts on the skin of the upper eyelid, as well as the superior tarsal plate. It is a skeletal muscle. The superior tarsal muscle, a smooth muscle, is attached to the levator palpebrae superioris, and inserts on the superior tarsal plate as well. (See figure 7)

**Figure 7.** Representation of cysticercosis in the Levator palpebrae superioris.

It is well known that cysticercosis of the extraocular musculature is rare and within this group the affectation of the eyelid is about 0.6% [77] despite the number of patients reported in the medical literature. [214-217] The Levator palpebrae superioris muscle cysticercosis presenting clinically as ptosis is much more uncommon than other affected extraocular muscles. [218, 219]

Cysticercosis of the Levator palpebrae superioris, superior rectus, eyelid or anterior orbit are important differential diagnoses for acquiring ptosis. [219] Features suggestive of Levator palpebrae superioris myocysticercosis include a young patient from an endemic region with a suggestive exposure history, proptosis, mild lid edema or erythema and a palpable cystic eyelid mass. [136] Sekhar et al [78] reported that subconjunctival cyst might be related to ptosis and suggested that the surgical removal of the parasite is a good management.

Only six patients (all from India) have been reported in the medical literature, they had between 9 and 23 years of age and most of some of them complained of unilateral ptosis, inflammation of the upper eyelid and restricted ocular motility while another complained of mild upper eyelid discomfort without display or decreased vision and no lid erythema or edema plus full extraocular movements in all directions of gaze. [14, 29, 218, 219]

There are also other parasitic infestations that can induce ptosis. Echinococcosis [220] and trichuriasis [221] are the two widely mentioned parasitic infestations that can induce ptosis. Therefore neuroimaging studies are useful to distinguish myocysticercosis from other cystic lesions of the extraocular muscles, such as echinocococus granulosus (hydatid cyst), cystic tumors or extraocular myositis with cystic changes. A hydatid cyst lacks a scolex and does not usually demonstrate intense rim enhancement. However, CT may be preferable as intracranial CT can identify cerebral cysticercosis, which was as high as 16.7% in a case series of myocysticercosis. [155] The diagnosis by neuroimagen is important as myocysticercosis responds well to anti-helminthic therapy, while surgical excision is the primary treatment modality for hydatid cysts or tumors. Ultrasound and CT scan appear comparable in ability to detect the scolex. [29, 136]

In patients presenting fluctuating palpebral ptosis with negative neostigmine and electrophysiological tests (pseudo myasthenia) cysticercosis of midbrain should be investigated because focal encephalitis and edema at the midbrain may cause this symptomatology. [222]

Indeed, ocular cysticercosis is not a rare disease in the developing countries, and ptosis due to cysticercosis is sporadically reported [223] but it should keep it mind during the differential diagnosis of unilateral eyelid ptosis.

## **5.3. Superior rectus muscle cysticercosis**

132 Novel Aspects on Cysticercosis and Neurocysticercosis

(proptosis), Beckwith–Wiedemann syndrome, aortic incompetence: manifests as a pulsatile

Proptosis due to cysticercosis has been reported also in children, usually presenting progressive painful protrusion of the eye with redness, lacrimination and diminution of vision [206] but it is an uncommon cause of proptosis in all age groups with reported incidence varying from 0 to 20%, [208-213] some authors say that the commonest cause of proptosis is medial rectus cysticercosis [208] and the frequency of proptosis among other signs of orbital cysticercosis is around 20-30% preceded by ocular motility restriction (60-

pseudo proptosis, Wegener's granulomatosis and Pfeiffer syndrome.

64%) and followed by diplopia (35-38%) and palpebral ptosis (12-16%). [77]

superioris, and inserts on the superior tarsal plate as well. (See figure 7)

**Figure 7.** Representation of cysticercosis in the Levator palpebrae superioris.

muscles. [218, 219]

It is well known that cysticercosis of the extraocular musculature is rare and within this group the affectation of the eyelid is about 0.6% [77] despite the number of patients reported in the medical literature. [214-217] The Levator palpebrae superioris muscle cysticercosis presenting clinically as ptosis is much more uncommon than other affected extraocular

Cysticercosis of the Levator palpebrae superioris, superior rectus, eyelid or anterior orbit are important differential diagnoses for acquiring ptosis. [219] Features suggestive of Levator

**5.2. Levator palpebrae superioris muscle cysticercosis and palpebral ptosis** 

The Levator palpebrae superioris originates on the lesser wing of the sphenoid bone, just above the optic foramen. It broadens and becomes the Levator aponeurosis. This portion inserts on the skin of the upper eyelid, as well as the superior tarsal plate. It is a skeletal muscle. The superior tarsal muscle, a smooth muscle, is attached to the levator palpebrae

> The superior rectus muscle is a muscle in the orbit. It is one of the extraocular muscles. It is innervated by the superior division of the oculomotor nerve (Cranial Nerve III). In the primary position (looking straight ahead), the superior rectus muscle's primary function is elevation, although it also contributes to intorsion and adduction.

> Extraocular muscle cysticercosis is the most common site of this parasitic disease when involving the orbit. [224]

> In some series of patients with extraocular cysticercosis, the superior rectus muscle is the most commonly affected (33.3%) compared other involved muscles. [77] and clinical features usually are painful unilateral ptosis, inflammation of the upper eyelid and some

restriction on down gaze. Diagnosis is made by neuroimagen studies that show a welldefined ring-enhancing lesion in the superior rectus muscle. [14]

Orbital Cisticercosis. A Challenger for Neurologists,

Ophthalmologists, Neuro-Opthalmologists, and General Practitioners 135

on the basis of the prism and cover test in the diagnostic positions and an examination of ductions and versions. The deviation is greatest on attempts to look downward with the affected eye in abduction (Fig. 9). The unopposed action of the antagonistic superior rectus muscle causes the paretic eye to be incyclotropic and hypertrophic in primary position. When the patient fixates with the paretic eye in primary position, pseudoptosis may occur in the sound eye, creating diagnostic problems. Ocular torticollis is a frequent occurrence but is

not of diagnostic value since the head may be tilted to either side. [197]

**Figure 9.** Representation of T solium cysticercosis in the inferior rectus muscle

and a tiny eccentric hypointense speck suggestive of scolex can be seen.

enhanced studies. [227, 228]

subcutaneous nodules were found.

The inferior rectus muscle was commonly involved in one study. [29] Inferior rectus cysticercosis like other the affected extraocular muscle typically shows fusiform enlargement of its belly and contain a well-defined, spherical cyst with a nodule attached to its wall. This nodule and the enlarged muscle show intense enhancement on the contrast-

In Meher' series [227] about six patients presenting extraocular muscle cysticercosis two of them had inferior rectus muscle involvement and proptosis for the eyes but imaging studies of the brain showed no evidence of cerebral cysticerci in any of the patients and not palpable

Based on the clinical features of patients reported in the medical literature [229] and our observations, the commonest symptom and signs of the inferior rectus cysticercosis are eccentric proptosis and restriction of upward gaze no associated with loss of vision or painful eyes. MRI orbit is the investigation of choice which can reveal a cystic lesion in the belly of inferior rectus muscle, hypointense on T1 and hyperintense on T2 weighted images

Shadangi et al [229] have noticed that the cysticerci of the extraocular muscles travel forward, come to lie in a subconjunctival location and then extrude out spontaneously and

Angotti-Neto et al [224] reported a 38-year-old Brazilian female patient with extraocular muscle enlargement and a small cystic lesion near the insertion of the superior rectus muscle treated with oral prednisone for almost one year due to a non-specific inflammation of right orbit diagnosis later confirmed as superior rectus muscle cysticercosis. That report illustrates the consequences of delayed antiparasitic treatment leading to leading to marked hypertrophy and limitation of infraduction and later an incomplete recovery. It also serves to emphasize that cysticercosis should be included in the differential diagnosis of extraocular muscle enlargement, especially in cases of orbital inflammation non-responsive to corticosteroid treatment. Although the differential diagnosis of extraocular muscle enlargement is extensive, including Graves' orbitopathy, myositis, carotid cavernous fistula, lymphoma, metastatic and primary tumors, when associated with other clinical signs such as lid retraction or pain with eye movement, a correct diagnosis can usually be established. [225] Less frequent conditions include arteriovenous fistulas and malformations and orbital tumors. [226]

**Figure 8.** Cysticercus near to insertion of superior rectus muscle in the eyeball.

### **5.4. Inferior rectus muscle cysticercosis**

The inferior rectus muscle is another muscle in the orbit. (See figure x) It depresses, adducts, and helps extort (rotate laterally) the eye. The inferior rectus muscle is the only muscle that is capable of depressing the pupil when it is in a fully abducted position.

An isolated paralysis of the inferior rectus muscle is often congenital in our experience. However, it may also occur following orbital trauma, especially after fracture of the orbital floor; from vascular disease; or in conjunction with myasthenia. [197] The diagnosis is made on the basis of the prism and cover test in the diagnostic positions and an examination of ductions and versions. The deviation is greatest on attempts to look downward with the affected eye in abduction (Fig. 9). The unopposed action of the antagonistic superior rectus muscle causes the paretic eye to be incyclotropic and hypertrophic in primary position. When the patient fixates with the paretic eye in primary position, pseudoptosis may occur in the sound eye, creating diagnostic problems. Ocular torticollis is a frequent occurrence but is not of diagnostic value since the head may be tilted to either side. [197]

134 Novel Aspects on Cysticercosis and Neurocysticercosis

tumors. [226]

defined ring-enhancing lesion in the superior rectus muscle. [14]

**Figure 8.** Cysticercus near to insertion of superior rectus muscle in the eyeball.

is capable of depressing the pupil when it is in a fully abducted position.

The inferior rectus muscle is another muscle in the orbit. (See figure x) It depresses, adducts, and helps extort (rotate laterally) the eye. The inferior rectus muscle is the only muscle that

An isolated paralysis of the inferior rectus muscle is often congenital in our experience. However, it may also occur following orbital trauma, especially after fracture of the orbital floor; from vascular disease; or in conjunction with myasthenia. [197] The diagnosis is made

**5.4. Inferior rectus muscle cysticercosis** 

restriction on down gaze. Diagnosis is made by neuroimagen studies that show a well-

Angotti-Neto et al [224] reported a 38-year-old Brazilian female patient with extraocular muscle enlargement and a small cystic lesion near the insertion of the superior rectus muscle treated with oral prednisone for almost one year due to a non-specific inflammation of right orbit diagnosis later confirmed as superior rectus muscle cysticercosis. That report illustrates the consequences of delayed antiparasitic treatment leading to leading to marked hypertrophy and limitation of infraduction and later an incomplete recovery. It also serves to emphasize that cysticercosis should be included in the differential diagnosis of extraocular muscle enlargement, especially in cases of orbital inflammation non-responsive to corticosteroid treatment. Although the differential diagnosis of extraocular muscle enlargement is extensive, including Graves' orbitopathy, myositis, carotid cavernous fistula, lymphoma, metastatic and primary tumors, when associated with other clinical signs such as lid retraction or pain with eye movement, a correct diagnosis can usually be established. [225] Less frequent conditions include arteriovenous fistulas and malformations and orbital

**Figure 9.** Representation of T solium cysticercosis in the inferior rectus muscle

The inferior rectus muscle was commonly involved in one study. [29] Inferior rectus cysticercosis like other the affected extraocular muscle typically shows fusiform enlargement of its belly and contain a well-defined, spherical cyst with a nodule attached to its wall. This nodule and the enlarged muscle show intense enhancement on the contrastenhanced studies. [227, 228]

In Meher' series [227] about six patients presenting extraocular muscle cysticercosis two of them had inferior rectus muscle involvement and proptosis for the eyes but imaging studies of the brain showed no evidence of cerebral cysticerci in any of the patients and not palpable subcutaneous nodules were found.

Based on the clinical features of patients reported in the medical literature [229] and our observations, the commonest symptom and signs of the inferior rectus cysticercosis are eccentric proptosis and restriction of upward gaze no associated with loss of vision or painful eyes. MRI orbit is the investigation of choice which can reveal a cystic lesion in the belly of inferior rectus muscle, hypointense on T1 and hyperintense on T2 weighted images and a tiny eccentric hypointense speck suggestive of scolex can be seen.

Shadangi et al [229] have noticed that the cysticerci of the extraocular muscles travel forward, come to lie in a subconjunctival location and then extrude out spontaneously and

they have adopted a policy to wait and watch in these cases. They avoid giving steroids and anthelmintic as these suppress inflammation and delay the movement of the cyst outward, and hence, its extrusion. Because delayed treatment can favored chronic inflammatory process, associated fibrosis and permanent damage on the physiology of the muscles, in our opinion, each patient should be assessed individually and a decision should be taken accordingly.

Orbital Cisticercosis. A Challenger for Neurologists,

Ophthalmologists, Neuro-Opthalmologists, and General Practitioners 137

during neurological examinations, the superior oblique is tested by having the patient look inwards and downwards, testing only the depressing action of the muscle. This is a source of confusion on the subject as although clinical testing asks the patient to adduct and

The great importance of intorsion and extorsion produced by the two oblique muscles can only be understood when it is considered with regards to the other muscle actions present. The two obliques prevent the eye from rotating about its long axis (retina to pupil) when the superior and inferior rectus muscles contract. This is because the orbit does not face directly forwards- the centre-line of the orbit is a little over 20 degrees out from the mid-line. But because the eyes do face forwards, when acting alone, as well as making the eye look up, superior rectus causes it to rotate slightly about the long axis, so the top of the eye moves medially (intorsion). Similarly, in addition to making the eye look down, inferior rectus would cause the eye to rotate about the long axis so the top of the eye moves slightly laterally (extortion), if acting alone. Clearly this is undesirable as our vision would rotate when we looked up and down. For this reason, these two rectus muscles work in conjunction with the two obliques. When acting alone, superior oblique causes intorsion, inferior oblique, and extorsion. Hence, when inferior rectus contracts so we look down, superior oblique has also contracted to prevent extorsion of the eye, and when superior rectus contracts so we look up, inferior oblique contracts to prevent intorsion, thus the undesired rotatory actions of the inferior and superior rectus about the long axis of the eye are cancelled out. This keeps our

In one of the large series of patients presenting extraocular muscle cysticercosis (n=35) the average age was 19.6 years, no differences between female and males were found and the most common clinical feature was restricted ocular motility with diplopia and recurrent pain and redness. [194] In the superior oblique muscle cysticercosis are kept the same demographic and clinical feature described for other extraocular muscles. However, the lateral rectus, medial rectus and the superior oblique were affected to a greater extent and in all cases the extraocular muscle cysticercosis can be suspected in cases of acquired ocular motility disorder with recurrent ocular congestion. Perhaps the most interesting aspect of superior oblique cysticercosis is its clinical presentation as: Brown syndrome and Canine

Brown syndrome is defined as an ocular motility disorder, characterized by an inability to elevate the adducted eye actively or passively. In 1950, Harold W. Brown first published on an unusual motility disorder, characterized by the following: limited elevation in adduction; divergence in straight upgaze (V-pattern); same degree of limitation on versions, ductions, and forced ductions; widening of the palpebral fissure on adduction; normal or near normal elevation in abduction; restricted forced ductions to elevation in adduction; and

Diplopia may occur when the patient looks up and to the contralateral side of the affected eye. Patients with congenital Brown syndrome rarely complain of diplopia, because most patients have developed suppression. The most common signs of Brown syndrome include:

depress the eye, anatomically the muscle depresses and abducts it.

vision horizontally level, irrespective of eye position in the orbit.

compensatory chin elevation for binocular fusion.

tooth syndrome.

Isolated inferior rectus muscle palsy resulting from a nuclear third nerve lesion as the initial manifestation of multiple sclerosis has been reported in the medical literature. [230]

## **5.5. Superior oblique muscle cysticercosis**

The superior oblique muscle, or obliquus oculi superior, is a fusiform muscle originating in the upper, medial side of the orbit (i.e. from beside the nose) which abducts, depresses and internally rotates the eye. It is the only extraocular muscle innervated by the trochlear nerve (the fourth cranial nerve).

The primary (main) action of the superior oblique muscle is intorsion (internal rotation), the secondary action is depression (primarily in the adducted position) and the tertiary action is abduction (lateral rotation).

**Figure 10.** Superior oblique myocysticercosis

The depressing action of the superior oblique (making the eye look down towards the mouth) is most effective when the eye is in an adducted position. This is because as the eye is abducted (looks laterally), the contribution made by superior oblique to depression of the eye decreases, as the inferior rectus muscle causes this movement more directly and powerfully. The main muscle for abduction is the lateral rectus, so although superior oblique contributes to a downwards and lateral eye movement, testing this motion would not be specific enough as inferior and lateral recti muscles would also be tested. Therefore, during neurological examinations, the superior oblique is tested by having the patient look inwards and downwards, testing only the depressing action of the muscle. This is a source of confusion on the subject as although clinical testing asks the patient to adduct and depress the eye, anatomically the muscle depresses and abducts it.

136 Novel Aspects on Cysticercosis and Neurocysticercosis

**5.5. Superior oblique muscle cysticercosis** 

accordingly.

(the fourth cranial nerve).

abduction (lateral rotation).

**Figure 10.** Superior oblique myocysticercosis

they have adopted a policy to wait and watch in these cases. They avoid giving steroids and anthelmintic as these suppress inflammation and delay the movement of the cyst outward, and hence, its extrusion. Because delayed treatment can favored chronic inflammatory process, associated fibrosis and permanent damage on the physiology of the muscles, in our opinion, each patient should be assessed individually and a decision should be taken

Isolated inferior rectus muscle palsy resulting from a nuclear third nerve lesion as the initial

The superior oblique muscle, or obliquus oculi superior, is a fusiform muscle originating in the upper, medial side of the orbit (i.e. from beside the nose) which abducts, depresses and internally rotates the eye. It is the only extraocular muscle innervated by the trochlear nerve

The primary (main) action of the superior oblique muscle is intorsion (internal rotation), the secondary action is depression (primarily in the adducted position) and the tertiary action is

The depressing action of the superior oblique (making the eye look down towards the mouth) is most effective when the eye is in an adducted position. This is because as the eye is abducted (looks laterally), the contribution made by superior oblique to depression of the eye decreases, as the inferior rectus muscle causes this movement more directly and powerfully. The main muscle for abduction is the lateral rectus, so although superior oblique contributes to a downwards and lateral eye movement, testing this motion would not be specific enough as inferior and lateral recti muscles would also be tested. Therefore,

manifestation of multiple sclerosis has been reported in the medical literature. [230]

The great importance of intorsion and extorsion produced by the two oblique muscles can only be understood when it is considered with regards to the other muscle actions present. The two obliques prevent the eye from rotating about its long axis (retina to pupil) when the superior and inferior rectus muscles contract. This is because the orbit does not face directly forwards- the centre-line of the orbit is a little over 20 degrees out from the mid-line. But because the eyes do face forwards, when acting alone, as well as making the eye look up, superior rectus causes it to rotate slightly about the long axis, so the top of the eye moves medially (intorsion). Similarly, in addition to making the eye look down, inferior rectus would cause the eye to rotate about the long axis so the top of the eye moves slightly laterally (extortion), if acting alone. Clearly this is undesirable as our vision would rotate when we looked up and down. For this reason, these two rectus muscles work in conjunction with the two obliques. When acting alone, superior oblique causes intorsion, inferior oblique, and extorsion. Hence, when inferior rectus contracts so we look down, superior oblique has also contracted to prevent extorsion of the eye, and when superior rectus contracts so we look up, inferior oblique contracts to prevent intorsion, thus the undesired rotatory actions of the inferior and superior rectus about the long axis of the eye are cancelled out. This keeps our vision horizontally level, irrespective of eye position in the orbit.

In one of the large series of patients presenting extraocular muscle cysticercosis (n=35) the average age was 19.6 years, no differences between female and males were found and the most common clinical feature was restricted ocular motility with diplopia and recurrent pain and redness. [194] In the superior oblique muscle cysticercosis are kept the same demographic and clinical feature described for other extraocular muscles. However, the lateral rectus, medial rectus and the superior oblique were affected to a greater extent and in all cases the extraocular muscle cysticercosis can be suspected in cases of acquired ocular motility disorder with recurrent ocular congestion. Perhaps the most interesting aspect of superior oblique cysticercosis is its clinical presentation as: Brown syndrome and Canine tooth syndrome.

Brown syndrome is defined as an ocular motility disorder, characterized by an inability to elevate the adducted eye actively or passively. In 1950, Harold W. Brown first published on an unusual motility disorder, characterized by the following: limited elevation in adduction; divergence in straight upgaze (V-pattern); same degree of limitation on versions, ductions, and forced ductions; widening of the palpebral fissure on adduction; normal or near normal elevation in abduction; restricted forced ductions to elevation in adduction; and compensatory chin elevation for binocular fusion.

Diplopia may occur when the patient looks up and to the contralateral side of the affected eye. Patients with congenital Brown syndrome rarely complain of diplopia, because most patients have developed suppression. The most common signs of Brown syndrome include:

supranasal orbital pain, tenderness. Limited elevation in adduction, an invariable sign, is the hallmark of Brown syndrome and pain that is associated with this ocular movement. The canine tooth syndrome is an ocular motility disorder comprising ipsilateral Brown's syndrome and superior oblique muscle dysfunction. Ocular motility shows ipsilateral deficit of elevation and depression, maximum in an adducted position. Typically this follows a dog bite (hence the name) that damages the trochlea and superior oblique muscle concurrently but it can be caused by trauma to the trochlear area, producing a "double Brown syndrome"; secondary to strengthening the superior oblique along with a residual superior oblique palsy, or a combination of local trauma to the trochlea causing restriction to upgaze along with closed head trauma producing a trochlear nerve disorder. Trochlear nerve palsy is the most common cause of vertical diplopia then apart from periocular pain, and lid swelling, an upgaze vertical diplopia can be present. [231-242]. Superior rectus muscle cysticercosis should be considered in the differential diagnosis of acquired motility disorder.

Orbital Cisticercosis. A Challenger for Neurologists,

Ophthalmologists, Neuro-Opthalmologists, and General Practitioners 139

potential points of origin. Because the inferior oblique is innervated near its middle, it may

The inferior oblique muscle is the only muscle that is capable of elevating the eye when it is in a fully adducted position. While commonly affected by palsies of the inferior division of the oculomotor nerve, isolated palsies of the inferior oblique (without affecting other functions of the oculomotor nerve) are quite rare and can be confused with Brown

In a series of 25 patients with inferior oblique palsy studied by Pollard [245], all presented with a head tilt to the side of the paretic muscle. None of these patients complained of tilting images, but incyclotorsion was measurable in all cases. The most important conclusion from this study is that inferior oblique palsy is a benign entity, with none of these patients having

While many vertical deviations that appear to be due to an inferior oblique palsy based on the results of the three-step test may be caused by inferior oblique weakness, skew deviation should also be considered in any patient with a history of head trauma, or other neurological findings. The cyclotorsion observed in inferior oblique palsy is opposite that seen by the ocular tilt reaction, and differentiates the two entities clinically. We postulate that these deviations are caused by damage to the otolithic projections that correspond to those from the ipsilateral posterior semicircular canal (on the side of the hypotropic eye).

**Figure 11.** Illustration of inferior oblique muscle cysticercosis close to its insertion in the eyeball.

The common causes of inferior oblique muscle dysfunctions are facial trauma, endoscopic ethmoidectomy, superior oblique muscle palsy (overaction of the inferior oblique muscle), unilateral inferior oblique anterior transposition. [24, 247, 248] Overaction of the inferior oblique muscle is manifest by over the elevation of the adducted eye. It is a common enigma

be weakened either proximal or distal to its point of innervation.

a brain tumor, myasthenia gravis or cysticercosis.

syndrome.

[246]

Lee and O'Halloran [239] described the case of a 5-year-old girl traumatized from a dog bite to the superior aspect of the orbit in the right eye. The dog's canine tooth penetrated deep into the posterior orbit and severed the attachment of the superior oblique muscle of the globe posterior to the trochlea leading to the clinical presentation before-cited.

The acquired Brown syndrome is known to occur after trauma, iatrogenic events, cysts of superior oblique muscle, tenosynovitis, and systemic lupus erythematosus. [238, 243,244] Although surgical excision and reconstruction of the superior oblique tendon has been reported [230] this aspect will be discussed under the subchapter of treatment.

Rao et al [238] reported seven patients presenting Brown syndrome secondary to superior oblique muscle cysticercosis. All patients had the ocular motility limitation, ie, limitation of elevation in adduction (characteristic of Brown syndrome) and associated pain and swelling in the superior nasal orbit.

## **5.6. Inferior oblique muscle cysticercosis**

The inferior oblique muscle is a thin, 36 mm long narrow muscle placed near the anterior margin of the floor of the orbit. It originates a few millimeters behind the medial end of the inferior orbital rim just lateral to the lacrimal fossa and proceeds posteriorly and temporally at an angle of 51 degrees with the frontal plane passing beneath the inferior rectus (between the inferior rectus and the floor of the orbit) and inserting posterior to the equator on the inferior and lateral aspect of the globe. [204] Its actions are extorsion, elevation and abduction of the eye. The primary action is extorsion; secondary action is elevation; tertiary action is abduction (i.e. It extorts the eye and moves it upward and outwards). The field of maximal inferior oblique elevation is in the adducted position.

This muscle receives its innervation on its upper surface at the point where it passes beneath the lateral border of the inferior rectus, approximately 12 mm posterior to the lateral corner of the insertion of the inferior rectus. The inferior oblique muscle is unique in its anatomic relationships. This muscle behaves as though it has two potential insertions and two potential points of origin. Because the inferior oblique is innervated near its middle, it may be weakened either proximal or distal to its point of innervation.

138 Novel Aspects on Cysticercosis and Neurocysticercosis

in the superior nasal orbit.

**5.6. Inferior oblique muscle cysticercosis** 

maximal inferior oblique elevation is in the adducted position.

supranasal orbital pain, tenderness. Limited elevation in adduction, an invariable sign, is the hallmark of Brown syndrome and pain that is associated with this ocular movement. The canine tooth syndrome is an ocular motility disorder comprising ipsilateral Brown's syndrome and superior oblique muscle dysfunction. Ocular motility shows ipsilateral deficit of elevation and depression, maximum in an adducted position. Typically this follows a dog bite (hence the name) that damages the trochlea and superior oblique muscle concurrently but it can be caused by trauma to the trochlear area, producing a "double Brown syndrome"; secondary to strengthening the superior oblique along with a residual superior oblique palsy, or a combination of local trauma to the trochlea causing restriction to upgaze along with closed head trauma producing a trochlear nerve disorder. Trochlear nerve palsy is the most common cause of vertical diplopia then apart from periocular pain, and lid swelling, an upgaze vertical diplopia can be present. [231-242]. Superior rectus muscle cysticercosis

should be considered in the differential diagnosis of acquired motility disorder.

globe posterior to the trochlea leading to the clinical presentation before-cited.

reported [230] this aspect will be discussed under the subchapter of treatment.

Lee and O'Halloran [239] described the case of a 5-year-old girl traumatized from a dog bite to the superior aspect of the orbit in the right eye. The dog's canine tooth penetrated deep into the posterior orbit and severed the attachment of the superior oblique muscle of the

The acquired Brown syndrome is known to occur after trauma, iatrogenic events, cysts of superior oblique muscle, tenosynovitis, and systemic lupus erythematosus. [238, 243,244] Although surgical excision and reconstruction of the superior oblique tendon has been

Rao et al [238] reported seven patients presenting Brown syndrome secondary to superior oblique muscle cysticercosis. All patients had the ocular motility limitation, ie, limitation of elevation in adduction (characteristic of Brown syndrome) and associated pain and swelling

The inferior oblique muscle is a thin, 36 mm long narrow muscle placed near the anterior margin of the floor of the orbit. It originates a few millimeters behind the medial end of the inferior orbital rim just lateral to the lacrimal fossa and proceeds posteriorly and temporally at an angle of 51 degrees with the frontal plane passing beneath the inferior rectus (between the inferior rectus and the floor of the orbit) and inserting posterior to the equator on the inferior and lateral aspect of the globe. [204] Its actions are extorsion, elevation and abduction of the eye. The primary action is extorsion; secondary action is elevation; tertiary action is abduction (i.e. It extorts the eye and moves it upward and outwards). The field of

This muscle receives its innervation on its upper surface at the point where it passes beneath the lateral border of the inferior rectus, approximately 12 mm posterior to the lateral corner of the insertion of the inferior rectus. The inferior oblique muscle is unique in its anatomic relationships. This muscle behaves as though it has two potential insertions and two The inferior oblique muscle is the only muscle that is capable of elevating the eye when it is in a fully adducted position. While commonly affected by palsies of the inferior division of the oculomotor nerve, isolated palsies of the inferior oblique (without affecting other functions of the oculomotor nerve) are quite rare and can be confused with Brown syndrome.

In a series of 25 patients with inferior oblique palsy studied by Pollard [245], all presented with a head tilt to the side of the paretic muscle. None of these patients complained of tilting images, but incyclotorsion was measurable in all cases. The most important conclusion from this study is that inferior oblique palsy is a benign entity, with none of these patients having a brain tumor, myasthenia gravis or cysticercosis.

While many vertical deviations that appear to be due to an inferior oblique palsy based on the results of the three-step test may be caused by inferior oblique weakness, skew deviation should also be considered in any patient with a history of head trauma, or other neurological findings. The cyclotorsion observed in inferior oblique palsy is opposite that seen by the ocular tilt reaction, and differentiates the two entities clinically. We postulate that these deviations are caused by damage to the otolithic projections that correspond to those from the ipsilateral posterior semicircular canal (on the side of the hypotropic eye). [246]

**Figure 11.** Illustration of inferior oblique muscle cysticercosis close to its insertion in the eyeball.

The common causes of inferior oblique muscle dysfunctions are facial trauma, endoscopic ethmoidectomy, superior oblique muscle palsy (overaction of the inferior oblique muscle), unilateral inferior oblique anterior transposition. [24, 247, 248] Overaction of the inferior oblique muscle is manifest by over the elevation of the adducted eye. It is a common enigma in the field of ocular motility and it may occur secondary to a weak contralateral superior rectus muscle or a weak ipsilateral superior oblique muscle. [248-250]

Orbital Cisticercosis. A Challenger for Neurologists,

Ophthalmologists, Neuro-Opthalmologists, and General Practitioners 141

found in skeletal muscle. There are two muscle fiber layers in the medial and lateral recti. The shorter orbital layer inserts in the muscle pulley, and the longer global fibers insert into sclera at the muscle's *insertion*. The muscle fibers are long, traversing the entire length of the muscle, or in some cases, nearly so. The medial and lateral extraocular muscles have the lowest innervation ratio of any of the muscles of the body; that is, they have the *most* nerve fibers per muscle fiber which support the stringent requirements of accuracy of fixation and smoothness of following required to support a visual apparatus capable of both rapid, accurate movement and sustained fixation. There is evidence, not always corroborated but accumulating nonetheless, showing that the extraocular muscles participate in proprioception. Muscle spindles and other muscle sensors communicate by means of an *inflow* mechanism which is functional but apparently less powerful than the *outflow* 

mechanism generated from stimuli arising in the retina. [251]

**Figure 12.** Illustration of the lateral rectus muscle myocysticercosis

useful to confirm the final diagnosis. [251]

Lateral rectus palsy is commonly seen in patients presenting intracranial hypertension due to NCC, because abducen nerves has the longest pathway along to the subarachnoid space and it can be damaged by raise intracranial pressure without focalizing the intracranial lesion. Like other extraocular muscles first clinical symptom is diplopia (horizontal) To differentiate horizontal diplopia secondary to abducen palsy from horizontal diplopia secondary to lateral rectus muscle cysticercosis is an easy task if other symptoms and signs of intracranial hypertension of orbital cysticercosis are present. In our series intracranial hypertension is most commonly seen in patients with intraventricular and subarachnoid NCC whom a complaint of epilepsy, headache, nausea, vomiting, and cognitive dysfunction and they can present meningeal sings and focal neurological signs. Patients with lateral rectus cysticercosis also complaint of proptosis of the affected eyes, painful eye movements and decrease visual acuity due to optic nerve damage. Imagenology studies are extremely

Based on our experience and an extensive review of the medical literature we conclude that cysticercosis of the inferior oblique muscle practically does not exist in human beings compare with other presentations. Despite the inferior oblique muscle has the same change to be infected by T solium like other extraocular muscles, we think it is rarer than the other because the inferior oblique is unique among the extraocular muscles in that, in many cases, weakening of this muscle, even with extensive surgery, seems to have relatively little effect on the movement of the globe or alignment of the eyes. Therefore isolated cysticercotic lesion on the inferior oblique muscle can be asymptomatic. Even in patients presenting a relatively uncommon inferior oblique paresis due to other causes, strabismus is much less than would occur after paresis of any of the other muscles. Effective weakening of this muscle could be made more difficult because of the unique anatomy. Likewise neurologically, the muscle innervation by the inferior branch of cranial nerve III makes isolated paralysis extremely uncommon. [250]

Of all the extraocular muscles supplied by the oculomotor nerve, the inferior oblique muscle is less likely to become paralyzed. The onset is usually congenital but the trauma has been mentioned as a cause. In primary position the affected eye may be hypotropic or the unaffected eye hypertrophic, depending on whether the patient fixates with the nonparalyzed or paralyzed eye. The greatest deviation occurs when the patient attempts to elevate the adducted paretic eye. Overreaction of the unopposed ipsilateral superior oblique muscle causes incyclotropia. In all patients whom we have evaluated, onset was congenital. As in the case of superior oblique paralysis, the anomalous head posture is more characteristic than in paralyses of the vertical rectus muscles. As a rule the head is inclined toward the paralyzed side, and the face is turned to the uninvolved side, but there are exceptions. The Bielschowsky head tilt test is positive on tilting the head to the normal side. The forced duction test is necessary in making this diagnosis, since the prevalence of Brown syndrome is far greater than paralysis of the inferior oblique muscle and since the defect of ocular motility is clinically similar. However, with Brown syndrome the involved eye is frequently depressed more severely in adduction than it is with inferior oblique paralysis.

#### **5.7. Lateral rectus muscle cysticercosis**

Each rectus muscle inserts at a different distance from the limbus. The lateral rectus muscle is one of six extraocular muscles that control the movements of the eye (abduction in this case) and the only muscle innervated by the abducens nerve, cranial nerve VI. Its function is to bring the pupil away from the midline of the body. It is tested clinically by asking the patient to look laterally.

As we before-mentioned the extraocular muscles have differences undoubtedly related to their very specialized function. Both skeletal and extraocular muscles have several types of twitch fibers, but the extraocular muscles are unique, having tonically contracting fibers not found in skeletal muscle. There are two muscle fiber layers in the medial and lateral recti. The shorter orbital layer inserts in the muscle pulley, and the longer global fibers insert into sclera at the muscle's *insertion*. The muscle fibers are long, traversing the entire length of the muscle, or in some cases, nearly so. The medial and lateral extraocular muscles have the lowest innervation ratio of any of the muscles of the body; that is, they have the *most* nerve fibers per muscle fiber which support the stringent requirements of accuracy of fixation and smoothness of following required to support a visual apparatus capable of both rapid, accurate movement and sustained fixation. There is evidence, not always corroborated but accumulating nonetheless, showing that the extraocular muscles participate in proprioception. Muscle spindles and other muscle sensors communicate by means of an *inflow* mechanism which is functional but apparently less powerful than the *outflow*  mechanism generated from stimuli arising in the retina. [251]

**Figure 12.** Illustration of the lateral rectus muscle myocysticercosis

140 Novel Aspects on Cysticercosis and Neurocysticercosis

isolated paralysis extremely uncommon. [250]

**5.7. Lateral rectus muscle cysticercosis** 

patient to look laterally.

in the field of ocular motility and it may occur secondary to a weak contralateral superior

Based on our experience and an extensive review of the medical literature we conclude that cysticercosis of the inferior oblique muscle practically does not exist in human beings compare with other presentations. Despite the inferior oblique muscle has the same change to be infected by T solium like other extraocular muscles, we think it is rarer than the other because the inferior oblique is unique among the extraocular muscles in that, in many cases, weakening of this muscle, even with extensive surgery, seems to have relatively little effect on the movement of the globe or alignment of the eyes. Therefore isolated cysticercotic lesion on the inferior oblique muscle can be asymptomatic. Even in patients presenting a relatively uncommon inferior oblique paresis due to other causes, strabismus is much less than would occur after paresis of any of the other muscles. Effective weakening of this muscle could be made more difficult because of the unique anatomy. Likewise neurologically, the muscle innervation by the inferior branch of cranial nerve III makes

Of all the extraocular muscles supplied by the oculomotor nerve, the inferior oblique muscle is less likely to become paralyzed. The onset is usually congenital but the trauma has been mentioned as a cause. In primary position the affected eye may be hypotropic or the unaffected eye hypertrophic, depending on whether the patient fixates with the nonparalyzed or paralyzed eye. The greatest deviation occurs when the patient attempts to elevate the adducted paretic eye. Overreaction of the unopposed ipsilateral superior oblique muscle causes incyclotropia. In all patients whom we have evaluated, onset was congenital. As in the case of superior oblique paralysis, the anomalous head posture is more characteristic than in paralyses of the vertical rectus muscles. As a rule the head is inclined toward the paralyzed side, and the face is turned to the uninvolved side, but there are exceptions. The Bielschowsky head tilt test is positive on tilting the head to the normal side. The forced duction test is necessary in making this diagnosis, since the prevalence of Brown syndrome is far greater than paralysis of the inferior oblique muscle and since the defect of ocular motility is clinically similar. However, with Brown syndrome the involved eye is frequently depressed more severely in adduction than it is with inferior oblique paralysis.

Each rectus muscle inserts at a different distance from the limbus. The lateral rectus muscle is one of six extraocular muscles that control the movements of the eye (abduction in this case) and the only muscle innervated by the abducens nerve, cranial nerve VI. Its function is to bring the pupil away from the midline of the body. It is tested clinically by asking the

As we before-mentioned the extraocular muscles have differences undoubtedly related to their very specialized function. Both skeletal and extraocular muscles have several types of twitch fibers, but the extraocular muscles are unique, having tonically contracting fibers not

rectus muscle or a weak ipsilateral superior oblique muscle. [248-250]

Lateral rectus palsy is commonly seen in patients presenting intracranial hypertension due to NCC, because abducen nerves has the longest pathway along to the subarachnoid space and it can be damaged by raise intracranial pressure without focalizing the intracranial lesion. Like other extraocular muscles first clinical symptom is diplopia (horizontal) To differentiate horizontal diplopia secondary to abducen palsy from horizontal diplopia secondary to lateral rectus muscle cysticercosis is an easy task if other symptoms and signs of intracranial hypertension of orbital cysticercosis are present. In our series intracranial hypertension is most commonly seen in patients with intraventricular and subarachnoid NCC whom a complaint of epilepsy, headache, nausea, vomiting, and cognitive dysfunction and they can present meningeal sings and focal neurological signs. Patients with lateral rectus cysticercosis also complaint of proptosis of the affected eyes, painful eye movements and decrease visual acuity due to optic nerve damage. Imagenology studies are extremely useful to confirm the final diagnosis. [251]

With acquired sixth nerve paralysis it is essential to determine whether the restriction of abduction is caused by paralysis of the lateral rectus muscle, contracture of the medial rectus muscle or a combination of both conditions. The estimation of generating muscle force determines whether residual lateral rectus muscle function is present. [251]

Orbital Cisticercosis. A Challenger for Neurologists,

Ophthalmologists, Neuro-Opthalmologists, and General Practitioners 143

Extraocular cysticercosis associated to disseminate cysticercosis including NCC can be seen. It was communicated by Patwardhan and Bhatti. [111] in India. They reported a young male patient presented with a complaint of blurring of vision in the left eye for a few days. Funduscopy examination showed vitreous haze, localized in the lower half, of the vitreous. A clearly defined, spherical, white mass somewhat resembling a dislocated lens, was seen in the vitreous body and anterior to the retina, freely mobile, lying in the lower temporal quadrant. This mass showed occasional contractile waves passing through it. Systemic examination showed the presence of subcutaneous nodules on the scalp , and neck. CT Scan confirmed multiple neurocysticercosis cysts involving superior and medial rectus bilaterally

One or more extraocular muscles may be simultaneously involved, although a propensity for involvement of the superior muscle complex and the lateral rectus muscles has been most commonly reported. [90, 194] As we explained before, according to muscle involved, patients will present different clinical manifestations such as; Brown syndrome, Canine tooth syndrome, Duane retraction, Skew deviation, overreaction, different types of diplopia, painful proptosis. Also acquired strabismus, recurrent redness and some of the clinical signs

The medial rectus muscle is a muscle in the orbit. The medial rectus is said to insert in the

As with most of the muscles of the orbit, it is innervated by the inferior division of the

This muscle shares an origin with several other extrinsic eye muscles, the annulus

It is the largest of the extraocular muscles and its only action is adduction of the eyeball. Its function is to bring the pupil closer to the midline of the body. It is tested clinically by

In our experience, an isolated paralysis of the medial rectus muscle due to cysticercosis without involvement of other muscles is very uncommon. In this type of weakness or paralysis the greatest defect of ocular motility occurs when the affected eye goes to adduction position. In the differential diagnosis of an isolated medial rectus paralysis,

Cysticercosis as a cause of medial rectus myositis have been reported by others [197, 208, 218, 221, 252- 256] most of patients presented headache, orthotropia in primary gaze, painful drooping of the eyelid, decrease visual acuity, and on examination of the ocular motility, abduction deficit with the resulting horizontal diplopia is found. Sometimes the head of patients turns to the non-paretic side to allow them to attain single binocular vision. The most common extra ocular muscle to get involved in cysticercosis is the medial rectus. [197]

in patients with orbital cysticercosis can be observed. [70]

**5.8. Medial rectus muscle cysticercosis** 

normal eye 5.5 mm from the limbus.

oculomotor nerve (Cranial Nerve III).

tendineus, or common tendon.

asking the patient to look medially.

internuclear ophthalmoplegia is listed.

and right lateral rectus.

To identify the cause of horizontal diplopia due to a lateral rectus muscle lesion or due to abducens palsy, imagenology studies are mandatory. Below, our readership can see the main differences in two well documented patients.

Abducens palsy is commonly seen as part of the clinical feature of intracranial hypertension because its long pathway across to the subarachnoid space but it is non useful as a focal neurological sing. Another multifactorial cause of abducens palsy is MÖBIUS syndrome which is characterized by congenital bilateral abducens paralysis associated with facial diplegia and microglossia.

In May 2012, the previous patient came to the department of ophthalmology in Nelson Mandela Academic Hospital complaining of pain on the left eye, horizontal diplopia, poor vision, and proptosis of the left eye. She was assessed by two of us (MCSC and HFS). Apart from CT scan findings, the ELISA test for Ig G antigen (*Tenia solium)* in the cerebro-spinal fluid was positive. She is still under medical treatment with prednisone and albendazol and responding well to the medications.

**Figure 13.** CT scan axial view (1) shows proptosis of the left eyes and lateral rectus myocysticercosis. Intramuscular cyst with "scolex" within is seen. Lateral view (2) shows enlargement of the lateral rectus muscle and an associated intraparenchymal neurocysticercosis (calcified lesions).

Another two patients from our series presented horizontal diplopia secondary to abducens palsy, headache and chronic seizure disorder came to Neurology clinic and CT scans of the brain confirmed intraventricular NCC (2) and subarachnoid NCC (1), in both patients ELISA tests for serum antigen and Western blot were positive for T solium.

Extraocular cysticercosis associated to disseminate cysticercosis including NCC can be seen. It was communicated by Patwardhan and Bhatti. [111] in India. They reported a young male patient presented with a complaint of blurring of vision in the left eye for a few days. Funduscopy examination showed vitreous haze, localized in the lower half, of the vitreous. A clearly defined, spherical, white mass somewhat resembling a dislocated lens, was seen in the vitreous body and anterior to the retina, freely mobile, lying in the lower temporal quadrant. This mass showed occasional contractile waves passing through it. Systemic examination showed the presence of subcutaneous nodules on the scalp , and neck. CT Scan confirmed multiple neurocysticercosis cysts involving superior and medial rectus bilaterally and right lateral rectus.

One or more extraocular muscles may be simultaneously involved, although a propensity for involvement of the superior muscle complex and the lateral rectus muscles has been most commonly reported. [90, 194] As we explained before, according to muscle involved, patients will present different clinical manifestations such as; Brown syndrome, Canine tooth syndrome, Duane retraction, Skew deviation, overreaction, different types of diplopia, painful proptosis. Also acquired strabismus, recurrent redness and some of the clinical signs in patients with orbital cysticercosis can be observed. [70]

## **5.8. Medial rectus muscle cysticercosis**

142 Novel Aspects on Cysticercosis and Neurocysticercosis

diplegia and microglossia.

responding well to the medications.

main differences in two well documented patients.

With acquired sixth nerve paralysis it is essential to determine whether the restriction of abduction is caused by paralysis of the lateral rectus muscle, contracture of the medial rectus muscle or a combination of both conditions. The estimation of generating muscle force

To identify the cause of horizontal diplopia due to a lateral rectus muscle lesion or due to abducens palsy, imagenology studies are mandatory. Below, our readership can see the

Abducens palsy is commonly seen as part of the clinical feature of intracranial hypertension because its long pathway across to the subarachnoid space but it is non useful as a focal neurological sing. Another multifactorial cause of abducens palsy is MÖBIUS syndrome which is characterized by congenital bilateral abducens paralysis associated with facial

In May 2012, the previous patient came to the department of ophthalmology in Nelson Mandela Academic Hospital complaining of pain on the left eye, horizontal diplopia, poor vision, and proptosis of the left eye. She was assessed by two of us (MCSC and HFS). Apart from CT scan findings, the ELISA test for Ig G antigen (*Tenia solium)* in the cerebro-spinal fluid was positive. She is still under medical treatment with prednisone and albendazol and

**Figure 13.** CT scan axial view (1) shows proptosis of the left eyes and lateral rectus myocysticercosis. Intramuscular cyst with "scolex" within is seen. Lateral view (2) shows enlargement of the lateral rectus

Another two patients from our series presented horizontal diplopia secondary to abducens palsy, headache and chronic seizure disorder came to Neurology clinic and CT scans of the brain confirmed intraventricular NCC (2) and subarachnoid NCC (1), in both patients ELISA

muscle and an associated intraparenchymal neurocysticercosis (calcified lesions).

tests for serum antigen and Western blot were positive for T solium.

determines whether residual lateral rectus muscle function is present. [251]

The medial rectus muscle is a muscle in the orbit. The medial rectus is said to insert in the normal eye 5.5 mm from the limbus.

As with most of the muscles of the orbit, it is innervated by the inferior division of the oculomotor nerve (Cranial Nerve III).

This muscle shares an origin with several other extrinsic eye muscles, the annulus tendineus, or common tendon.

It is the largest of the extraocular muscles and its only action is adduction of the eyeball. Its function is to bring the pupil closer to the midline of the body. It is tested clinically by asking the patient to look medially.

In our experience, an isolated paralysis of the medial rectus muscle due to cysticercosis without involvement of other muscles is very uncommon. In this type of weakness or paralysis the greatest defect of ocular motility occurs when the affected eye goes to adduction position. In the differential diagnosis of an isolated medial rectus paralysis, internuclear ophthalmoplegia is listed.

Cysticercosis as a cause of medial rectus myositis have been reported by others [197, 208, 218, 221, 252- 256] most of patients presented headache, orthotropia in primary gaze, painful drooping of the eyelid, decrease visual acuity, and on examination of the ocular motility, abduction deficit with the resulting horizontal diplopia is found. Sometimes the head of patients turns to the non-paretic side to allow them to attain single binocular vision. The most common extra ocular muscle to get involved in cysticercosis is the medial rectus. [197]

Isolated medial rectus cysticercosis may be associated with ptosis and upper lid retraction on attempted adduction (aberrant regeneration) in patients with partial third nerve paralysis. Medial rectus cysticercosis must be distinguished from internuclear ophthalmoplegia (INO), caused by lesions in the medial longitudinal fasciculus. In this condition unilateral or bilateral limitation of adduction is associated with nystagmus of the abducting eye. Convergence may or may not be normal and we will discuss briefly about this matter below. A clinical picture similar isolated media rectus cysticercosis or INO can be caused by myasthenia gravis but a Tensilon test is diagnostic. Restriction of adduction may be caused by an excessively resected lateral rectus muscle.

Orbital Cisticercosis. A Challenger for Neurologists,

Ophthalmologists, Neuro-Opthalmologists, and General Practitioners 145

underlying muscle. [215] The potential risk of damage to adjacent tissue and adhesion from surgical exploration should not be taken lightly, particularly when effective medical therapy is available. As a general consent medical treatment with albendazol and prednisone is the best choice taken by most of the authors and recommended duration of treatment varies from a few days to up to 6 weeks. [29, 95,155, 194,197, 204- 208, 218,219, 220, 223, 224, 227-

In some, cyst elimination rates were more than 90%, and time to recovery of ocular motility

Although oral albendazole and prednisone are efficient, a long history of disease can lead to important residual ocular motility restriction [220] and some author found that oral albendazole and prednisone are not effective thus their role in ocular cysticercosis need to

In the next figure we summarize the frequency of affected extraocular muscle by

In some, cyst elimination rates were more than 90%, and time to recovery of ocular motility

Although oral albendazole and prednisone are efficient, a long history of disease can lead to important residual ocular motility restriction [220] and some author found that oral albendazole and prednisone are not effective thus their role in ocular cysticercosis need to

In the next figure we summarize the frequency of affected extraocular muscle by

**Figure 15.** Distribution of extraocular myocysticercosis in the orbital cavity.

232,237-240, 242, 252, 253,255, 264-266]

be studied. [256]

be studied. [256]

cysticercosis.

ranged between 0.5 and 35 month. [136, 155, 265]

ranged between 0.5 and 35 month. [136, 155, 265]

cysticercosis before medical treatment.

One of the most important differential diagnoses of medial rectus palsy cysticercosis is with isolated medial rectus palsy as a sign then clinically is easier to perform. There are an important number of publications related to isolated medial rectus palsy in of midbrain infarction but if impaired adduction of the ipsilateral eye is associated with a bilateral ptosis and normal pupil function midbrain infarction. [259-263]

## **6. Treatment of extraocular muscle cysticercosis**

Contact B-scan ultrasonography was a diagnostic test of cysticercosis in 84.4% of patients [77] but the accuracy of a CT scan and MRI is more than 98% in our series.

Surgical excision of an extraocular muscle cyst had been described [77, 177, 194, 202]. In contrast to medical treatment, surgical excision is technically difficult due to the attachment of the cysts to underlying orbital structures, the amorphous consistency of degenerating cysticerci and the risk to neurovascular structures in the posterior orbit. [29] There is also a likelihood of postoperative restrictive myopathy arising from the brotic response in surgical excisions of large cysts or in cysts requiring extensive dissection from the underlying muscle. [215] The potential risk of damage to adjacent tissue and adhesion from surgical exploration should not be taken lightly, particularly when effective medical therapy is available. As a general consent medical treatment with albendazol and prednisone is the best choice taken by most of the authors and recommended duration of treatment varies from a few days to up to 6 weeks. [29, 95,155, 194,197, 204- 208, 218,219, 220, 223, 224, 227- 232,237-240, 242, 252, 253,255, 264-266]

144 Novel Aspects on Cysticercosis and Neurocysticercosis

**Figure 14.** Medial rectus myocysticercosis

and normal pupil function midbrain infarction. [259-263]

**6. Treatment of extraocular muscle cysticercosis** 

[77] but the accuracy of a CT scan and MRI is more than 98% in our series.

may be caused by an excessively resected lateral rectus muscle.

Isolated medial rectus cysticercosis may be associated with ptosis and upper lid retraction on attempted adduction (aberrant regeneration) in patients with partial third nerve paralysis. Medial rectus cysticercosis must be distinguished from internuclear ophthalmoplegia (INO), caused by lesions in the medial longitudinal fasciculus. In this condition unilateral or bilateral limitation of adduction is associated with nystagmus of the abducting eye. Convergence may or may not be normal and we will discuss briefly about this matter below. A clinical picture similar isolated media rectus cysticercosis or INO can be caused by myasthenia gravis but a Tensilon test is diagnostic. Restriction of adduction

One of the most important differential diagnoses of medial rectus palsy cysticercosis is with isolated medial rectus palsy as a sign then clinically is easier to perform. There are an important number of publications related to isolated medial rectus palsy in of midbrain infarction but if impaired adduction of the ipsilateral eye is associated with a bilateral ptosis

Contact B-scan ultrasonography was a diagnostic test of cysticercosis in 84.4% of patients

Surgical excision of an extraocular muscle cyst had been described [77, 177, 194, 202]. In contrast to medical treatment, surgical excision is technically difficult due to the attachment of the cysts to underlying orbital structures, the amorphous consistency of degenerating cysticerci and the risk to neurovascular structures in the posterior orbit. [29] There is also a likelihood of postoperative restrictive myopathy arising from the brotic response in surgical excisions of large cysts or in cysts requiring extensive dissection from the In some, cyst elimination rates were more than 90%, and time to recovery of ocular motility ranged between 0.5 and 35 month. [136, 155, 265]

Although oral albendazole and prednisone are efficient, a long history of disease can lead to important residual ocular motility restriction [220] and some author found that oral albendazole and prednisone are not effective thus their role in ocular cysticercosis need to be studied. [256]

In the next figure we summarize the frequency of affected extraocular muscle by cysticercosis.

In some, cyst elimination rates were more than 90%, and time to recovery of ocular motility ranged between 0.5 and 35 month. [136, 155, 265]

Although oral albendazole and prednisone are efficient, a long history of disease can lead to important residual ocular motility restriction [220] and some author found that oral albendazole and prednisone are not effective thus their role in ocular cysticercosis need to be studied. [256]

In the next figure we summarize the frequency of affected extraocular muscle by cysticercosis before medical treatment.

**Figure 15.** Distribution of extraocular myocysticercosis in the orbital cavity.

## **7. Conclusion**

The cysticercosis of the orbit is an uncommon zoonotic parasitic disease. Its early diagnosis and appropriate treatment avoid the permanent loss of vision. The surgical treatment is the best choice in intraocular cysticercosis while medical treatment is the best choice for extraocular presentations including the extraocular muscles cysticercosis. The adequate knowledge of the anatomy and physiology of the intra orbital content contributes to a better diagnosis. Sometimes the patience helps resolve the conflicts that create the conjunctiva cysticercosis. It must take into account other processes that mimic the orbital cysticercosis in their differential diagnosis. Although it is a rare disease it is a challenge for those health professionals committed to the management of these patients.

Orbital Cisticercosis. A Challenger for Neurologists,

Ophthalmologists, Neuro-Opthalmologists, and General Practitioners 147

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[16] Narang P, Chhibbers S, Puri SK. Middle-aged man with altered behaviour and seizures.

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## **Author details**

Humberto Foyaca Sibat, María Carolina Salazar Campos and Lourdes de Fátima Ibañez Valdés *Department of Neurology and Department of Ophthalmology, Nelson Mandela Academic Hospital, Faculty of Health Sciences, Walter Sisulu University, South Africa* 

## **Acknowledgement**

For understanding my long nights at the computer, I'd like to thank my wife Lourdes de Fátima, who was worked with me on this project; to my first daughter Zayra Susana who died in 1979 but continues inspiring me from wherever she is; to my second daughter Lorna Maria who is a good lawyer and encourages me all the time to continue moving forward, and to my children Fatima Susana Adolfina (3 years old) and Thabo Humberto Jorge (4 years old) for helping me to find peace of mind, persistence and hope every time I needed. My father, my sisters, nephews, nieces, aunts, uncles, cousins and almost all members of my family contributed to this project in one way or another - to all of them: a great Thank You indeed.

## **8. References**


professionals committed to the management of these patients.

Humberto Foyaca Sibat, María Carolina Salazar Campos

*Faculty of Health Sciences, Walter Sisulu University, South Africa* 

Diseases 2008; 2: e300 doi:10.1371/journal.pntd.0000300.

Taenia solium cysticercosis. Lancet. 2003; 362: 547-556.

and Lourdes de Fátima Ibañez Valdés

The cysticercosis of the orbit is an uncommon zoonotic parasitic disease. Its early diagnosis and appropriate treatment avoid the permanent loss of vision. The surgical treatment is the best choice in intraocular cysticercosis while medical treatment is the best choice for extraocular presentations including the extraocular muscles cysticercosis. The adequate knowledge of the anatomy and physiology of the intra orbital content contributes to a better diagnosis. Sometimes the patience helps resolve the conflicts that create the conjunctiva cysticercosis. It must take into account other processes that mimic the orbital cysticercosis in their differential diagnosis. Although it is a rare disease it is a challenge for those health

*Department of Neurology and Department of Ophthalmology, Nelson Mandela Academic Hospital,* 

For understanding my long nights at the computer, I'd like to thank my wife Lourdes de Fátima, who was worked with me on this project; to my first daughter Zayra Susana who died in 1979 but continues inspiring me from wherever she is; to my second daughter Lorna Maria who is a good lawyer and encourages me all the time to continue moving forward, and to my children Fatima Susana Adolfina (3 years old) and Thabo Humberto Jorge (4 years old) for helping me to find peace of mind, persistence and hope every time I needed. My father, my sisters, nephews, nieces, aunts, uncles, cousins and almost all members of my family contributed to this project in one way or another - to all of them: a great Thank You

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**Chapter 7** 

© 2013 Foyaca Sibat and de Fátima Ibañez Valdés, licensee InTech. This is an open access chapter

(http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction

© 2013 Foyaca Sibat and de Fátima Ibañez Valdés, licensee InTech. This is a paper distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

distributed under the terms of the Creative Commons Attribution License

Neurocysticercosis (NCC) is the parasitic disease more frequent of the CNS and the causal agent of the same is the larval stage of the T solium cysticerci when invades brain of a human being that has swallowed the viable eggs from food or contaminated liquids, from a carrier, by infection secondary to a reverse peristalsis or by the well-known fecal-oral route [4-6]. The man is in turn the only known host for the adult form of the parasite. This disease is a serious health problem for many third-world countries of Asia, Africa, and Latin America; moreover, a growing number of cases are being reported in many highly developed countries in Europe and North America, notwithstanding the foregoing, the

in any medium, provided the original work is properly cited.

**What is a Low Frequency of the Disseminated** 

**Cysticercosis Suggests that Neurocysticercosis** 

The zoonotic infections that affect the man can be caused by viruses (rabies, avian influenza, immunodeficiency virus from apes [VIS]), bacteria (brucellosis, salmonellosis), parasites (leishmaniasis and schistosomiasis, neurocysticercosis, toxocariasis) and other nonconventional agents such as prions (bovine spongiform encephalopathy and a variant of

There are 1407 pathogens that affect humans (excluding the ectoparasites), more than half (816) are zoonotic and 73% (130) cause zoonotic diseases that affect community health [2]. Parasitic diseases have occurred through the times more deaths and economic damage to humanity that all the genocidal wars together. Parasitic infections previously seen only in developing tropical settings can be currently diagnosed worldwide due to travel and population migration. Generally in countries with little socio-economic development is where parasitic diseases are presented with greater frequency, still this is offered by climatic conditions warm or temperate zone and by the lack of education for health in the population; because in the developed countries social, medical and economically, parasitic

Humberto Foyaca Sibat and Lourdes de Fátima Ibañez Valdés

Jakob-Creutzfeldt disease) on which enough has been written[1].

diseases have been eradicated or have very little significance [3].

**is Going to Disappear?** 

http://dx.doi.org/10.5772/51395

**1. Introduction** 

Additional information is available at the end of the chapter


## **What is a Low Frequency of the Disseminated Cysticercosis Suggests that Neurocysticercosis is Going to Disappear?**

Humberto Foyaca Sibat and Lourdes de Fátima Ibañez Valdés

Additional information is available at the end of the chapter

http://dx.doi.org/10.5772/51395

## **1. Introduction**

160 Novel Aspects on Cysticercosis and Neurocysticercosis

Science. 2010;7(2)

(accesed on July 08, 2012)

(accessed on July 08, 2012)

Eye 1998;12:795–799

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[256] Tuli N. A Non Responding Case Of Extraocular Muscle Cysticercosis Mimicking As Pseudo-Tumor Orbit In A Child. The Internet Journal of Ophthalmology and Visual

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horizontal diplopia in a young adult. Br J Ophthalmol. 2005;89(3):390–391.

The zoonotic infections that affect the man can be caused by viruses (rabies, avian influenza, immunodeficiency virus from apes [VIS]), bacteria (brucellosis, salmonellosis), parasites (leishmaniasis and schistosomiasis, neurocysticercosis, toxocariasis) and other nonconventional agents such as prions (bovine spongiform encephalopathy and a variant of Jakob-Creutzfeldt disease) on which enough has been written[1].

There are 1407 pathogens that affect humans (excluding the ectoparasites), more than half (816) are zoonotic and 73% (130) cause zoonotic diseases that affect community health [2]. Parasitic diseases have occurred through the times more deaths and economic damage to humanity that all the genocidal wars together. Parasitic infections previously seen only in developing tropical settings can be currently diagnosed worldwide due to travel and population migration. Generally in countries with little socio-economic development is where parasitic diseases are presented with greater frequency, still this is offered by climatic conditions warm or temperate zone and by the lack of education for health in the population; because in the developed countries social, medical and economically, parasitic diseases have been eradicated or have very little significance [3].

Neurocysticercosis (NCC) is the parasitic disease more frequent of the CNS and the causal agent of the same is the larval stage of the T solium cysticerci when invades brain of a human being that has swallowed the viable eggs from food or contaminated liquids, from a carrier, by infection secondary to a reverse peristalsis or by the well-known fecal-oral route [4-6]. The man is in turn the only known host for the adult form of the parasite. This disease is a serious health problem for many third-world countries of Asia, Africa, and Latin America; moreover, a growing number of cases are being reported in many highly developed countries in Europe and North America, notwithstanding the foregoing, the

© 2013 Foyaca Sibat and de Fátima Ibañez Valdés, licensee InTech. This is an open access chapter distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. © 2013 Foyaca Sibat and de Fátima Ibañez Valdés, licensee InTech. This is a paper distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

incidence and prevalence accurate of this disease have not been confirmed from welldesigned studies. Disseminated cysticercosis (DCC) was reported in 1912 by doctors of the English Navy stationed in India. Priest in 1926, describes a British soldier patient presenting an inflammation of the skeletal muscles, seizures, mental dulling and a large amount of subcutaneous nodules distributed throughout the body [7]. An intensive search performed in 1988 revealed 22 cases [7] and only 16 additional cases were reported until 2006 reported in the international medical literature most of them from India. [8]. It is considered that cysticercosis (CC) is a major problem for public health in several developing countries where the social, economic and cultural conditions favor the maintenance of this zoonotic disease and it is seen as a growing community problem in those developed countries with a high rate of immigrants from endemic areas. WHO includes the NCC between neglected diseases that cause a significant impact on the economy in several regions of the world. It affects 4% of the population in endemic areas [4, 9] and is the main cause of symptomatic epilepsy worldwide [1, 10-15] but where the hygiene habits-food and sometimes religious trends can determine the incidence and prevalence of the disease [1].

What is a Low Frequency of the Disseminated

Cysticercosis Suggests that Neurocysticercosis is Going to Disappear? 163

36]. These manifestations begin between one and 35 years after the exposure to the parasite, as demonstrated in patients from the British army stationed in India where it is defined exactly the time of exposure to the parasite and the onset of clinical manifestations. The average life Expectancy of the parasite is estimated to be between four and five years [37]. The epilepsy secondary to NCC (ESNCC) responds well to first line antiepileptic drugs (AED) [1,10-15, 19,24, 28,31, 33, 38]. Other AED as the levetiracitam has also shown good results, but its high cost and lack of availability in the public sector and the primary health care system make it considered without practical usefulness for the control of the ESNCC [1]. Patients with DCC can present: refractory epilepsy, neuropsychiatric disturbances, pseudo hypertrophy in the four limbs and affectation of any other organ or system [8]. However, a fairly typical manifestation of the DCC is the subcutaneous cyst that occurs as a asymptomatic nodule. These subcutaneous nodules can be slide easily in the muscle tissue and measure up to 1.5 cm in diameter. In the case of patients who have suffered from the disease for more than five

Recently, Bielsa-Fernández [40] reported a patient carrier of severe rheumatic polymyalgia of the trunk and limbs with serological surveys negative for the CC and two muscle biopsy confirmed encapsulated nodules with diffuse calcification, compatible with parasitic diseases, whose state of calcification prevented identify the characteristics of the parasite. Logically, the presence of active NCC leaves no doubt about the nature of the subcutaneous nodules and reinforces the importance of bearing in mind the diagnostic criteria of Del

The main characteristic the DCC is structural epilepsy due to neurocysticercosis and the pseudo-hypertrophy in the four members and the allocation of any other organ or system [8]. However, a manifestation fairly typical of the DCC may be subcutaneous cyst that occurs as a nodule asymptomatic. These subcutaneous nodules can be sliders in the muscle and measure up to 1.5 cm. In the case of patients who have had for more than 5 years cysticerci, these are calcified [39]. A few months ago, Bielsa-Fernández [40] reported a female patient with a severe rheumatic polymyalgia of the trunk and limbs with serological studies for the CC negative and two muscle biopsy confirmed two nodes encapsulated with diffuse calcification, compatible with parasitosis, whose calcification of the content made it difficult determine the type of parasite. Logically, the presence of NCC active leaves no

The pharmacological treatment of DCC reported good results for some patients with subcutaneous and muscular involvement if the CNS is not severely affected [41, 42]. On the other hand, have been confirmed cases with DCC and chronic liver disease in which case even if the ELISA test was suggestive, the final diagnosis was obtained by CT scans of the brain [43], and have been reported cases with unilateral eye lesion [44], and even bilaterally [80], thyroid involvement [8, 45] , pancreatic enzyme alterations [46] or without clinical or biochemical alterations of pancreatic function [47], spleen damage [47,47], cardiac [8, 48-53], even without associated DCC [52], lung [45, 53-55], joints [56] and muscles of the face and neck [44, 48]. There is not reported cases of DCC without involvement of CNS and its frequency may be the expression of an increase or decrease in the overall prevalence of

years, usually these are calcified [39].

Brutto for the correct differential diagnosis [41].

doubt about the nature of the subcutaneous nodules.

Based in our experience and according to this review of the literature we can say that in regions where the CC is endemic, the presence of late onset seizures in subjects older than 25 years of age is highly suggestive of NCC what had already been established since 1982 [16]. In the published series, the age group most commonly affected by the NCC, is the group of 35 to 63 years [18-20]. The males are most affected than females in the majority of the studios [17-19]. In 2003, Mafojane [20] reported a high prevalence in children and young South Africans in a region where the CC virtually does not exist.

Probably, there is a progressive decrease incidence of DCC based in our local perception and disseminated cysticercosis is always associated with NCC but it does not mean that the NCC is on the way to disappear completely in the next decade. In this chapter we will present a bibliographic investigation on the DCC, its prevalence in our region, and our arguments on the because we are going to spend many years before that the DCC eradicated from the face of the earth.

The pathogenesis and clinical manifestations of CC depend on the number, size, location, evolutionary state of the parasite and the host's immune response [1,5,10-15, 21-23]. In the above-mentioned studies, appear only the seizures and the headache as initial clinical manifestations while in others it is reported: meningitis, dementia, intracranial Hypertension, psychiatric symptoms, ischemic stroke, and radicular compression [10-15,17, 24-30]. It is important to note that the headache is not the most frequent clinical manifestation of the NCC in the majority of published studies but that occupies the second place; only in one of the studies consulted is presented as the main reason for consultation [19]. In the majority of the series, the frequency of headache varies from 4.6 % to 61.5 % [19,23-24]. While the frequency of seizures as a form of presentation of the NCC ranges from 54.3 % to 62.5 % in general series [12, 23, 27, 31-34]. In children the frequency of seizures to be the initial manifestation is higher, ranging from 72% to 94.8 % according to other authors [29, 35]. In summary, the majority of published studies confirm that the epilepsy and headache were the most frequent clinical manifestations of the NCC [1,10-15, 17, 24, 27-30, 36]. These manifestations begin between one and 35 years after the exposure to the parasite, as demonstrated in patients from the British army stationed in India where it is defined exactly the time of exposure to the parasite and the onset of clinical manifestations. The average life Expectancy of the parasite is estimated to be between four and five years [37]. The epilepsy secondary to NCC (ESNCC) responds well to first line antiepileptic drugs (AED) [1,10-15, 19,24, 28,31, 33, 38]. Other AED as the levetiracitam has also shown good results, but its high cost and lack of availability in the public sector and the primary health care system make it considered without practical usefulness for the control of the ESNCC [1]. Patients with DCC can present: refractory epilepsy, neuropsychiatric disturbances, pseudo hypertrophy in the four limbs and affectation of any other organ or system [8]. However, a fairly typical manifestation of the DCC is the subcutaneous cyst that occurs as a asymptomatic nodule. These subcutaneous nodules can be slide easily in the muscle tissue and measure up to 1.5 cm in diameter. In the case of patients who have suffered from the disease for more than five years, usually these are calcified [39].

162 Novel Aspects on Cysticercosis and Neurocysticercosis

incidence and prevalence accurate of this disease have not been confirmed from welldesigned studies. Disseminated cysticercosis (DCC) was reported in 1912 by doctors of the English Navy stationed in India. Priest in 1926, describes a British soldier patient presenting an inflammation of the skeletal muscles, seizures, mental dulling and a large amount of subcutaneous nodules distributed throughout the body [7]. An intensive search performed in 1988 revealed 22 cases [7] and only 16 additional cases were reported until 2006 reported in the international medical literature most of them from India. [8]. It is considered that cysticercosis (CC) is a major problem for public health in several developing countries where the social, economic and cultural conditions favor the maintenance of this zoonotic disease and it is seen as a growing community problem in those developed countries with a high rate of immigrants from endemic areas. WHO includes the NCC between neglected diseases that cause a significant impact on the economy in several regions of the world. It affects 4% of the population in endemic areas [4, 9] and is the main cause of symptomatic epilepsy worldwide [1, 10-15] but where the hygiene habits-food and sometimes religious

Based in our experience and according to this review of the literature we can say that in regions where the CC is endemic, the presence of late onset seizures in subjects older than 25 years of age is highly suggestive of NCC what had already been established since 1982 [16]. In the published series, the age group most commonly affected by the NCC, is the group of 35 to 63 years [18-20]. The males are most affected than females in the majority of the studios [17-19]. In 2003, Mafojane [20] reported a high prevalence in children and young South

Probably, there is a progressive decrease incidence of DCC based in our local perception and disseminated cysticercosis is always associated with NCC but it does not mean that the NCC is on the way to disappear completely in the next decade. In this chapter we will present a bibliographic investigation on the DCC, its prevalence in our region, and our arguments on the because we are going to spend many years before that the DCC eradicated

The pathogenesis and clinical manifestations of CC depend on the number, size, location, evolutionary state of the parasite and the host's immune response [1,5,10-15, 21-23]. In the above-mentioned studies, appear only the seizures and the headache as initial clinical manifestations while in others it is reported: meningitis, dementia, intracranial Hypertension, psychiatric symptoms, ischemic stroke, and radicular compression [10-15,17, 24-30]. It is important to note that the headache is not the most frequent clinical manifestation of the NCC in the majority of published studies but that occupies the second place; only in one of the studies consulted is presented as the main reason for consultation [19]. In the majority of the series, the frequency of headache varies from 4.6 % to 61.5 % [19,23-24]. While the frequency of seizures as a form of presentation of the NCC ranges from 54.3 % to 62.5 % in general series [12, 23, 27, 31-34]. In children the frequency of seizures to be the initial manifestation is higher, ranging from 72% to 94.8 % according to other authors [29, 35]. In summary, the majority of published studies confirm that the epilepsy and headache were the most frequent clinical manifestations of the NCC [1,10-15, 17, 24, 27-30,

trends can determine the incidence and prevalence of the disease [1].

Africans in a region where the CC virtually does not exist.

from the face of the earth.

Recently, Bielsa-Fernández [40] reported a patient carrier of severe rheumatic polymyalgia of the trunk and limbs with serological surveys negative for the CC and two muscle biopsy confirmed encapsulated nodules with diffuse calcification, compatible with parasitic diseases, whose state of calcification prevented identify the characteristics of the parasite. Logically, the presence of active NCC leaves no doubt about the nature of the subcutaneous nodules and reinforces the importance of bearing in mind the diagnostic criteria of Del Brutto for the correct differential diagnosis [41].

The main characteristic the DCC is structural epilepsy due to neurocysticercosis and the pseudo-hypertrophy in the four members and the allocation of any other organ or system [8]. However, a manifestation fairly typical of the DCC may be subcutaneous cyst that occurs as a nodule asymptomatic. These subcutaneous nodules can be sliders in the muscle and measure up to 1.5 cm. In the case of patients who have had for more than 5 years cysticerci, these are calcified [39]. A few months ago, Bielsa-Fernández [40] reported a female patient with a severe rheumatic polymyalgia of the trunk and limbs with serological studies for the CC negative and two muscle biopsy confirmed two nodes encapsulated with diffuse calcification, compatible with parasitosis, whose calcification of the content made it difficult determine the type of parasite. Logically, the presence of NCC active leaves no doubt about the nature of the subcutaneous nodules.

The pharmacological treatment of DCC reported good results for some patients with subcutaneous and muscular involvement if the CNS is not severely affected [41, 42]. On the other hand, have been confirmed cases with DCC and chronic liver disease in which case even if the ELISA test was suggestive, the final diagnosis was obtained by CT scans of the brain [43], and have been reported cases with unilateral eye lesion [44], and even bilaterally [80], thyroid involvement [8, 45] , pancreatic enzyme alterations [46] or without clinical or biochemical alterations of pancreatic function [47], spleen damage [47,47], cardiac [8, 48-53], even without associated DCC [52], lung [45, 53-55], joints [56] and muscles of the face and neck [44, 48]. There is not reported cases of DCC without involvement of CNS and its frequency may be the expression of an increase or decrease in the overall prevalence of porcine cysticercosis in general [1]. The host inflammatory response depends on the parasite's ability to evade host immunity. Usually both 'healthy' (active) and 'involuted' (inactive) cysticerci lack inflammatory response, which is restricted to 'currently degenerating' cysts whose ability to evade host defenses is becoming faulty. Involution of cysts implies granulomatous inflammation [56].

What is a Low Frequency of the Disseminated

Cysticercosis Suggests that Neurocysticercosis is Going to Disappear? 165

1974 but was only recognized by South Africa with whom confronted territorial disputes between 1974 and 1978. The natural history of the NCC must be viewed within the historical context of this region in order to understand why the NCC has been confined almost to the former Transkei (currently region C and D of Eastern Cape Province), still the only endemic area of the country from where they come from all the sick people who could be diagnosed

Human cysticercosis appears to be most prevalent in Eastern Cape Province particularly in the poor, former black homeland, rural areas of Transkei, where pigs are allowed to roam freely and sanitation facilities are inadequate or nonexistent. Pig keeping and pork consumption have increased significantly during the past decade especially in rural smallholder communities, primarily due to the lack of grazing land for ruminants and the recognition of farmers of a quicker and more impressive return on their investment from

The first inform about patients presenting DCC in South Africa was made by Schultz and Mentis [58] In 1987. They reported a 48-year-old black man with two month history of a painless mass on the left side of his neck. He also complained of mild attacks of paroxysmal dyspnoea at night and progressive deafness of his left ear. On direct questioning the patient referred few grand mal seizures during the 6 years before admission. No other complaints or previous illnesses of note were elicited. The patient was born in Transkei (near to Mthatha) and had travelled throughout many parts of the country seeking work. On examination he appeared healthy and was of normal build. The pulse rate was 72/min, blood pressure 110/60 mmHg and temperature 37,2°C. Numerous subcutaneous nodules, ranging from one to three centimeter in diameter, which were well circumscribed and freely movable, could be palpated all over the neck, chest and legs. A bigger mass was palpable inferior to the left ear, extending down the left side of the neck. There was conduction deafness of the left ear. On chest auscultation a few fine crepitations were audible at the bases of both lungs, chest radiography revealed multiple nodular opacities; computed tomography of the brain, neck, thorax and upper abdomen revealed extensive organ involvement, including brain, thyroid, lungs, pancreas, kidney, spleen and muscle; and biopsies of two of the subcutaneous nodules were histologically pathognomonic of cysticercosis. The patient received praziquantel (Biltricide; Bayer-Miles) 1, 75 g twice daily and prednisone 20 mg per day for 4 weeks. He was reassessed radiologically after 4 weeks. There was marked regression in the size and numbers of nodules in the lung fields. Clinically, most of the subcutaneous nodules had disappeared. This is the only report in the international medical literary on CCD with so large involvement of organs and systems

Bhigjee in 1999 [59], reported the second patient presenting DCC in South Africa. His patient was an Indian male presenting generalized subcutaneous and muscular nodules and neurocysticercosis. Diagnosis was confirmed by plain X-rays of the soft tissues in upper and lower limbs and CT scan of the brain where multiple bilateral active and calcify cysticerci on

in the rest of the country.

raising pigs.

including the kidneys.

both cerebral hemisphere were found. (Figure 1)

Epilepsy is the most common neurological manifestation of DCC in our region and it has been well documented in previous publication. [41,50] However seizures disorder in HIV patients present special problem with regard to choice of antiepileptic drugs (AED) and the potential for drug-drug interactions with antiretroviral (ARV) treatments also in our region. Newer AEDs with simpler pharmacokinetic profiles may be the preferred agents, particularly when protease inhibitors form part of ARV regimens. Seizures in NCC are easily controlled with the first line AEDs. Although there has been some debate about the value of anti-parasitic drugs in NCC, accumulating data suggest that the use of these agents in active disease decreases the risk for development of chronic epilepsy [57].

## **2. Methodology**

In this chapter we included only cases reported to the medical literature, therefore no major ethical problems are afforded. Nevertheless, in order to protect all subjects participating in our studies, as part of a general measure aimed at protecting patients selected it was agreed that the principal investigator (HFS) and collaborators (LdeFIV) should adopt a program of basic training on the protection of human beings and animals participating in research studies and obtain the corresponding certificate which was done. All protocols were approved by the Review Committee from the University of Transkei and the Walter Sisulu University plus their respective Committee on Medical Ethics and the numbers of references are the following: Mthatha Hospital (UGH: 0001/99, letter of approval by the Director of Medical Services), University of Transkei (UNITRA:0018/05), and Walter Sisulu University (IRB) and their respective Ethics Committee (WSU:0068/09). The model of informed consent was signed by all participants in the first contact as a prerequisite to participate. To illiterate patients were explained the objectives of the study, the procedures which were to be subjected and their risks and the model of consent was signed by a family member, relative or a witness. To all patients we explained their rights to participate or not, with the assurance that in any case, this would not cause change in management or the attention of the same or other rights of any patient, their rights on anonymity (personal data replaced by keys), and to keep any information in strict confidentiality. In this chapter we did not use any graphical or written confidential information from patients reported by other author.

## **3. Results**

## **3.1. Disseminated cysticercosis in South Africa**

The former Transkei is the poorest region in South Africa and it was a well known batustan during the apartheid régimen before 1994. To the population of Xhosa origin were assigned the regions of the Ciskei and Transkei and Transkei is termed as an independent state in 1974 but was only recognized by South Africa with whom confronted territorial disputes between 1974 and 1978. The natural history of the NCC must be viewed within the historical context of this region in order to understand why the NCC has been confined almost to the former Transkei (currently region C and D of Eastern Cape Province), still the only endemic area of the country from where they come from all the sick people who could be diagnosed in the rest of the country.

164 Novel Aspects on Cysticercosis and Neurocysticercosis

**2. Methodology** 

**3. Results** 

**3.1. Disseminated cysticercosis in South Africa** 

cysts implies granulomatous inflammation [56].

porcine cysticercosis in general [1]. The host inflammatory response depends on the parasite's ability to evade host immunity. Usually both 'healthy' (active) and 'involuted' (inactive) cysticerci lack inflammatory response, which is restricted to 'currently degenerating' cysts whose ability to evade host defenses is becoming faulty. Involution of

Epilepsy is the most common neurological manifestation of DCC in our region and it has been well documented in previous publication. [41,50] However seizures disorder in HIV patients present special problem with regard to choice of antiepileptic drugs (AED) and the potential for drug-drug interactions with antiretroviral (ARV) treatments also in our region. Newer AEDs with simpler pharmacokinetic profiles may be the preferred agents, particularly when protease inhibitors form part of ARV regimens. Seizures in NCC are easily controlled with the first line AEDs. Although there has been some debate about the value of anti-parasitic drugs in NCC, accumulating data suggest that the use of these agents

In this chapter we included only cases reported to the medical literature, therefore no major ethical problems are afforded. Nevertheless, in order to protect all subjects participating in our studies, as part of a general measure aimed at protecting patients selected it was agreed that the principal investigator (HFS) and collaborators (LdeFIV) should adopt a program of basic training on the protection of human beings and animals participating in research studies and obtain the corresponding certificate which was done. All protocols were approved by the Review Committee from the University of Transkei and the Walter Sisulu University plus their respective Committee on Medical Ethics and the numbers of references are the following: Mthatha Hospital (UGH: 0001/99, letter of approval by the Director of Medical Services), University of Transkei (UNITRA:0018/05), and Walter Sisulu University (IRB) and their respective Ethics Committee (WSU:0068/09). The model of informed consent was signed by all participants in the first contact as a prerequisite to participate. To illiterate patients were explained the objectives of the study, the procedures which were to be subjected and their risks and the model of consent was signed by a family member, relative or a witness. To all patients we explained their rights to participate or not, with the assurance that in any case, this would not cause change in management or the attention of the same or other rights of any patient, their rights on anonymity (personal data replaced by keys), and to keep any information in strict confidentiality. In this chapter we did not use any graphical or written confidential information from patients reported by other author.

The former Transkei is the poorest region in South Africa and it was a well known batustan during the apartheid régimen before 1994. To the population of Xhosa origin were assigned the regions of the Ciskei and Transkei and Transkei is termed as an independent state in

in active disease decreases the risk for development of chronic epilepsy [57].

Human cysticercosis appears to be most prevalent in Eastern Cape Province particularly in the poor, former black homeland, rural areas of Transkei, where pigs are allowed to roam freely and sanitation facilities are inadequate or nonexistent. Pig keeping and pork consumption have increased significantly during the past decade especially in rural smallholder communities, primarily due to the lack of grazing land for ruminants and the recognition of farmers of a quicker and more impressive return on their investment from raising pigs.

The first inform about patients presenting DCC in South Africa was made by Schultz and Mentis [58] In 1987. They reported a 48-year-old black man with two month history of a painless mass on the left side of his neck. He also complained of mild attacks of paroxysmal dyspnoea at night and progressive deafness of his left ear. On direct questioning the patient referred few grand mal seizures during the 6 years before admission. No other complaints or previous illnesses of note were elicited. The patient was born in Transkei (near to Mthatha) and had travelled throughout many parts of the country seeking work. On examination he appeared healthy and was of normal build. The pulse rate was 72/min, blood pressure 110/60 mmHg and temperature 37,2°C. Numerous subcutaneous nodules, ranging from one to three centimeter in diameter, which were well circumscribed and freely movable, could be palpated all over the neck, chest and legs. A bigger mass was palpable inferior to the left ear, extending down the left side of the neck. There was conduction deafness of the left ear. On chest auscultation a few fine crepitations were audible at the bases of both lungs, chest radiography revealed multiple nodular opacities; computed tomography of the brain, neck, thorax and upper abdomen revealed extensive organ involvement, including brain, thyroid, lungs, pancreas, kidney, spleen and muscle; and biopsies of two of the subcutaneous nodules were histologically pathognomonic of cysticercosis. The patient received praziquantel (Biltricide; Bayer-Miles) 1, 75 g twice daily and prednisone 20 mg per day for 4 weeks. He was reassessed radiologically after 4 weeks. There was marked regression in the size and numbers of nodules in the lung fields. Clinically, most of the subcutaneous nodules had disappeared. This is the only report in the international medical literary on CCD with so large involvement of organs and systems including the kidneys.

Bhigjee in 1999 [59], reported the second patient presenting DCC in South Africa. His patient was an Indian male presenting generalized subcutaneous and muscular nodules and neurocysticercosis. Diagnosis was confirmed by plain X-rays of the soft tissues in upper and lower limbs and CT scan of the brain where multiple bilateral active and calcify cysticerci on both cerebral hemisphere were found. (Figure 1)

What is a Low Frequency of the Disseminated

Cysticercosis Suggests that Neurocysticercosis is Going to Disappear? 167

complete heart block (Figure 5). The patient was initially treated only with prednisolone, and anti-helminthic treatment was deferred fearing peri-cystic inflammation and aggravation of the conduction abnormalities. Five days later praziquantel 50 mg/kg/day for 14 days were added to the treatment regimen. Repeat ECG after a week of treatment showed sinus rhythm with a heart rate of 70/min. Diagnostic criteria for disseminated cysticercosis for this patient is based on the presence of NCC, subcutaneous, and muscular

**Figure 2.** Multiple subcutaneous nodules on the chest wall and a few calcified lesions on chest X-ray. (Photo taken by H Foyaca-Sibat. Source: Foyaca et al., Rev Electron Biomed / Electron J Biomed

2004;3:39-43. Written patient consent is obtained).

cysticercosis simultaneously.

**Figure 1.** Shows generalized subcutaneous and intramuscular calcified nodules on lower limbs and intraparenchymal neurocysticercosis in vesicular, colloid and calcified stages. (Source: J Neurol Neurosurg Psychiatry 1999; 66(4): 545. Bhigjee A).

Five years later we reported a 42-year-old man (third report) admitted at Nelson Mandela Academic Hospital Mthatha in South Africa. He presented a history of recurrent generalized tonic-clonic epileptic seizures of six years duration, disseminated nodules all over the body of two-year duration and headache. On general examination multiple subcutaneous and intramuscularly, mobile, no tender nodules, measuring from 0.7 to 2.5 cm, were palpable on the chest, back, abdomen, and proximal regions of the four limbs. (Figure 2-3-4) Respiratory and cardiovascular system were normal except for a bradycardia of 42 bpm. A computed tomography (CT) scan of the brain showed multiple active and calcified lesions. Biopsy of the subcutaneous nodules confirmed the diagnosis of cysticercosis (Figure 3). A detailed neurological examination revealed unremarkable findings. Laboratory data were normal, ELISA test and IgG for cysticercosis were strongly positive. ECG and cardiac ultrasound confirmed: sinus bradicardia, II grade heart block, and calcifications in papillary muscles and upper septum respectively. CT Scan of the brain (Figure 4) shows bilateral cysts in different active stages and calcified NCC. [41]

We referred this patient to the cardiology clinic in our hospital for assessment of bradycardia and some findings were reported later by other authors [60]. Patient felt dizzy on getting up suddenly from a supine position. His pulse rate was 41/min, irregular and his blood pressure 76/124 mmHg. An electrocardiogram (ECG) showed sinus bradycardia with complete heart block (Figure 5). The patient was initially treated only with prednisolone, and anti-helminthic treatment was deferred fearing peri-cystic inflammation and aggravation of the conduction abnormalities. Five days later praziquantel 50 mg/kg/day for 14 days were added to the treatment regimen. Repeat ECG after a week of treatment showed sinus rhythm with a heart rate of 70/min. Diagnostic criteria for disseminated cysticercosis for this patient is based on the presence of NCC, subcutaneous, and muscular cysticercosis simultaneously.

166 Novel Aspects on Cysticercosis and Neurocysticercosis

Neurosurg Psychiatry 1999; 66(4): 545. Bhigjee A).

different active stages and calcified NCC. [41]

**Figure 1.** Shows generalized subcutaneous and intramuscular calcified nodules on lower limbs and intraparenchymal neurocysticercosis in vesicular, colloid and calcified stages. (Source: J Neurol

Five years later we reported a 42-year-old man (third report) admitted at Nelson Mandela Academic Hospital Mthatha in South Africa. He presented a history of recurrent generalized tonic-clonic epileptic seizures of six years duration, disseminated nodules all over the body of two-year duration and headache. On general examination multiple subcutaneous and intramuscularly, mobile, no tender nodules, measuring from 0.7 to 2.5 cm, were palpable on the chest, back, abdomen, and proximal regions of the four limbs. (Figure 2-3-4) Respiratory and cardiovascular system were normal except for a bradycardia of 42 bpm. A computed tomography (CT) scan of the brain showed multiple active and calcified lesions. Biopsy of the subcutaneous nodules confirmed the diagnosis of cysticercosis (Figure 3). A detailed neurological examination revealed unremarkable findings. Laboratory data were normal, ELISA test and IgG for cysticercosis were strongly positive. ECG and cardiac ultrasound confirmed: sinus bradicardia, II grade heart block, and calcifications in papillary muscles and upper septum respectively. CT Scan of the brain (Figure 4) shows bilateral cysts in

We referred this patient to the cardiology clinic in our hospital for assessment of bradycardia and some findings were reported later by other authors [60]. Patient felt dizzy on getting up suddenly from a supine position. His pulse rate was 41/min, irregular and his blood pressure 76/124 mmHg. An electrocardiogram (ECG) showed sinus bradycardia with

**Figure 2.** Multiple subcutaneous nodules on the chest wall and a few calcified lesions on chest X-ray. (Photo taken by H Foyaca-Sibat. Source: Foyaca et al., Rev Electron Biomed / Electron J Biomed 2004;3:39-43. Written patient consent is obtained).

What is a Low Frequency of the Disseminated

Cysticercosis Suggests that Neurocysticercosis is Going to Disappear? 169

**Figure 5.** shows sinus bradycardia with complete heart block.[60]

seen in Chagas' disease).

The principal electrocardiographic (ECG) alterations are first-degree atrioventricular (AV) block, low QRS (QRS interval of the ECG) voltage, and primary T-wave changes (as can

**Figure 6.** CT scan of chest confirmed the presence of cysts within the myocardium.[60]

**Figure 3.** Multiple calcified subcutaneous and paravertebral nodules. (Photo taken by H Foyaca-Sibat. Source: Foyaca et al., Rev Electron Biomed / Electron J Biomed 2004;3:39-43. Patient written consent is obtained).

**Figure 4.** CT scan of the brain showing multiples intraparenchymal cysticerci in vesicular, colloid and calcified stages.

What is a Low Frequency of the Disseminated Cysticercosis Suggests that Neurocysticercosis is Going to Disappear? 169

**Figure 5.** shows sinus bradycardia with complete heart block.[60]

168 Novel Aspects on Cysticercosis and Neurocysticercosis

obtained).

calcified stages.

**Figure 3.** Multiple calcified subcutaneous and paravertebral nodules. (Photo taken by H Foyaca-Sibat. Source: Foyaca et al., Rev Electron Biomed / Electron J Biomed 2004;3:39-43. Patient written consent is

**Figure 4.** CT scan of the brain showing multiples intraparenchymal cysticerci in vesicular, colloid and

The principal electrocardiographic (ECG) alterations are first-degree atrioventricular (AV) block, low QRS (QRS interval of the ECG) voltage, and primary T-wave changes (as can seen in Chagas' disease).

**Figure 6.** CT scan of chest confirmed the presence of cysts within the myocardium.[60]

In 2007, we reported a 48-year-old African male patient (Fouth patient) admitted at Nelson Mandela Academic Hospital in Mthatha, South Africa presenting a history of recurrent generalized tonic-clonic epileptic seizures with urinary incontinence of five years duration. Patient reported to have fitted at home with three episodes on the day of admission and sustained burns on the right leg while he was unconscious (Figure 7).

What is a Low Frequency of the Disseminated

Cysticercosis Suggests that Neurocysticercosis is Going to Disappear? 171

(FBC, U&E, glucose, urinalysis) were normal, erythrocyte sedimentation rate and cardiac enzymes were also normal. ELISA test and IgG for cysticercosis were strongly positive.

Plain chest X-rays and X-rays of long bones showed multiple "cigar-shape calcifications" (Figure 11) and abdomen ultrasound confirmed multiple subcutaneous cystic lesions seen with centric enhancing form remembering the typical "dot-in-hole". ECG and cardiac ultrasound confirmed: sinus bradicardia, II grade heart block and calcifications in papillary muscles and upper septum respectively (Figure 7). CT scan of the brain showed bilateral cystic lesions in vesicular and colloid stages, and calcified NCC [50]. Plain X-ray studies of the muscles are also useful to identify the typical "cigar-shape" calcifications on the subcutaneous tissue, and if any query arise then a positive serological test for T solium (antigen/antibody) will help while the final diagnosis is confirmed by biopsy of the

**Figure 8.** Show subcutaneous nodule on the right lateral aspect of the neck and multiple subcutaneous nodules in the upper back mainly on the right side. (Photo taken by H Foyaca-Sibat. Source: H. Foyaca-Sibat & L. Ibanez-Valdes: Generalized Cysticercosis With Cardiac Involvement. *The Internet Journal of* 

*Neurology.* 2007 Volume 7 Number 2.)

subcutaneous nodules or confirming NCC by imagenology.

**Figure 7.** Show a burning lesion acquired during tonic-clonic generalized epileptic seizure. (Photo taken by H Foyaca-Sibat. Source: H. Foyaca-Sibat & L. Ibanez-Valdes: Generalized Cysticercosis With Cardiac Involvement. The Internet Journal of Neurology. (ISSN: 1531-295X) 2007; 7(2):6-11.

Burn is a frequent accident for epileptic peoples while they having epileptic seizures in rural areas when electricity is not available and they need to keep small fire inside the room as alternative way to survive during the winter. Unfortunately, there is not sustainable solution for this problem at the present moment.

This patient was on oral carbamazepine 200mg three times a day, but he discontinued treatment 2 weeks back because medication was not available at the nearest medical clinic. On general examination multiple subcutaneous and intramuscularly, mobile, no tender nodules, measuring from 0.7 to 2.5 cm, were palpable on the chest, back, abdomen, proximal regions of the four limbs and hemi-face (Figure 8-12). Respiratory and cardiovascular system were normal except for a bradicardia of 46 beat per minute. A detailed neurological examination revealed unremarkable findings. Laboratory data included routine blood test (FBC, U&E, glucose, urinalysis) were normal, erythrocyte sedimentation rate and cardiac enzymes were also normal. ELISA test and IgG for cysticercosis were strongly positive.

170 Novel Aspects on Cysticercosis and Neurocysticercosis

In 2007, we reported a 48-year-old African male patient (Fouth patient) admitted at Nelson Mandela Academic Hospital in Mthatha, South Africa presenting a history of recurrent generalized tonic-clonic epileptic seizures with urinary incontinence of five years duration. Patient reported to have fitted at home with three episodes on the day of admission and

**Figure 7.** Show a burning lesion acquired during tonic-clonic generalized epileptic seizure. (Photo taken by H Foyaca-Sibat. Source: H. Foyaca-Sibat & L. Ibanez-Valdes: Generalized Cysticercosis With

Burn is a frequent accident for epileptic peoples while they having epileptic seizures in rural areas when electricity is not available and they need to keep small fire inside the room as alternative way to survive during the winter. Unfortunately, there is not sustainable

This patient was on oral carbamazepine 200mg three times a day, but he discontinued treatment 2 weeks back because medication was not available at the nearest medical clinic. On general examination multiple subcutaneous and intramuscularly, mobile, no tender nodules, measuring from 0.7 to 2.5 cm, were palpable on the chest, back, abdomen, proximal regions of the four limbs and hemi-face (Figure 8-12). Respiratory and cardiovascular system were normal except for a bradicardia of 46 beat per minute. A detailed neurological examination revealed unremarkable findings. Laboratory data included routine blood test

Cardiac Involvement. The Internet Journal of Neurology. (ISSN: 1531-295X) 2007; 7(2):6-11.

solution for this problem at the present moment.

sustained burns on the right leg while he was unconscious (Figure 7).

Plain chest X-rays and X-rays of long bones showed multiple "cigar-shape calcifications" (Figure 11) and abdomen ultrasound confirmed multiple subcutaneous cystic lesions seen with centric enhancing form remembering the typical "dot-in-hole". ECG and cardiac ultrasound confirmed: sinus bradicardia, II grade heart block and calcifications in papillary muscles and upper septum respectively (Figure 7). CT scan of the brain showed bilateral cystic lesions in vesicular and colloid stages, and calcified NCC [50]. Plain X-ray studies of the muscles are also useful to identify the typical "cigar-shape" calcifications on the subcutaneous tissue, and if any query arise then a positive serological test for T solium (antigen/antibody) will help while the final diagnosis is confirmed by biopsy of the subcutaneous nodules or confirming NCC by imagenology.

**Figure 8.** Show subcutaneous nodule on the right lateral aspect of the neck and multiple subcutaneous nodules in the upper back mainly on the right side. (Photo taken by H Foyaca-Sibat. Source: H. Foyaca-Sibat & L. Ibanez-Valdes: Generalized Cysticercosis With Cardiac Involvement. *The Internet Journal of Neurology.* 2007 Volume 7 Number 2.)

What is a Low Frequency of the Disseminated

Cysticercosis Suggests that Neurocysticercosis is Going to Disappear? 173

**Figure 11.** Plain X-ray of the chest and upper limbs show multiple subcutaneous and intramuscular

**Figure 12.** Cardiac ultrasound shows cysticercus in the cardiac muscle.

"cigar-shape" calcifications.

**Figure 9.** Shows multiple subcutaneous nodules in the left arm some of them were located in the biceps brachialis. (Photo taken by H Foyaca-Sibat. Source: H. Foyaca-Sibat & L. Ibanez-Valdes: Generalized Cysticercosis With Cardiac Involvement. *The Internet Journal of Neurology.* 2007 Volume 7 Number 2.)

**Figure 10.** Shows a nodular lesion in the left hemiface at the zigomatic arch level removed by surgical procedure and histological examination confirmed subcutaneous cysticercosis (Photo taken by H Foyaca-Sibat. Source: H. Foyaca-Sibat & L. Ibanez-Valdes: Generalized Cysticercosis With Cardiac Involvement. *The Internet Journal of Neurology.* 2007 Volume 7 Number 2. Patient written consent is obtained)

obtained)

**Figure 9.** Shows multiple subcutaneous nodules in the left arm some of them were located in the biceps brachialis. (Photo taken by H Foyaca-Sibat. Source: H. Foyaca-Sibat & L. Ibanez-Valdes: Generalized Cysticercosis With Cardiac Involvement. *The Internet Journal of Neurology.* 2007 Volume 7 Number 2.)

**Figure 10.** Shows a nodular lesion in the left hemiface at the zigomatic arch level removed by surgical procedure and histological examination confirmed subcutaneous cysticercosis (Photo taken by H Foyaca-Sibat. Source: H. Foyaca-Sibat & L. Ibanez-Valdes: Generalized Cysticercosis With Cardiac Involvement. *The Internet Journal of Neurology.* 2007 Volume 7 Number 2. Patient written consent is

**Figure 11.** Plain X-ray of the chest and upper limbs show multiple subcutaneous and intramuscular "cigar-shape" calcifications.

**Figure 12.** Cardiac ultrasound shows cysticercus in the cardiac muscle.

Ultrasography to diagnose subcutaneous cysticercosis was introduced a few years ago and from the beginning we also found some limitations to differentiated cysticercosis from lymphadenopathies, neurofibromas, and epidermoid cysts [41] however at the present moment is possible to confirm a diagnosis for subcutaneous nodules showing the scolex inside. Echocardiogram has a hight level of accuracy in cardiocysticercosis and we could confirm it in patients.

What is a Low Frequency of the Disseminated

Cysticercosis Suggests that Neurocysticercosis is Going to Disappear? 175

expressing anti-Semitic sentiment and young people participating in radical social and political mobilization among others [76]. How many of these hidden groups suffer of *T.* 

In our opinion, even when the poverty disappear from underdeveloped countries and every person get a proper access to clean and safe water, living in very good hygienic conditions under an ideal health education and primary health care sustainable system program, we could not proclaim that the CC has been eradicated because of that important number of human beings sharing our planet whose epidemiological situation remain unknown. We are

Congo region is included in our Cysticercosis Working Group for Eastern and Southern Africa (CGWESA). The Bambuti are pygmy hunter-gatherers, and are one of the oldest indigenous people of the Congo region of Africa. They are composed by bands which are relatively small in size, ranging from 15 to 60 people. The Bambuti population totals about 30,000 to 40,000 people. The forest where they live is a moist, humid region strewn with rivers and lakes. Disease is prevalent in the forests and can spread quickly, killing not only humans, but plants, and animals, the major source of food, as well. Their animal foodstuffs include crabs, shellfish, ants, larvae, snails, pigs, antelopes, monkeys, fishes, and honey. The vegetable component of their diet includes wild yams, berries, fruits, roots, leaves, and cola

Many parts of the Americas are still populated by indigenous Americans; some countries have sizable populations, especially Bolivia, Peru, Mexico, Guatemala, Colombia, and Ecuador. Some indigenous peoples still live in relative isolation from Western society, and a few are still counted as uncontacted peoples [78]. Most uncontacted communities are located in densely forested areas in South America and New Guinea. Knowledge of the existence of these groups comes mostly from infrequent and sometimes violent encounters with neighboring tribes, and from aerial footage. Isolated tribes may lack immunity to common diseases, which can kill 50 to 80 per cent of their people after contact [79] but nobody knows

The Sentinelese continues to actively and violently reject contact. They live on North Sentinel Island, a small and remote island which lies to the west of the southern part of South Andaman Island. They are thus considered the most isolated people in the world, and they are likely to remain so [73]. The current epidemiological situation on parasitic zoonoses

In Paraguay remains perhaps as many as 300 Totobiegosode who have not been contacted; they belong to the Ayoreo ethnicity, which numbers around 2,000. As of 2006, the presence of five uncontacted groups was confirmed in Bolivia; three more uncontacted groups are believed to exist. On January 18, 2007, the National Indian Foundation reported that it had confirmed the presence of 67 uncontacted tribes in Brazil, up from 40 in 2005. With this reported increase, Brazil has surpassed the island of New Guinea as the region having the highest number of uncontacted tribes. Ecuador continues to be the country with the largest number of uncontacted people killed since 2000. After Brazil and New Guinea (Papua New

aware about an important number of individuals that still remains in that situation.

nuts [77]. Anyone is infected by *T. solium*?, Who knows? We do not know.

*solium* cysticercosis? We do not know.

how many parasites are affecting them.

of Sentinel is still unknown.

All South African patients presenting CCD also suffrering of symptomatic epilepsy because all had cerebro-cortical lesions caused by *T. solium.* 

## **4. Disseminated cysticercosis and epilepsy**

Epilepsy is also the most common clinical manifestation of NCC in our region [61-64] and it prevalence has been well documented in several communities of Eastern Cape Province in South Africa. [65-71]. While disseminated cysticercosis is an exceptional expression of CC characterized by high morbidity due to massive symptomatic parasite burden in the central nervous system, striated muscles, subcutaneous tissues and other organs. Less than 50 such cases have been reported worldwide and fewer than 10 children. In 2010, Kumar and collaborators [72] reported their findings on whole-body MRI as a diagnostic tool for extensively disseminated cysticercosis in a child and highlighted its role in diagnosis and management of this pleomorphic disease. Unfortunately, MRI is not available for most of patients with CCD worldwide and it can not replace the traditional way of diagnosis based on CT scan of the brain for NCC and plain X-ray for muscular and subcutaneous cysticercosis. Diagnosis of NCC can be made by Del Brutto's recommendation [73] but novel criterias suggested by Carpio in this boork, should be considerd

## **5. The disseminated cysticercosis going to disappear in the near future?**

Before moving to another topic we would like to answer that research question.

We are convinced that CC will be eradicated from our planet, but today this is far from happen especially in some countries of Sub-Saharan Africa, Asia and Latin America, where there are communities who live, or have lived, either by choice (peoples living in voluntary isolation) or by circumstance, without significant contact with globalized civilization, therefore their current epidemiological situation is unknown and we even don't know how many peoples are infected by parasites.

We know that *T. solium* infested man long before there was any evidence of animal domestication (See chapter 1) [74, 75] by which we can assume that even in communities where very backward the taming of animals there is no zoonotic diseases may be acquired by other means.

For the other hand, there is an important number of hidden groups of peoples such as: racist groups and racist activists; drug traffickers in prisons; natives from the Central African Rain Forest; male and female prisoners and guards from a number of prison colonies; nationalists expressing anti-Semitic sentiment and young people participating in radical social and political mobilization among others [76]. How many of these hidden groups suffer of *T. solium* cysticercosis? We do not know.

174 Novel Aspects on Cysticercosis and Neurocysticercosis

all had cerebro-cortical lesions caused by *T. solium.* 

**4. Disseminated cysticercosis and epilepsy** 

criterias suggested by Carpio in this boork, should be considerd

many peoples are infected by parasites.

by other means.

confirm it in patients.

Ultrasography to diagnose subcutaneous cysticercosis was introduced a few years ago and from the beginning we also found some limitations to differentiated cysticercosis from lymphadenopathies, neurofibromas, and epidermoid cysts [41] however at the present moment is possible to confirm a diagnosis for subcutaneous nodules showing the scolex inside. Echocardiogram has a hight level of accuracy in cardiocysticercosis and we could

All South African patients presenting CCD also suffrering of symptomatic epilepsy because

Epilepsy is also the most common clinical manifestation of NCC in our region [61-64] and it prevalence has been well documented in several communities of Eastern Cape Province in South Africa. [65-71]. While disseminated cysticercosis is an exceptional expression of CC characterized by high morbidity due to massive symptomatic parasite burden in the central nervous system, striated muscles, subcutaneous tissues and other organs. Less than 50 such cases have been reported worldwide and fewer than 10 children. In 2010, Kumar and collaborators [72] reported their findings on whole-body MRI as a diagnostic tool for extensively disseminated cysticercosis in a child and highlighted its role in diagnosis and management of this pleomorphic disease. Unfortunately, MRI is not available for most of patients with CCD worldwide and it can not replace the traditional way of diagnosis based on CT scan of the brain for NCC and plain X-ray for muscular and subcutaneous cysticercosis. Diagnosis of NCC can be made by Del Brutto's recommendation [73] but novel

**5. The disseminated cysticercosis going to disappear in the near future?** 

We are convinced that CC will be eradicated from our planet, but today this is far from happen especially in some countries of Sub-Saharan Africa, Asia and Latin America, where there are communities who live, or have lived, either by choice (peoples living in voluntary isolation) or by circumstance, without significant contact with globalized civilization, therefore their current epidemiological situation is unknown and we even don't know how

We know that *T. solium* infested man long before there was any evidence of animal domestication (See chapter 1) [74, 75] by which we can assume that even in communities where very backward the taming of animals there is no zoonotic diseases may be acquired

For the other hand, there is an important number of hidden groups of peoples such as: racist groups and racist activists; drug traffickers in prisons; natives from the Central African Rain Forest; male and female prisoners and guards from a number of prison colonies; nationalists

Before moving to another topic we would like to answer that research question.

In our opinion, even when the poverty disappear from underdeveloped countries and every person get a proper access to clean and safe water, living in very good hygienic conditions under an ideal health education and primary health care sustainable system program, we could not proclaim that the CC has been eradicated because of that important number of human beings sharing our planet whose epidemiological situation remain unknown. We are aware about an important number of individuals that still remains in that situation.

Congo region is included in our Cysticercosis Working Group for Eastern and Southern Africa (CGWESA). The Bambuti are pygmy hunter-gatherers, and are one of the oldest indigenous people of the Congo region of Africa. They are composed by bands which are relatively small in size, ranging from 15 to 60 people. The Bambuti population totals about 30,000 to 40,000 people. The forest where they live is a moist, humid region strewn with rivers and lakes. Disease is prevalent in the forests and can spread quickly, killing not only humans, but plants, and animals, the major source of food, as well. Their animal foodstuffs include crabs, shellfish, ants, larvae, snails, pigs, antelopes, monkeys, fishes, and honey. The vegetable component of their diet includes wild yams, berries, fruits, roots, leaves, and cola nuts [77]. Anyone is infected by *T. solium*?, Who knows? We do not know.

Many parts of the Americas are still populated by indigenous Americans; some countries have sizable populations, especially Bolivia, Peru, Mexico, Guatemala, Colombia, and Ecuador. Some indigenous peoples still live in relative isolation from Western society, and a few are still counted as uncontacted peoples [78]. Most uncontacted communities are located in densely forested areas in South America and New Guinea. Knowledge of the existence of these groups comes mostly from infrequent and sometimes violent encounters with neighboring tribes, and from aerial footage. Isolated tribes may lack immunity to common diseases, which can kill 50 to 80 per cent of their people after contact [79] but nobody knows how many parasites are affecting them.

The Sentinelese continues to actively and violently reject contact. They live on North Sentinel Island, a small and remote island which lies to the west of the southern part of South Andaman Island. They are thus considered the most isolated people in the world, and they are likely to remain so [73]. The current epidemiological situation on parasitic zoonoses of Sentinel is still unknown.

In Paraguay remains perhaps as many as 300 Totobiegosode who have not been contacted; they belong to the Ayoreo ethnicity, which numbers around 2,000. As of 2006, the presence of five uncontacted groups was confirmed in Bolivia; three more uncontacted groups are believed to exist. On January 18, 2007, the National Indian Foundation reported that it had confirmed the presence of 67 uncontacted tribes in Brazil, up from 40 in 2005. With this reported increase, Brazil has surpassed the island of New Guinea as the region having the highest number of uncontacted tribes. Ecuador continues to be the country with the largest number of uncontacted people killed since 2000. After Brazil and New Guinea (Papua New

Guinea and Iriyan Jaya), Peru has the largest number of uncontacted tribes in the world [79] and there is not available information about their current situation related to incidence or prevalence of infectious diseases.

What is a Low Frequency of the Disseminated

Cysticercosis Suggests that Neurocysticercosis is Going to Disappear? 177

detail the most important features of the cysticercosis of the cardiac muscle followed by

As before-mentioned DCC means distribution of cysticerci along to the brain, skeletal muscles and subcutaneous tissues. In this part of the chapter we will discuss about other

Long before the emergence of computerized tomography knew each other from the first cases with cardiocysticercosis with other associated diseases of the heart in living patients [98], even with silent presentation [ 99] or disorders of cardiac conduction [100]. The lack of brain imaging studies did not confirm the presence of associated NCC in those cases. Spent 10 years and then was possible achieve a better image of the heart and confirm best results with the use of praziquantel in this type of presentation associated with DCC [101]. Some parasites may directly or indirectly affect various anatomical structures of the heart, with infections manifested as myocarditis, pericarditis, pancarditis, or pulmonary hypertension. In other situations, parasitic infections may have more direct effects on various structures of the heart (myocardium, pericardium, endocardium, or the cardiac vasculature). The involvement of the myocardium may lead to myocarditis or different types of cardiomyopathies (i.e., dilated or restrictive). When the pericardium is affected, it may lead to pericarditis, pericardial effusion, cardiac tamponade, or constrictive pericarditis. Due to growing migration, population displacement, and travel, clinicians anywhere around the globe must be aware of the potential cardiac manifestations of parasitic diseases [57,102].

The myocardial inflammatory response is variable, resulting in granuloma formation and fibrosis, which subsequently leads to arrhythmias and conduction abnormalities either

Cardiac involvement in cysticercosis was thought to be rare, but autopsy studies have shown a prevalence of 20 to 25% in patients with concomitant documented neurocysticercosis [104,105]. Cardiac cysticercosis is often asymptomatic and discovered during cardiac surgery or at autopsy. Cysticerci are usually multiple and randomly distributed in cardiac tissues, including the subpericardium, subendocardium, and myocardium [104]. Rarely, a single cardiac cyst may be present [102]. The computed tomography and ultrasonographic studies have demonstrated a fundamental importance in the diagnosis of cysticercosis of the heart according with results reported by other authors

The DCC also affects children severely, an example of that is in the five years old patient reported by Asrani [108] in 2004, who presented subcutaneous swellings all over her body. The swellings were each between 0.5 and 2 cm in size. There were only 1 or 2 to start with on the back of the neck, and then they gradually increased to involve the chest, head, neck, arms, and legs in 2 to 3 months. These swellings were accompanied by onset of abnormal behavior in the form of irritability, talkativeness, disobeying commands, and bladder incontinence (secondary enuresis). Also, there was abnormal weight gain of 10 kg. The patient also had decreased vision for one month. Examination revealed multiple

spontaneously or during treatment [57,103-105].

[57,106-108] and own personal experience [41, 50].

cysticercosis in the lungs and the combination of both presentations.

organs affected.

Today there are more than 150 million tribal peoples worldwide, including at least 70 uncontacted tribes, living in 60 countries. Some organization such as: Survival International supports these endangered tribes on a global level, with campaigns established in America, Africa and Asia [80, 81]. In our opinion, many of them can be parasitized by *T. solium* as was the *H ergatus* more than a million of the year ago (See chapter 1).

Due to globalization, a growing number of uncontrolled immigrants from an endemic area in Latin America come to the USA every day, significantly increasing the number of cases of NCC in the country especially in Texas and California [82-91] where a total of 1494 patients with NCC were confirmed between 1980 and 2004 of which 66% suffered from epilepsy, 16% had a obstructive hydrocephalus and the 15% headache due to intraparechymal NCC (91 % ), intraventricular (6 %) or subarachnoid (2 %) either because travelled to endemic areas, were of Hispanic origin or had any contact with carriers of the parasite [92-93]. In Latin America has been described the existence of NCC in 18 countries with an estimated 350,000 patients infected by the complex CC/TE. In 2008, Pawlowski stipulated that in the world would have approximately 2.5 million people infected T solium and at least 20 million with CC [70].

Disseminate presentations of cysticercosis is not common in our region, only six patients were previously confirmed and four of them reported to the medical literature [41, 50, 58, 59]. Typical manifestation is subcutaneous cyst present as nodules that tend to be asymptomatic [41, 50]. The natural history of the infection remains unknown up to date. However, is well known that most cysticercus complete their development within two to four months after larval entry living there months to years, and their locations in order of frequency are the central nervous system, subcutaneous tissues, striated muscle, heart, orbits, and other tissues. Human cysticercosis is acquired after eating food contaminated with fertilized eggs excreted in the feces of Taenia's carriers. In humans the most common routes of infection (cysticercosis) are ingestion of *T .solium* eggs from contamined food and fecal-oral auto-infestation in patients harbouring the adult parasite in their intestines. While the cysts can develop in most human tissue, they have a predilection for CNS. Cysticercosis is estimated to affect approximately 50 million people worldwide and is common in rural South Africa [1, 10-15, 61-71].

## **6. Disseminated cysticercosis with involvement of other organs**

## **6.1. Cardiocysticercosis**

The vast majority of patients with DCC reported to international medical literature are from India and although CC can affect almost any tissue the most frequently reported are the central nervous system and skeletal muscles [41,50,58]. Myocardial cysticercosis is rare [94,95] but its diagnosis is easier with modern radiological tools [95-97] . We will see in more detail the most important features of the cysticercosis of the cardiac muscle followed by cysticercosis in the lungs and the combination of both presentations.

176 Novel Aspects on Cysticercosis and Neurocysticercosis

prevalence of infectious diseases.

million with CC [70].

South Africa [1, 10-15, 61-71].

**6.1. Cardiocysticercosis** 

Guinea and Iriyan Jaya), Peru has the largest number of uncontacted tribes in the world [79] and there is not available information about their current situation related to incidence or

Today there are more than 150 million tribal peoples worldwide, including at least 70 uncontacted tribes, living in 60 countries. Some organization such as: Survival International supports these endangered tribes on a global level, with campaigns established in America, Africa and Asia [80, 81]. In our opinion, many of them can be parasitized by *T. solium* as was

Due to globalization, a growing number of uncontrolled immigrants from an endemic area in Latin America come to the USA every day, significantly increasing the number of cases of NCC in the country especially in Texas and California [82-91] where a total of 1494 patients with NCC were confirmed between 1980 and 2004 of which 66% suffered from epilepsy, 16% had a obstructive hydrocephalus and the 15% headache due to intraparechymal NCC (91 % ), intraventricular (6 %) or subarachnoid (2 %) either because travelled to endemic areas, were of Hispanic origin or had any contact with carriers of the parasite [92-93]. In Latin America has been described the existence of NCC in 18 countries with an estimated 350,000 patients infected by the complex CC/TE. In 2008, Pawlowski stipulated that in the world would have approximately 2.5 million people infected T solium and at least 20

Disseminate presentations of cysticercosis is not common in our region, only six patients were previously confirmed and four of them reported to the medical literature [41, 50, 58, 59]. Typical manifestation is subcutaneous cyst present as nodules that tend to be asymptomatic [41, 50]. The natural history of the infection remains unknown up to date. However, is well known that most cysticercus complete their development within two to four months after larval entry living there months to years, and their locations in order of frequency are the central nervous system, subcutaneous tissues, striated muscle, heart, orbits, and other tissues. Human cysticercosis is acquired after eating food contaminated with fertilized eggs excreted in the feces of Taenia's carriers. In humans the most common routes of infection (cysticercosis) are ingestion of *T .solium* eggs from contamined food and fecal-oral auto-infestation in patients harbouring the adult parasite in their intestines. While the cysts can develop in most human tissue, they have a predilection for CNS. Cysticercosis is estimated to affect approximately 50 million people worldwide and is common in rural

**6. Disseminated cysticercosis with involvement of other organs** 

The vast majority of patients with DCC reported to international medical literature are from India and although CC can affect almost any tissue the most frequently reported are the central nervous system and skeletal muscles [41,50,58]. Myocardial cysticercosis is rare [94,95] but its diagnosis is easier with modern radiological tools [95-97] . We will see in more

the *H ergatus* more than a million of the year ago (See chapter 1).

As before-mentioned DCC means distribution of cysticerci along to the brain, skeletal muscles and subcutaneous tissues. In this part of the chapter we will discuss about other organs affected.

Long before the emergence of computerized tomography knew each other from the first cases with cardiocysticercosis with other associated diseases of the heart in living patients [98], even with silent presentation [ 99] or disorders of cardiac conduction [100]. The lack of brain imaging studies did not confirm the presence of associated NCC in those cases. Spent 10 years and then was possible achieve a better image of the heart and confirm best results with the use of praziquantel in this type of presentation associated with DCC [101]. Some parasites may directly or indirectly affect various anatomical structures of the heart, with infections manifested as myocarditis, pericarditis, pancarditis, or pulmonary hypertension. In other situations, parasitic infections may have more direct effects on various structures of the heart (myocardium, pericardium, endocardium, or the cardiac vasculature). The involvement of the myocardium may lead to myocarditis or different types of cardiomyopathies (i.e., dilated or restrictive). When the pericardium is affected, it may lead to pericarditis, pericardial effusion, cardiac tamponade, or constrictive pericarditis. Due to growing migration, population displacement, and travel, clinicians anywhere around the globe must be aware of the potential cardiac manifestations of parasitic diseases [57,102].

The myocardial inflammatory response is variable, resulting in granuloma formation and fibrosis, which subsequently leads to arrhythmias and conduction abnormalities either spontaneously or during treatment [57,103-105].

Cardiac involvement in cysticercosis was thought to be rare, but autopsy studies have shown a prevalence of 20 to 25% in patients with concomitant documented neurocysticercosis [104,105]. Cardiac cysticercosis is often asymptomatic and discovered during cardiac surgery or at autopsy. Cysticerci are usually multiple and randomly distributed in cardiac tissues, including the subpericardium, subendocardium, and myocardium [104]. Rarely, a single cardiac cyst may be present [102]. The computed tomography and ultrasonographic studies have demonstrated a fundamental importance in the diagnosis of cysticercosis of the heart according with results reported by other authors [57,106-108] and own personal experience [41, 50].

The DCC also affects children severely, an example of that is in the five years old patient reported by Asrani [108] in 2004, who presented subcutaneous swellings all over her body. The swellings were each between 0.5 and 2 cm in size. There were only 1 or 2 to start with on the back of the neck, and then they gradually increased to involve the chest, head, neck, arms, and legs in 2 to 3 months. These swellings were accompanied by onset of abnormal behavior in the form of irritability, talkativeness, disobeying commands, and bladder incontinence (secondary enuresis). Also, there was abnormal weight gain of 10 kg. The patient also had decreased vision for one month. Examination revealed multiple

subcutaneous nodules between 0.5 and 2 cm in size over the forehead, abdomen, back, and legs. She weighed 29 kg (19 kg in a previous month). Her vision was 6/30 according to the Snellen chart. The patient was referred for sonography of the abdomen. The abdominal visceras on examination with a 3.5-MHz convex transducer were unremarkable, but the abdominal wall showed some tiny cystic lesions. Further examination using a highfrequency 7- to 12-MHz linear transducer (HDI 3000; Philips Medical Systems, Andover, MA) showed innumerable oval to circular, 0.5- to 1.5-cm, predominantly anechoic lesions in the subcutaneous tissues and abdominal wall muscles. Similar lesions were distributed over the back, neck, thighs, calves, legs, axillae, forearms, and arms. Sonography of the tongue also revealed similar multiple lesions. There was no increased vascularity surrounding these lesions on color Doppler examination. Only the palms and soles seemed to be uninvolved. A sonographic diagnosis of disseminated muscular cysticercosis was made. Whole-body contrast-enhanced computed tomography (CT) confirmed the sonographic findings and showed the classic "starry night" in the brain. The entire muscular system and the subcutaneous tissue had hypodense, nonenhancing, approximately 1-cm lesions without calcification, suggesting that all the cysts were live. Magnetic resonance imaging (MRI) of the brain,orbit, and musculoskeletal system showed multiple hyperintense lesions on transverse relaxation time (T2)-weighted images.

What is a Low Frequency of the Disseminated

Cysticercosis Suggests that Neurocysticercosis is Going to Disappear? 179

visible at the posterior wall of the cyst. One internal septation of the mass was noted. After gadolinium administration, no enhancement was present. The patient underwent biopsy of the mass. Histologic evaluation showed a fluid-containing cystic structure, which exhibited a single protoscolex consistent with a cysticercus. These authors mentioned that involvement of the heart by *T. solium* is very rare and is usually diagnosed postmortem. After review the international medical literature we agree it is rare but diagnosis of cardiocysticercosis is made accurately in most of patient seeking for medical attention. Shogan's report is extremelly uncommon because his patient had not an associated DCC or NCC [112], but without doubt the cardiocysticercosis must always be borne in mind in patients with similar presentations and because it is commonly associated with intraparenchymal NCC therefore to look for epilepsy is strong recommended. (See figure 13)

**Figure 13.** Graphical representation of other affected organs and location of cysticerci in the CNS.

Most of patients presenting pulmonary cysticercosis have multiple rather than solitary pulmonary nodules (a "spot" on the lung that is less than 3 cm or 1½ inches in diameter by

The differential diagnosis of multiple pulmonary nodules is large and includes congenital and inherited disorders, malignancy, infectious etiologies, noninfectious granulomatous and inflammatory conditions, among many others. Disseminated cysticercosis with pulmonary involvement should be suspected in any patient presenting with multiple pulmonary nodules who is an immigrant from an endemic region or an individual who has resided in

Taking into consideration that the only infectious disease causing multiple pulmonary nodules and associated cutaneous manifestation are: blastomycosis, coccidiomycosis,

**6.2. Pulmonary cysticercosis** 

one of the countries where cysticercosis is endemic [113].

chest X-ray).

A biopsy of the subcutaneous nodules showed classic cysts with a central scolex. Multiple ocular cysts were also found on ocular examination. After this, the patient started receiving steroids and anthelminthic agents. Within 4 weeks of starting treatment, the patient's weight decreased by 10 kg (to 19 kg again). She became quiet and began obeying commands. Her vision became 6/12 (Snellen chart). Echocardiography revealed extensive myocardial involvement. The major differential diagnosis is hydatid cyst, which is usually larger and multilocular. Antihelminthic treatment may result in pericystic inflammatory reaction which might worsen the clinical state [109].

Other parasite can also affect the myocardium and the pericardium as we have previously pointed out, and that the most widely studied parasitic infection affecting the heart is Chagas' disease or American trypanosomiasis but African trypanosomiasis may also cause a myocarditis. The protozoan parasite, Entamoeba histolytica rarely causes a pericarditis while Toxoplasma gondii may cause myocarditis, usually in immunocompromised hosts. The larval forms of the tapeworms Echinococcus and *T. solium* may cause space occupying lesions of the heart. Severe infection with the nematode Trichinella spiralis may cause myocarditis [110].

Not always cardiocysticercosis is part of the disseminated cysticercosis as reported by Eberly and collaborators and it may occur as an isolated infection of the left ventricle [111].

In 2009, Shogan [112] reported a 17-year-old boy from Cameroon presenting a sessile cystic cardiac mass. A CT scan revealed a 1.0 × 1.5 cm nonenhancing, exophytic, ovoid muralbased fluid density in the left ventricle along the endocardial surface of the anterior wall near the anteroseptal basal region. On T1-weighted MR images, the mass was hypointense. On T2-weighted MR images, the mass was hyperintense, with a rounded hypointense signal visible at the posterior wall of the cyst. One internal septation of the mass was noted. After gadolinium administration, no enhancement was present. The patient underwent biopsy of the mass. Histologic evaluation showed a fluid-containing cystic structure, which exhibited a single protoscolex consistent with a cysticercus. These authors mentioned that involvement of the heart by *T. solium* is very rare and is usually diagnosed postmortem. After review the international medical literature we agree it is rare but diagnosis of cardiocysticercosis is made accurately in most of patient seeking for medical attention. Shogan's report is extremelly uncommon because his patient had not an associated DCC or NCC [112], but without doubt the cardiocysticercosis must always be borne in mind in patients with similar presentations and because it is commonly associated with intraparenchymal NCC therefore to look for epilepsy is strong recommended. (See figure 13)

**Figure 13.** Graphical representation of other affected organs and location of cysticerci in the CNS.

#### **6.2. Pulmonary cysticercosis**

178 Novel Aspects on Cysticercosis and Neurocysticercosis

transverse relaxation time (T2)-weighted images.

which might worsen the clinical state [109].

myocarditis [110].

subcutaneous nodules between 0.5 and 2 cm in size over the forehead, abdomen, back, and legs. She weighed 29 kg (19 kg in a previous month). Her vision was 6/30 according to the Snellen chart. The patient was referred for sonography of the abdomen. The abdominal visceras on examination with a 3.5-MHz convex transducer were unremarkable, but the abdominal wall showed some tiny cystic lesions. Further examination using a highfrequency 7- to 12-MHz linear transducer (HDI 3000; Philips Medical Systems, Andover, MA) showed innumerable oval to circular, 0.5- to 1.5-cm, predominantly anechoic lesions in the subcutaneous tissues and abdominal wall muscles. Similar lesions were distributed over the back, neck, thighs, calves, legs, axillae, forearms, and arms. Sonography of the tongue also revealed similar multiple lesions. There was no increased vascularity surrounding these lesions on color Doppler examination. Only the palms and soles seemed to be uninvolved. A sonographic diagnosis of disseminated muscular cysticercosis was made. Whole-body contrast-enhanced computed tomography (CT) confirmed the sonographic findings and showed the classic "starry night" in the brain. The entire muscular system and the subcutaneous tissue had hypodense, nonenhancing, approximately 1-cm lesions without calcification, suggesting that all the cysts were live. Magnetic resonance imaging (MRI) of the brain,orbit, and musculoskeletal system showed multiple hyperintense lesions on

A biopsy of the subcutaneous nodules showed classic cysts with a central scolex. Multiple ocular cysts were also found on ocular examination. After this, the patient started receiving steroids and anthelminthic agents. Within 4 weeks of starting treatment, the patient's weight decreased by 10 kg (to 19 kg again). She became quiet and began obeying commands. Her vision became 6/12 (Snellen chart). Echocardiography revealed extensive myocardial involvement. The major differential diagnosis is hydatid cyst, which is usually larger and multilocular. Antihelminthic treatment may result in pericystic inflammatory reaction

Other parasite can also affect the myocardium and the pericardium as we have previously pointed out, and that the most widely studied parasitic infection affecting the heart is Chagas' disease or American trypanosomiasis but African trypanosomiasis may also cause a myocarditis. The protozoan parasite, Entamoeba histolytica rarely causes a pericarditis while Toxoplasma gondii may cause myocarditis, usually in immunocompromised hosts. The larval forms of the tapeworms Echinococcus and *T. solium* may cause space occupying lesions of the heart. Severe infection with the nematode Trichinella spiralis may cause

Not always cardiocysticercosis is part of the disseminated cysticercosis as reported by Eberly and collaborators and it may occur as an isolated infection of the left ventricle [111].

In 2009, Shogan [112] reported a 17-year-old boy from Cameroon presenting a sessile cystic cardiac mass. A CT scan revealed a 1.0 × 1.5 cm nonenhancing, exophytic, ovoid muralbased fluid density in the left ventricle along the endocardial surface of the anterior wall near the anteroseptal basal region. On T1-weighted MR images, the mass was hypointense. On T2-weighted MR images, the mass was hyperintense, with a rounded hypointense signal Most of patients presenting pulmonary cysticercosis have multiple rather than solitary pulmonary nodules (a "spot" on the lung that is less than 3 cm or 1½ inches in diameter by chest X-ray).

The differential diagnosis of multiple pulmonary nodules is large and includes congenital and inherited disorders, malignancy, infectious etiologies, noninfectious granulomatous and inflammatory conditions, among many others. Disseminated cysticercosis with pulmonary involvement should be suspected in any patient presenting with multiple pulmonary nodules who is an immigrant from an endemic region or an individual who has resided in one of the countries where cysticercosis is endemic [113].

Taking into consideration that the only infectious disease causing multiple pulmonary nodules and associated cutaneous manifestation are: blastomycosis, coccidiomycosis,

tuberculosis and cysticercosis then the differential diagnosis of pulmonary cysticercosis is very easy if DCC/NCC complex is present.

What is a Low Frequency of the Disseminated

Cysticercosis Suggests that Neurocysticercosis is Going to Disappear? 181

clinical symptoms (as before-mentioned) and they suggest open lung biopsy for confirmation, for treatment PZQ (50 mg/kg per day for 15 days) and to consider pulmonary cysticercosis as a diagnostic possibility in patients with nodular infiltrates in the lungs,

In 2011, a radiologist from Punjab, India [119] reported a well documented 28-year-old man presented with a history of fever, dry cough, headache and decreased responsiveness over 7 days. One year previously, he had suffered seizures and was hospitalised in another centre with an initial diagnosis of NCC and received treatment for it. MRI at that time depicted numerous ring enhancing lesions with eccentric scolex ('cyst with dot' appearance) in the cerebral hemispheres, subarachnoid space and orbits. The patient had a history of pork intake and intravenous drug misuse. Physical examination at the time of admission revealed small, movable, painless subcutaneous nodules that were palpable over the arms, axillae and trunk. No abnormalities were noted on auscultation of the lungs. Blood and urine cultures were negative. Sputum analysis was normal. A chest x-ray revealed small nodules in both lungs. This is the first report about pulmonary cysticercosis in a patient presenting

Next patient was reported by Strawter et.al., [113] a few weeks ago. They studied a 31-yearold incarcerated Hispanic male presented with a nonproductive cough for several months and one episode of blood tinged sputum. He admitted to weight loss and night sweats, headaches, and visual disturbances. He was an immigrant from Honduras and lived in Arizona for the past 15 years. He had chronic hepatitis C infection and was receiving treatment with pegylated interferon-alfa-2a (IFN-α) and ribavirin. His symptoms began one month after initiating antiviral therapy.They confirmed pulmonary nodules with cutaneous manifestations and concluded that: Disseminated cysticercosis with pulmonary involvement should be suspected in any patient presenting with multiple pulmonary nodules who is an immigrant from an endemic region or an individual who has resided in one of the countries where cysticercosis is present or when NCC is well documented. Lung are the second

thoracic organ affected by *T solium* cysticercosis preceded by the heart (See Figure 14)

**Figure 14.** Thoracic organs involved in disseminated cysticercosis

especially in endemic areas, until such infiltrates are otherwise explained [118].

DCC and subarachnoid NCC.

In 1971, Bassermann [114] found some radiographic problems in pulmonary cysticercosis because at that time computed tomography system was not available. Siemens Medical Solutions (then known as Siemens Medical Engineering) presented the first machine commercially available in May 1974. Therefore confirmation of NCC by imagenology was not done. Other authors studied patients with pulmonary cysticercosis at different times and all of them agree that this is an uncommon condition [115-119].

Schotz and Mentis [116] in 1987 reported a 48-years-old black man admitted to hospital with a 2-month history of a painless mass on the left side of his neck. He also complained of mild attacks of paroxysmal dyspnoea at night and progressive deafness of his left ear. On direct questioning the patient referred a few grand mal seizures during the six years before admission. No other complaints or previous illnesses of note were elicited. The patient was born in Transkei (near to Mthatha in South Africa) and had travelled throughout many parts of the country seeking work, which he secured in the gold mines mainly. He ate a variety of meats including pork and smoked and drank excessively at times.On examination he appeared healthy and was of normal build. The pulse rate was 72/min, blood pressure 110/60 mmHg and temperature 37,2°C. Numerous subcutaneous nodules, ranging in size from 1 cm to 3 cm in diameter, which were well circumscribed and freely movable, could be palpated all over the neck, chest and legs. A bigger mass was palpable inferior to the left ear, extending down the left side of the neck. There was conduction deafness of the left ear. On chest auscultation a few fine crepitations were audible at the bases of both lungs. Computed tomography of the brain, neck, thorax and upper abdomen revealed extensive organ involvement, including brain, thyroid, lungs, pancreas, kidney, spleen and muscle; and biopsies of two of the subcutaneous nodules were histologically pathognomonic of *T. solium* cysticercosis. The patient received praziquantel (Biltricide; Bayer-Miles) 1,75 g twice daily and prednisone 20 mg per day for 4 weeks. He was reassessed radiologically after 4 weeks. There was marked regression in the size and numbers of nodules in the lung fields. Clinically, most of the subcutaneous nodules had disappeared. Authors highlighted that the radiological appearance of cysticercosis in the lung cannot be differentiated from other parasitic infections, e.g. echinococcosis, pentastomiasis, paragonimiasis and histoplasmosis, or conditions such as tuberculosis, alveolar carcinoma and metastases due to the varying reaction of the lung tissue and difference in size of the larvae [114]. Pulmonary sparganosis should be exluded from the list of differential diagnosis as well [120]. Although rare, pulmonary cysticercosis does occur and in developing countries should not be overlooked in the differential diagnosis of multiple lung opacities.

From our knowledge, this is the only patient with renal involvement reported to the medical literature ever.

From one case reported in Korea, authors concluded that the presence of cysticerci are difficult to discern from pulmonary infiltrates, because other parasitic infestations or tuberculosis, as well as metastatic lesions, produce similar chest X-ray findings and similar clinical symptoms (as before-mentioned) and they suggest open lung biopsy for confirmation, for treatment PZQ (50 mg/kg per day for 15 days) and to consider pulmonary cysticercosis as a diagnostic possibility in patients with nodular infiltrates in the lungs, especially in endemic areas, until such infiltrates are otherwise explained [118].

180 Novel Aspects on Cysticercosis and Neurocysticercosis

very easy if DCC/NCC complex is present.

tuberculosis and cysticercosis then the differential diagnosis of pulmonary cysticercosis is

In 1971, Bassermann [114] found some radiographic problems in pulmonary cysticercosis because at that time computed tomography system was not available. Siemens Medical Solutions (then known as Siemens Medical Engineering) presented the first machine commercially available in May 1974. Therefore confirmation of NCC by imagenology was not done. Other authors studied patients with pulmonary cysticercosis at different times

Schotz and Mentis [116] in 1987 reported a 48-years-old black man admitted to hospital with a 2-month history of a painless mass on the left side of his neck. He also complained of mild attacks of paroxysmal dyspnoea at night and progressive deafness of his left ear. On direct questioning the patient referred a few grand mal seizures during the six years before admission. No other complaints or previous illnesses of note were elicited. The patient was born in Transkei (near to Mthatha in South Africa) and had travelled throughout many parts of the country seeking work, which he secured in the gold mines mainly. He ate a variety of meats including pork and smoked and drank excessively at times.On examination he appeared healthy and was of normal build. The pulse rate was 72/min, blood pressure 110/60 mmHg and temperature 37,2°C. Numerous subcutaneous nodules, ranging in size from 1 cm to 3 cm in diameter, which were well circumscribed and freely movable, could be palpated all over the neck, chest and legs. A bigger mass was palpable inferior to the left ear, extending down the left side of the neck. There was conduction deafness of the left ear. On chest auscultation a few fine crepitations were audible at the bases of both lungs. Computed tomography of the brain, neck, thorax and upper abdomen revealed extensive organ involvement, including brain, thyroid, lungs, pancreas, kidney, spleen and muscle; and biopsies of two of the subcutaneous nodules were histologically pathognomonic of *T. solium* cysticercosis. The patient received praziquantel (Biltricide; Bayer-Miles) 1,75 g twice daily and prednisone 20 mg per day for 4 weeks. He was reassessed radiologically after 4 weeks. There was marked regression in the size and numbers of nodules in the lung fields. Clinically, most of the subcutaneous nodules had disappeared. Authors highlighted that the radiological appearance of cysticercosis in the lung cannot be differentiated from other parasitic infections, e.g. echinococcosis, pentastomiasis, paragonimiasis and histoplasmosis, or conditions such as tuberculosis, alveolar carcinoma and metastases due to the varying reaction of the lung tissue and difference in size of the larvae [114]. Pulmonary sparganosis should be exluded from the list of differential diagnosis as well [120]. Although rare, pulmonary cysticercosis does occur and in developing countries should not be overlooked

From our knowledge, this is the only patient with renal involvement reported to the medical

From one case reported in Korea, authors concluded that the presence of cysticerci are difficult to discern from pulmonary infiltrates, because other parasitic infestations or tuberculosis, as well as metastatic lesions, produce similar chest X-ray findings and similar

and all of them agree that this is an uncommon condition [115-119].

in the differential diagnosis of multiple lung opacities.

literature ever.

In 2011, a radiologist from Punjab, India [119] reported a well documented 28-year-old man presented with a history of fever, dry cough, headache and decreased responsiveness over 7 days. One year previously, he had suffered seizures and was hospitalised in another centre with an initial diagnosis of NCC and received treatment for it. MRI at that time depicted numerous ring enhancing lesions with eccentric scolex ('cyst with dot' appearance) in the cerebral hemispheres, subarachnoid space and orbits. The patient had a history of pork intake and intravenous drug misuse. Physical examination at the time of admission revealed small, movable, painless subcutaneous nodules that were palpable over the arms, axillae and trunk. No abnormalities were noted on auscultation of the lungs. Blood and urine cultures were negative. Sputum analysis was normal. A chest x-ray revealed small nodules in both lungs. This is the first report about pulmonary cysticercosis in a patient presenting DCC and subarachnoid NCC.

Next patient was reported by Strawter et.al., [113] a few weeks ago. They studied a 31-yearold incarcerated Hispanic male presented with a nonproductive cough for several months and one episode of blood tinged sputum. He admitted to weight loss and night sweats, headaches, and visual disturbances. He was an immigrant from Honduras and lived in Arizona for the past 15 years. He had chronic hepatitis C infection and was receiving treatment with pegylated interferon-alfa-2a (IFN-α) and ribavirin. His symptoms began one month after initiating antiviral therapy.They confirmed pulmonary nodules with cutaneous manifestations and concluded that: Disseminated cysticercosis with pulmonary involvement should be suspected in any patient presenting with multiple pulmonary nodules who is an immigrant from an endemic region or an individual who has resided in one of the countries where cysticercosis is present or when NCC is well documented. Lung are the second thoracic organ affected by *T solium* cysticercosis preceded by the heart (See Figure 14)

**Figure 14.** Thoracic organs involved in disseminated cysticercosis

### **6.3. Cardio-pulmonary cysticercosis**

The heart and the lungs are the thoracic organs more frequently affected by parasitic infections. The involvement of the heart may be part of a more generalized illness, as is the case with human African trypanosomiasis [102] and other parasitic diseases as before-cited. The first case reported under this condition presented a massive cardiopulmonary cysticercosis and an associated leukemia. [121] being this one the only patient presenting this type of co-morbidity. Bastos et al., [122] from Brazil reported another patient and highlighted the importance of imagenology (CT scan) of the chest for confirmation of diagnosis based on their findings: multiple pulmonary, cardiac and chest wall nodules concluding that cysticercosis should be considered in the differential diagnosis of multiple pulmonary nodules, mainly in those patients with similar lesions in the cardiac muscle and/or in the chest wall which made the overall diagnosis easy to perform.

What is a Low Frequency of the Disseminated

Cysticercosis Suggests that Neurocysticercosis is Going to Disappear? 183

with cardiac, pulmonary, pancreatic, spinal, extradural, and ocular involvement reported in the international medical literature up today and clearly demonstrate the extent to which cysticercosis can be disseminated and almost all probable combination of involved organs. Although this report does not describe the state of the evolutionary of cysticercus, it is logical to assume that a parasitic infection of this magnitud is due to uninterrupted ingestion of *T. solium* eggs which is only possible under deplorable sanitary conditions. Such a quantity of larvae of *T. solium* solium when driving through the bloodstream in a young patient allows its dissemination almost uniformly throughout the body including the CNS.

In the next chapter we will refer more details to the ocular cysticercosis according to their different locations within and outside of the eyeball but without exceeding the limits of the orbital cavity. In this chapter we will review only the clinical features of the ocular cysticercosis associated with damage to other organs as part of the disseminate cysticercosis,

Almost all patients presenting cysticercotic orbital involvement and associated DCC were from India as we show below. Cheung et al., [124] in 1987 reported a patient with disseminated cysticercosis involving orbit, tongue, parotid glands, epicardial fat tissue, muscles, and subcutaneous tissues confirmed by MRI. This was the first communication on combination of cysticercosis of the orbit, parotid gland and epicardial fat. Cysticercosis of the eyelid was studied by Gupta et al., [125]. One of the youngest patient (5-years-old) ever studied was reported by Asrani and Morani [108] their patient apart from cardiocysticercosis (before-mentioned) also presented multiple orbital cysts with decreased visual acuity and neuropsychiatric manifestations. Despite this ocular involvement this patient received anthelmintic agents and not further complications were informed. Combination of epilepsy, DCC, proptosis of the eyes, fever and arthralgia can be seen [8]. Disseminated cysticercosis can cause intravitreal lesions with exudative retinal detachment and bilateral extra ocular muscular damage at the same time [126] or afect only the brain, orbits, subcutaneous tissue [127, 128] the eyes, tongue and spinal cord [127] or the tongue, eyes, face and scalp muscles [130], and an extensive involvement of both orbits [131]; face, scalp muscles, subconjuctival cysts in the eyes and cysticercotic encephalitis [132], cardiopulmonary cysticercosis plus proptosis of the eyes, extra ocular muscles of both orbits, extradural spinal, and cerebellar involvement [123] and other combinations such as: subarachnoid NCC, Brown syndrome, different extra ocular muscles involvement, mimicking idiopathic orbital inflammation, retina involvement , and canine tooth syndrome

As we have seen, the disseminate cysticercosis (muscular and subcutaneous tissue) is always associated with the NCC and in addition, it can be associate to heart, lung, eyes,

based on the results published in the international medical literature.

have been reported to the medical literature [119, 133-138].

**6.5. Other organs affected** 

This aspect will be discussed below.

**6.4. Orbital cysticercosis** 

Other important contribution is made by Jain et al., [123]. They reported a 19-year-old boy from Mumbai (India) who presented with a history of headache and vomiting for six months, seizures for three months, and decreased vision and bilateral proptosis (right more than left) for one month. The patient was apparently asymptomatic six months back. The patient was a vegetarian. Examination revealed subcutaneous nodules over the right eyelid, with mild proptosis of both eyes. Using the Snellen chart, his vision was found to be 6/30 in the right eye and 7/30 in the left eye. Neurological examination did not reveal any abnormality. All the laboratory investigations were normal. The patient was referred for MRI of the brain, which revealed multiple 3–8 mm-sized cystic lesions with T2-hypointense foci within both cerebral hemispheres, cerebellum, extraocular muscles of both orbits and soft tissues of the neck. Similar lesions were distributed in the muscles and adjacent subcutaneous tissues of the back, abdominal wall, thighs, calves, legs, forearms and arms as well as the extradural spinal space. Multiple cystic lesions were seen in both lungs and in the cardiac muscles. Hyperintense nodules were also seen in the pancreas. For further evaluation, a high-resolution CT (HRCT) of the lungs was performed, which revealed bilateral multiple, randomly distributed, 3-8 mm nodules. A B-scan USG was performed, which revealed a large intravitreal cyst within the right eye, with a tiny hyperechoic scolex. The left eye was normal. On superficial probe evaluation of the heart and pericardium through a parasternal window, using a high-frequency 7 to 12 MHz linear transducer (HDI 3000; Philips Medical Systems, Andover, MA, USA), there were multiple oval to circular, 0.5–1.5-cm sized, predominantly anechoic lesions in the heart muscle. Later, a dedicated 2D echocardiography was performed, which revealed multiple disseminated cysticerci involving the muscle of the heart. On the basis of these imaging findings, a diagnosis of disseminated cysticercosis was made. The patient also had right bundle branch block on electrocardiogram; however, other functional parameters such as ejection fraction, valve function, systolic function and chamber size were normal on 2D echocardiography. A final diagnosis of cysticercosis was made and this was confirmed on muscle biopsy. Because drug-induced inflammation due to praziquantel may cause irreversible damage in cases with ocular lesions, this medication was not given, and the patient was treated symptomatically with antiepileptic drugs, steroids, and diuretics. To the best of our knowledge, this is the best documented patient presented with disseminated cysticercosis with cardiac, pulmonary, pancreatic, spinal, extradural, and ocular involvement reported in the international medical literature up today and clearly demonstrate the extent to which cysticercosis can be disseminated and almost all probable combination of involved organs.

Although this report does not describe the state of the evolutionary of cysticercus, it is logical to assume that a parasitic infection of this magnitud is due to uninterrupted ingestion of *T. solium* eggs which is only possible under deplorable sanitary conditions. Such a quantity of larvae of *T. solium* solium when driving through the bloodstream in a young patient allows its dissemination almost uniformly throughout the body including the CNS. This aspect will be discussed below.

## **6.4. Orbital cysticercosis**

182 Novel Aspects on Cysticercosis and Neurocysticercosis

**6.3. Cardio-pulmonary cysticercosis** 

The heart and the lungs are the thoracic organs more frequently affected by parasitic infections. The involvement of the heart may be part of a more generalized illness, as is the case with human African trypanosomiasis [102] and other parasitic diseases as before-cited. The first case reported under this condition presented a massive cardiopulmonary cysticercosis and an associated leukemia. [121] being this one the only patient presenting this type of co-morbidity. Bastos et al., [122] from Brazil reported another patient and highlighted the importance of imagenology (CT scan) of the chest for confirmation of diagnosis based on their findings: multiple pulmonary, cardiac and chest wall nodules concluding that cysticercosis should be considered in the differential diagnosis of multiple pulmonary nodules, mainly in those patients with similar lesions in the cardiac muscle

Other important contribution is made by Jain et al., [123]. They reported a 19-year-old boy from Mumbai (India) who presented with a history of headache and vomiting for six months, seizures for three months, and decreased vision and bilateral proptosis (right more than left) for one month. The patient was apparently asymptomatic six months back. The patient was a vegetarian. Examination revealed subcutaneous nodules over the right eyelid, with mild proptosis of both eyes. Using the Snellen chart, his vision was found to be 6/30 in the right eye and 7/30 in the left eye. Neurological examination did not reveal any abnormality. All the laboratory investigations were normal. The patient was referred for MRI of the brain, which revealed multiple 3–8 mm-sized cystic lesions with T2-hypointense foci within both cerebral hemispheres, cerebellum, extraocular muscles of both orbits and soft tissues of the neck. Similar lesions were distributed in the muscles and adjacent subcutaneous tissues of the back, abdominal wall, thighs, calves, legs, forearms and arms as well as the extradural spinal space. Multiple cystic lesions were seen in both lungs and in the cardiac muscles. Hyperintense nodules were also seen in the pancreas. For further evaluation, a high-resolution CT (HRCT) of the lungs was performed, which revealed bilateral multiple, randomly distributed, 3-8 mm nodules. A B-scan USG was performed, which revealed a large intravitreal cyst within the right eye, with a tiny hyperechoic scolex. The left eye was normal. On superficial probe evaluation of the heart and pericardium through a parasternal window, using a high-frequency 7 to 12 MHz linear transducer (HDI 3000; Philips Medical Systems, Andover, MA, USA), there were multiple oval to circular, 0.5–1.5-cm sized, predominantly anechoic lesions in the heart muscle. Later, a dedicated 2D echocardiography was performed, which revealed multiple disseminated cysticerci involving the muscle of the heart. On the basis of these imaging findings, a diagnosis of disseminated cysticercosis was made. The patient also had right bundle branch block on electrocardiogram; however, other functional parameters such as ejection fraction, valve function, systolic function and chamber size were normal on 2D echocardiography. A final diagnosis of cysticercosis was made and this was confirmed on muscle biopsy. Because drug-induced inflammation due to praziquantel may cause irreversible damage in cases with ocular lesions, this medication was not given, and the patient was treated symptomatically with antiepileptic drugs, steroids, and diuretics. To the best of our knowledge, this is the best documented patient presented with disseminated cysticercosis

and/or in the chest wall which made the overall diagnosis easy to perform.

In the next chapter we will refer more details to the ocular cysticercosis according to their different locations within and outside of the eyeball but without exceeding the limits of the orbital cavity. In this chapter we will review only the clinical features of the ocular cysticercosis associated with damage to other organs as part of the disseminate cysticercosis, based on the results published in the international medical literature.

Almost all patients presenting cysticercotic orbital involvement and associated DCC were from India as we show below. Cheung et al., [124] in 1987 reported a patient with disseminated cysticercosis involving orbit, tongue, parotid glands, epicardial fat tissue, muscles, and subcutaneous tissues confirmed by MRI. This was the first communication on combination of cysticercosis of the orbit, parotid gland and epicardial fat. Cysticercosis of the eyelid was studied by Gupta et al., [125]. One of the youngest patient (5-years-old) ever studied was reported by Asrani and Morani [108] their patient apart from cardiocysticercosis (before-mentioned) also presented multiple orbital cysts with decreased visual acuity and neuropsychiatric manifestations. Despite this ocular involvement this patient received anthelmintic agents and not further complications were informed. Combination of epilepsy, DCC, proptosis of the eyes, fever and arthralgia can be seen [8]. Disseminated cysticercosis can cause intravitreal lesions with exudative retinal detachment and bilateral extra ocular muscular damage at the same time [126] or afect only the brain, orbits, subcutaneous tissue [127, 128] the eyes, tongue and spinal cord [127] or the tongue, eyes, face and scalp muscles [130], and an extensive involvement of both orbits [131]; face, scalp muscles, subconjuctival cysts in the eyes and cysticercotic encephalitis [132], cardiopulmonary cysticercosis plus proptosis of the eyes, extra ocular muscles of both orbits, extradural spinal, and cerebellar involvement [123] and other combinations such as: subarachnoid NCC, Brown syndrome, different extra ocular muscles involvement, mimicking idiopathic orbital inflammation, retina involvement , and canine tooth syndrome have been reported to the medical literature [119, 133-138].

#### **6.5. Other organs affected**

As we have seen, the disseminate cysticercosis (muscular and subcutaneous tissue) is always associated with the NCC and in addition, it can be associate to heart, lung, eyes, spleen, pancreas, parotid, liver, kidneys and pharynx involvement. Here we will discuss about some cases presenting this modalities of DCC.

What is a Low Frequency of the Disseminated

Cysticercosis Suggests that Neurocysticercosis is Going to Disappear? 185

155] believe that awareness about this technique may enable to his fellow colleagues to explore its valuable potential in disseminated cysticercosis. Most of patients presenting DCC are from India (New Delhi) as can be seen in the figure 16 but even there, the number of well documented patient is still no enough to design an accurate clinical trial looking for a medication of choice for this condition. Based on our experiency, to combine Praziquantel and Albendazole with steroids provide good results if there is not involvement of the heart,

the eyes or massive NCC.

**Figure 16.** Distribution of disseminated cysticercosis worldwide.

**7. Mortality rate in disseminated cysticercosis** 

always below 0.05 % in the past 15 years.

As noted in the previous map there are two large areas in Latin America and Africa where the disease is may be present but the ignored epidemiological conditions of those not having established contact with the civilized world make impossible to predict the possible number

The mortality rate for the cysticercosis is widespread and extremely low, in our series was

of patients although by photos published in Internet we could not identified anyone.

Other presentations of the DCC include epilepsy with extensive muscular involvement, predominantly [139-144], associated liver damage [145], epilepsy with damage of the soft tissues of the scalp plus supra and infratentorial lesions; spinal cord [129] epilepsy and DCC with huge muscle involvement [146-148], vocal cord [149], thyroid gland [150], the tongue [129,151], spleen and pancreas [46]; or even without neurological symptoms [152] or immunocompromised patient [153].

Cysticercosis of the pharynx the kidneys have been reported in South Africa only [58, 61], as we have mentioned previously that serves to highlight that these two locations must also keep in mind when planning to perform clinical investigations for this type of patient. Most of isolated damaged organs by CC without dissemination of cysticerci throught the subcutaneous tissues are not associated with NCC and when it happen then extraparenchymal location can be seen. Because only anecdotic reports to the medical literature are available at the present moment (See figure 15) we can not arrive to solid conclusions and only advices are suggested.

**Figure 15.** Graphic representation of number of patients with cysticercosis in different organs as part of disseminated cysticercosis with involvement of the central nervous system at different levels.

India has the highest prevalence of disseminate cysticercosis worldwide (Figure 17). Given the fact that cysticercosis is an endemic disease in India and that WB-MRI is now available on most modern MRI scanners, especially in academic institutions, Goenka and Kumar [154, 155] believe that awareness about this technique may enable to his fellow colleagues to explore its valuable potential in disseminated cysticercosis. Most of patients presenting DCC are from India (New Delhi) as can be seen in the figure 16 but even there, the number of well documented patient is still no enough to design an accurate clinical trial looking for a medication of choice for this condition. Based on our experiency, to combine Praziquantel and Albendazole with steroids provide good results if there is not involvement of the heart, the eyes or massive NCC.

184 Novel Aspects on Cysticercosis and Neurocysticercosis

immunocompromised patient [153].

conclusions and only advices are suggested.

about some cases presenting this modalities of DCC.

spleen, pancreas, parotid, liver, kidneys and pharynx involvement. Here we will discuss

Other presentations of the DCC include epilepsy with extensive muscular involvement, predominantly [139-144], associated liver damage [145], epilepsy with damage of the soft tissues of the scalp plus supra and infratentorial lesions; spinal cord [129] epilepsy and DCC with huge muscle involvement [146-148], vocal cord [149], thyroid gland [150], the tongue [129,151], spleen and pancreas [46]; or even without neurological symptoms [152] or

Cysticercosis of the pharynx the kidneys have been reported in South Africa only [58, 61], as we have mentioned previously that serves to highlight that these two locations must also keep in mind when planning to perform clinical investigations for this type of patient. Most of isolated damaged organs by CC without dissemination of cysticerci throught the subcutaneous tissues are not associated with NCC and when it happen then extraparenchymal location can be seen. Because only anecdotic reports to the medical literature are available at the present moment (See figure 15) we can not arrive to solid

**Figure 15.** Graphic representation of number of patients with cysticercosis in different organs as part of

India has the highest prevalence of disseminate cysticercosis worldwide (Figure 17). Given the fact that cysticercosis is an endemic disease in India and that WB-MRI is now available on most modern MRI scanners, especially in academic institutions, Goenka and Kumar [154,

disseminated cysticercosis with involvement of the central nervous system at different levels.

**Figure 16.** Distribution of disseminated cysticercosis worldwide.

As noted in the previous map there are two large areas in Latin America and Africa where the disease is may be present but the ignored epidemiological conditions of those not having established contact with the civilized world make impossible to predict the possible number of patients although by photos published in Internet we could not identified anyone.

## **7. Mortality rate in disseminated cysticercosis**

The mortality rate for the cysticercosis is widespread and extremely low, in our series was always below 0.05 % in the past 15 years.

Based on our experience and in the cases reported in the international medical literature the causes of death are almost always related to the NCC and anecdotal cases secondary to pancreatic or cardiopulmonary complications.

What is a Low Frequency of the Disseminated

Cysticercosis Suggests that Neurocysticercosis is Going to Disappear? 187

rotation. Depending on the relative value of these forces can be produce different flow states. When the gradient of speed is low, the inertia force is greater than the friction, the cysticerci move but do not rotate, or they make it but with very little power, the final result is a movement in which the particles follow paths defined, and all of the particles that pass by a point in the field of flow follow the same trajectory. This type of flow was identified by O. Reynolds and is called "laminar", meaning that the particles are moving in the form of layers or sheets. The increase of velocity gradient increases the friction between neighboring cysticercus to the fluid, and these become a significant energy of rotation, the viscosity loses its effect, and due to the rotation of the cysticercus change trajectory. Going from one path to another, the cysticerci collide and change course in erratic. This type of flow is called "turbulent". Turbulent flow can also be due to abnormalities in the wall of blood vessels.The

The cysticercus in the fluid does not move along with defined trajectories.

**Figure 18.** Massive neurocysticercosis showing very few active cerebellar lesions.

The cysticerci in the fluid possess appreciable energy of rotation, and they move

When entering the fluid articles to layers of different speed, its linear time increases or decreases, and the particles of the neighboring does so in a manner that is contrary. When the forces of inertia of the fluid in movement are very low, the viscosity is the dominant force and the flow is laminar. When dominated by the forces of inertia the flow is turbulent.

turbulent flow is characterized by:

The action of the viscosity is negligible.

erratically colliding with each other.

The most frequent cause of death related to the NCC in patients with CCD is the intraventricular NCC with intracranial hypertension by obstructive hydrocephalus, the subarachnoid NCC with cerebrovascular complications, the NCC of the insula with associated neurogénic heart or sudden unexpected death and the massive NCC. When there are more than a thousand cysts in the brain the mortality rate is extremely high (see figure 17).

**Figure 17.** CT scan of the brain showing massive neurocystciercosis.

## **8. Why some cisticercus move to one path while other move to another one?**

The cerebellar lesions are an expression of this type of distribution as they generally cisticercotic lesions in the posterior fossa are only seen when there are massive infestations or when the cysticerci traveling through a non-laminar flow secondary to vascular lesions not described in the medical literature (See figure 18).

We think this situation happen because of the haemodynamic characteristic of the blood flow can change in different blood vessel. Let us to explain what probably happen when cysticerci coincide in the same place at the same time. When between two cysticercus in movement speed gradient exists, or is that a moves faster than the other, they develop friccian forces acting tangentially to the same. The friction forces are trying to introduce rotation between the cysticerci in motion, but simultaneously the viscosity seeks to prevent rotation. Depending on the relative value of these forces can be produce different flow states. When the gradient of speed is low, the inertia force is greater than the friction, the cysticerci move but do not rotate, or they make it but with very little power, the final result is a movement in which the particles follow paths defined, and all of the particles that pass by a point in the field of flow follow the same trajectory. This type of flow was identified by O. Reynolds and is called "laminar", meaning that the particles are moving in the form of layers or sheets. The increase of velocity gradient increases the friction between neighboring cysticercus to the fluid, and these become a significant energy of rotation, the viscosity loses its effect, and due to the rotation of the cysticercus change trajectory. Going from one path to another, the cysticerci collide and change course in erratic. This type of flow is called "turbulent". Turbulent flow can also be due to abnormalities in the wall of blood vessels.The turbulent flow is characterized by:


186 Novel Aspects on Cysticercosis and Neurocysticercosis

pancreatic or cardiopulmonary complications.

**Figure 17.** CT scan of the brain showing massive neurocystciercosis.

not described in the medical literature (See figure 18).

**one?** 

**8. Why some cisticercus move to one path while other move to another** 

The cerebellar lesions are an expression of this type of distribution as they generally cisticercotic lesions in the posterior fossa are only seen when there are massive infestations or when the cysticerci traveling through a non-laminar flow secondary to vascular lesions

We think this situation happen because of the haemodynamic characteristic of the blood flow can change in different blood vessel. Let us to explain what probably happen when cysticerci coincide in the same place at the same time. When between two cysticercus in movement speed gradient exists, or is that a moves faster than the other, they develop friccian forces acting tangentially to the same. The friction forces are trying to introduce rotation between the cysticerci in motion, but simultaneously the viscosity seeks to prevent

Based on our experience and in the cases reported in the international medical literature the causes of death are almost always related to the NCC and anecdotal cases secondary to

The most frequent cause of death related to the NCC in patients with CCD is the intraventricular NCC with intracranial hypertension by obstructive hydrocephalus, the subarachnoid NCC with cerebrovascular complications, the NCC of the insula with associated neurogénic heart or sudden unexpected death and the massive NCC. When there are more

than a thousand cysts in the brain the mortality rate is extremely high (see figure 17).

 The cysticerci in the fluid possess appreciable energy of rotation, and they move erratically colliding with each other.

When entering the fluid articles to layers of different speed, its linear time increases or decreases, and the particles of the neighboring does so in a manner that is contrary. When the forces of inertia of the fluid in movement are very low, the viscosity is the dominant force and the flow is laminar. When dominated by the forces of inertia the flow is turbulent.

**Figure 18.** Massive neurocysticercosis showing very few active cerebellar lesions.

## **9. Conclusion**

The disseminated cysticercosis is a rare disease and the largest number of cases have been confirmed and published in India. Disseminated cysticercosis is accompanied by neurocysticercosis especially in its intraparenchymal localization but other locations are possible. There have been no reported cases of neurocysticercosis intraventricular associated to disseminated cysticercosis.

What is a Low Frequency of the Disseminated

Cysticercosis Suggests that Neurocysticercosis is Going to Disappear? 189

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**10. References** 

2011.

4484.

Apart from the subcutaneous tissue, muscles and the brain, the organ most frequently affected is the heart and the disorder of cardiac conduction is the main problem. Orbital cysticercosis (intra or extra ocular) is not associated with the disseminated cysticercosis in South Africa while in India makes it with more frequency.

At the present time, DCC seems to be not associated to HIV infections.

The low prevalence of the disseminated cysticercosis does not mean that cysticercosis is about to disappear in the near future.

It is possible that the defining characteristics of blood flow may be determined by the erratic locations of cysticerci, but well designed hemodynamic studies are needed to demonstrate this hypothesis.

## **Author details**

Humberto Foyaca Sibat and Lourdes de Fátima Ibañez Valdés *Walter Sisulu University, Faculty of Health Sciences, Nelson Mandela Academic Hospital, Department of Neurology, Mthatha, South Africa* 

## **Acknowledgement**

We like to thanks to all veterinarian researches working on this field.

We also want to thank to all radiologists and radiographers from Nelson Mandela Academic Hospital and Inkhosi Albert Luthuli Central Hospital in South Africa for their contribution to this study.

Special thanks are due to the Cuban Ministry of Health and Institute of Tropical Medicine "Pedro Kouri", and authorities of Nelson Mandela Academic Hospital, School of Medicine, Faculty of Health Sciences and Directorate: Research Development from Walter Sisulu University for their kind support. Finally, we would like to declare our eternal and deepest gratitude to Lorna María Foyaca García, Fátima Susana Foyaca Ibañez and Thabo Humberto Foyaca Ibañez for their delight support.

Hereby, we acknowledge financial support from the South African Medical Research Council. The founder had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript

## **10. References**

188 Novel Aspects on Cysticercosis and Neurocysticercosis

South Africa while in India makes it with more frequency.

Humberto Foyaca Sibat and Lourdes de Fátima Ibañez Valdés

We like to thanks to all veterinarian researches working on this field.

*Department of Neurology, Mthatha, South Africa* 

Foyaca Ibañez for their delight support.

publish, or preparation of the manuscript

At the present time, DCC seems to be not associated to HIV infections.

The disseminated cysticercosis is a rare disease and the largest number of cases have been confirmed and published in India. Disseminated cysticercosis is accompanied by neurocysticercosis especially in its intraparenchymal localization but other locations are possible. There have been no reported cases of neurocysticercosis intraventricular associated

Apart from the subcutaneous tissue, muscles and the brain, the organ most frequently affected is the heart and the disorder of cardiac conduction is the main problem. Orbital cysticercosis (intra or extra ocular) is not associated with the disseminated cysticercosis in

The low prevalence of the disseminated cysticercosis does not mean that cysticercosis is

It is possible that the defining characteristics of blood flow may be determined by the erratic locations of cysticerci, but well designed hemodynamic studies are needed to demonstrate

We also want to thank to all radiologists and radiographers from Nelson Mandela Academic Hospital and Inkhosi Albert Luthuli Central Hospital in South Africa for their contribution

Special thanks are due to the Cuban Ministry of Health and Institute of Tropical Medicine "Pedro Kouri", and authorities of Nelson Mandela Academic Hospital, School of Medicine, Faculty of Health Sciences and Directorate: Research Development from Walter Sisulu University for their kind support. Finally, we would like to declare our eternal and deepest gratitude to Lorna María Foyaca García, Fátima Susana Foyaca Ibañez and Thabo Humberto

Hereby, we acknowledge financial support from the South African Medical Research Council. The founder had no role in study design, data collection and analysis, decision to

*Walter Sisulu University, Faculty of Health Sciences, Nelson Mandela Academic Hospital,* 

**9. Conclusion** 

this hypothesis.

**Author details** 

**Acknowledgement** 

to this study.

to disseminated cysticercosis.

about to disappear in the near future.


http://www.ispub.com/journal/the\_internet\_journal\_of\_neurology/volume\_1\_number\_ 2\_39/article/clinical\_trial\_of\_praziquantel\_and\_prednisone\_in\_rural\_patients\_with\_neu rocysticercosis\_presenting\_with\_recurrent\_epileptic\_attacks.html


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**Chapter 8** 

© 2013 Foyaca Sibat and de Fátima Ibañez Valdés, licensee InTech. This is an open access chapter

(http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction

© 2013 Foyaca Sibat and de Fátima Ibañez Valdés, licensee InTech. This is a paper distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

distributed under the terms of the Creative Commons Attribution License

Each day the cysticercosis is more and better known in the developed world or in developing countries, the immune diagnostic techniques are more reliable and neuroimaging studies are more accurate. The greatest success in the diagnosis of the disease

in any medium, provided the original work is properly cited.

**Uncommon Clinical Manifestations of** 

Humberto Foyaca Sibat and Lourdes de Fátima Ibañez Valdés

Cysticercosis, a parasitic infection caused by the larval form of the pork tapeworm, *Taenia solium*, is increasingly recognized as a cause of epilepsy, headache, and neurological signs when it is located in the brain, optic nerve or spinal cord, known as neurocysticercosis (NCC). A high prevalence of cysticercosis/taeniosis has been reported from the developing countries because of the coexistence of poor sanitary conditions and domestic pig raising without veterinary control or surveillance systems. It occurs mainly in Eastern and Southern Africa, China, India, Mexico, Central America, Chile, Ecuador, Colombia, Venezuela, Peru, Brazil, Papua New Guinea, and non-Islamic South East Asia where human faces reach pigs

An infection which leads to extra-intestinal disease (including NCC) usually occurs as a result of eating food or drinking water contaminated by human feces containing *T. solium* eggs. It is a preventable and potentially eradicable neurological disease which primarily affects people living in the developing world. The disease is endemic in Central and South America, Asia and Africa. There is a variable time interval between point of infection and

Seizures are widely reported to be the most common symptom, occurring in 70-90% of patients. Most patients respond to praziquantel if cystic lesions are located in the parenchyma tissue and albendazole when parasites are located in the ventricular system and subarachnoid space [1-16]. Seizure disorder is the most frequent clinical manifestation of the NCC observed in 50% to 80% of cases, in children and adults presenting

Additional information is available at the end of the chapter

**Cysticercosis** 

http://dx.doi.org/10.5772/53078

and pork is eaten raw or undercooked.

intraparenchymal cystic lesions. [13-44]

the onset of symptoms: ranging from 1-30 years.

**1. Introduction** 

[155] Kumar A, Goenka AH, Choudhary A, Sahu JK, Gulati S. Disseminated cysticercosis in a child: whole-body MR diagnosis with the use of parallel imaging. Pediatr Radiol. 2010;40(2):223-227.

## **Uncommon Clinical Manifestations of Cysticercosis**

Humberto Foyaca Sibat and Lourdes de Fátima Ibañez Valdés

Additional information is available at the end of the chapter

http://dx.doi.org/10.5772/53078

## **1. Introduction**

198 Novel Aspects on Cysticercosis and Neurocysticercosis

2010;40(2):223-227.

Radiol Imaging. 2011; 21(2): 157–158.

[154] Goenka AH, Kumar A. Whole body MR and disseminated cysticercosis. Indian J

[155] Kumar A, Goenka AH, Choudhary A, Sahu JK, Gulati S. Disseminated cysticercosis in a child: whole-body MR diagnosis with the use of parallel imaging. Pediatr Radiol.

> Cysticercosis, a parasitic infection caused by the larval form of the pork tapeworm, *Taenia solium*, is increasingly recognized as a cause of epilepsy, headache, and neurological signs when it is located in the brain, optic nerve or spinal cord, known as neurocysticercosis (NCC). A high prevalence of cysticercosis/taeniosis has been reported from the developing countries because of the coexistence of poor sanitary conditions and domestic pig raising without veterinary control or surveillance systems. It occurs mainly in Eastern and Southern Africa, China, India, Mexico, Central America, Chile, Ecuador, Colombia, Venezuela, Peru, Brazil, Papua New Guinea, and non-Islamic South East Asia where human faces reach pigs and pork is eaten raw or undercooked.

> An infection which leads to extra-intestinal disease (including NCC) usually occurs as a result of eating food or drinking water contaminated by human feces containing *T. solium* eggs. It is a preventable and potentially eradicable neurological disease which primarily affects people living in the developing world. The disease is endemic in Central and South America, Asia and Africa. There is a variable time interval between point of infection and the onset of symptoms: ranging from 1-30 years.

> Seizures are widely reported to be the most common symptom, occurring in 70-90% of patients. Most patients respond to praziquantel if cystic lesions are located in the parenchyma tissue and albendazole when parasites are located in the ventricular system and subarachnoid space [1-16]. Seizure disorder is the most frequent clinical manifestation of the NCC observed in 50% to 80% of cases, in children and adults presenting intraparenchymal cystic lesions. [13-44]

> Each day the cysticercosis is more and better known in the developed world or in developing countries, the immune diagnostic techniques are more reliable and neuroimaging studies are more accurate. The greatest success in the diagnosis of the disease

© 2013 Foyaca Sibat and de Fátima Ibañez Valdés, licensee InTech. This is an open access chapter distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. © 2013 Foyaca Sibat and de Fátima Ibañez Valdés, licensee InTech. This is a paper distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

control passes at the achievements attained by the agricultural sector, without which it is not possible try to sustain successful results. The knowledge that we have on the porcine cysticercosis today are much more certain and deep. In the epidemiological aspects, the African continent goes up to the head because it is the only basic information that has been made safe for almost all their countries, as can be seen in the chapter: "*Agricultural Impact of Porcine Cyisticercosis in Africa: A Review"* in this book written by Helena A Ngowi et al. This study included information from 54 African countries that were fully recognized by the United Nations as of 2012. The total population of pigs estimated in the study area in 2010 amounted 29,606,438. This was approximately 3.1% of the world pig population estimate (N = 965,855,414) of 2010. Out of the Africa regional pig population, approximately 0.2% was reared in the northern countries, while 6.1%, 43.3%, 16,4%, and 34.0% were reared in the southern, western, central, and eastern Africa, respectively. Overall, 19 (35.2%) of the 54 study countries reported prevalence of cysticercosis in pigs. While no data were available in the northern region, the remaining regions provided prevalence data, with more reporting frequency observed in the Eastern followed by Central Africa.

Uncommon Clinical Manifestations of Cysticercosis 201

recurrent epileptic seizures frequently. [3,5] Usually patients with NCC have partial seizures with or without secondary generalization. The epileptogenesis in patients with NCC can be attributed to several factors, such as: inflammation, gliosis and its frequent location in the frontal and temporal lobes. [47] While the basis for seizure production in HIV-positive patients include HIV itself, mass lesions, meningitis, encephalitis, ischemia, metabolic derangement, and drugs [48] In both conditions we recognize patients as epileptic when they present

We consider refractory epilepsy when seizures are so frequent that limit the patient's ability to live fully in line with their wishes and their physical and mental capabilities or when the antiepileptic drug (AED) does not control the crisis or their side effects are limited to a person's normal development. [6] Other authors have the same criteria. [49] Recently, The International League against Epilepsy has defined the patients with epilepsy resistant to drugs when there are no controls over the attacks with a combination of two AED well tolerated, and managed properly elected. BY understanding how the lack of control when the patients continue to have attacks with an interval of not less than one third of which

Although this clinical presentation is not uncommon in our setting it may be very uncommon in other countries. Nevertheless, there is a novel presentation of motor partial simple seizures localized in the lower part of the hemiface in females at the stage IV of HIV/AIDS and CD4 below 100 x106. This type of seizures becomes secondarily generalized a few days or weeks of the beginning and are still uncommon in our region. At the present

Managing seizures in HIV-positive patients present specific challenges, including the identification and treatment of the underlying cause, recognition that seizure production may be multifactorial, selection of the appropriate AED, and consideration of the effects of co-administered highly active antiretroviral therapy (HAART). Unfortunately, there has been little systematic study in these areas. The available information is confined to case reports, retrospective analyses, and small studies. [48] The cause of the seizures will depend on the stage of HIV infection. Since the causes are many and there may be more than one mechanism for seizure production, a meticulous clinical, laboratory, and neuroimaging survey must be undertaken. The older AEDs, phenobarbital, phenytoin, carbamazepine, and valproic acid are still widely used. [48] In our experience Valproic acid is the AED of choice for HIV/AIDS patients with epilepsy due to NCC and we did not find increased viral load in

Finally we like to highlight that apart from NCC/HIV/AIDS sometimes other causes should

Dementia is another uncommon presentation of NCC [50] and its prognosis is better if there

Massive brain infection with viable cysticerci, undetectable inflammatory reaction on CNS imaging, and an unexpectedly high (82%) prevalence of tapeworm infection represents a

recurrent unprovoked epileptic seizures associated to a chronic brain disorder. [14]

presented before starting the antiepiileptic treatment [14]

moment the mortality rate for these patients is 100%.

our series of these patients on HAART.

is not an associated HIH/AIDS.

*Massive neurocysticercosis* 

be investigated for a better control of the seizures. [9]

All these previous arguments together have favored the growing number of diagnosed uncommon presentations of cysticercosis that before passing unnoticed.

For purely didactic reasons we are going to aggregate all the uncommon forms of cysticercosis in accordance with the following classification:


## **2. Uncommon cysticercosis in the head and neck**

### **2.1. Uncommon clinical presentations neurocysticercosis (UNCC)**

#### *Pseudoseizures and epilepsy in patients presenting neurocysticercosis*

In our experience, the South African patients with NCC and fictitious attacks (pseudoseizures) are more often in young women with a history of violations and/or sexual abuse and their attacks are usually characterized by absence of post ictal signs, irregular and asymmetrical stereotyped movements, and often accompanied by shouts or sounds in the middle of the "attack" that usually last for more than three minutes if the patients are not alone. We found that these manifestations contribute to characterize clinically patients with pseudo-crisis. These conclusions were the result of a study conducted some years ago. [4]

#### *Refractory epilepsy in HIV/AIDS patients presenting NCC*

At the present moment, more than 40 million people are living with HIV and more than 50 million people have neurocysticercosis (NCC) worldwide. About 5% of patients with HIV and most of the patients with NCC develop recurrent epileptic seizures. Although this comorbidity has not been reported as an additional public health problem, NCC and the HIV/AIDS infections are very common in the former Transkei (South Africa) and both cause recurrent epileptic seizures frequently. [3,5] Usually patients with NCC have partial seizures with or without secondary generalization. The epileptogenesis in patients with NCC can be attributed to several factors, such as: inflammation, gliosis and its frequent location in the frontal and temporal lobes. [47] While the basis for seizure production in HIV-positive patients include HIV itself, mass lesions, meningitis, encephalitis, ischemia, metabolic derangement, and drugs [48] In both conditions we recognize patients as epileptic when they present recurrent unprovoked epileptic seizures associated to a chronic brain disorder. [14]

We consider refractory epilepsy when seizures are so frequent that limit the patient's ability to live fully in line with their wishes and their physical and mental capabilities or when the antiepileptic drug (AED) does not control the crisis or their side effects are limited to a person's normal development. [6] Other authors have the same criteria. [49] Recently, The International League against Epilepsy has defined the patients with epilepsy resistant to drugs when there are no controls over the attacks with a combination of two AED well tolerated, and managed properly elected. BY understanding how the lack of control when the patients continue to have attacks with an interval of not less than one third of which presented before starting the antiepiileptic treatment [14]

Although this clinical presentation is not uncommon in our setting it may be very uncommon in other countries. Nevertheless, there is a novel presentation of motor partial simple seizures localized in the lower part of the hemiface in females at the stage IV of HIV/AIDS and CD4 below 100 x106. This type of seizures becomes secondarily generalized a few days or weeks of the beginning and are still uncommon in our region. At the present moment the mortality rate for these patients is 100%.

Managing seizures in HIV-positive patients present specific challenges, including the identification and treatment of the underlying cause, recognition that seizure production may be multifactorial, selection of the appropriate AED, and consideration of the effects of co-administered highly active antiretroviral therapy (HAART). Unfortunately, there has been little systematic study in these areas. The available information is confined to case reports, retrospective analyses, and small studies. [48] The cause of the seizures will depend on the stage of HIV infection. Since the causes are many and there may be more than one mechanism for seizure production, a meticulous clinical, laboratory, and neuroimaging survey must be undertaken. The older AEDs, phenobarbital, phenytoin, carbamazepine, and valproic acid are still widely used. [48] In our experience Valproic acid is the AED of choice for HIV/AIDS patients with epilepsy due to NCC and we did not find increased viral load in our series of these patients on HAART.

Finally we like to highlight that apart from NCC/HIV/AIDS sometimes other causes should be investigated for a better control of the seizures. [9]

Dementia is another uncommon presentation of NCC [50] and its prognosis is better if there is not an associated HIH/AIDS.

#### *Massive neurocysticercosis*

200 Novel Aspects on Cysticercosis and Neurocysticercosis

frequency observed in the Eastern followed by Central Africa.

cysticercosis in accordance with the following classification:

**2. Uncommon cysticercosis in the head and neck** 

*Pseudoseizures and epilepsy in patients presenting neurocysticercosis* 

*Refractory epilepsy in HIV/AIDS patients presenting NCC* 

**2.1. Uncommon clinical presentations neurocysticercosis (UNCC)** 

Uncommon cysticercosis in the head and neck

 Uncommon cysticercosis in the trunk Uncommon cysticercosis in the abdomen Uncommon cysticercosis in the limbs

uncommon presentations of cysticercosis that before passing unnoticed.

control passes at the achievements attained by the agricultural sector, without which it is not possible try to sustain successful results. The knowledge that we have on the porcine cysticercosis today are much more certain and deep. In the epidemiological aspects, the African continent goes up to the head because it is the only basic information that has been made safe for almost all their countries, as can be seen in the chapter: "*Agricultural Impact of Porcine Cyisticercosis in Africa: A Review"* in this book written by Helena A Ngowi et al. This study included information from 54 African countries that were fully recognized by the United Nations as of 2012. The total population of pigs estimated in the study area in 2010 amounted 29,606,438. This was approximately 3.1% of the world pig population estimate (N = 965,855,414) of 2010. Out of the Africa regional pig population, approximately 0.2% was reared in the northern countries, while 6.1%, 43.3%, 16,4%, and 34.0% were reared in the southern, western, central, and eastern Africa, respectively. Overall, 19 (35.2%) of the 54 study countries reported prevalence of cysticercosis in pigs. While no data were available in the northern region, the remaining regions provided prevalence data, with more reporting

All these previous arguments together have favored the growing number of diagnosed

For purely didactic reasons we are going to aggregate all the uncommon forms of

In our experience, the South African patients with NCC and fictitious attacks (pseudoseizures) are more often in young women with a history of violations and/or sexual abuse and their attacks are usually characterized by absence of post ictal signs, irregular and asymmetrical stereotyped movements, and often accompanied by shouts or sounds in the middle of the "attack" that usually last for more than three minutes if the patients are not alone. We found that these manifestations contribute to characterize clinically patients with pseudo-crisis. These conclusions were the result of a study conducted some years ago. [4]

At the present moment, more than 40 million people are living with HIV and more than 50 million people have neurocysticercosis (NCC) worldwide. About 5% of patients with HIV and most of the patients with NCC develop recurrent epileptic seizures. Although this comorbidity has not been reported as an additional public health problem, NCC and the HIV/AIDS infections are very common in the former Transkei (South Africa) and both cause

Massive brain infection with viable cysticerci, undetectable inflammatory reaction on CNS imaging, and an unexpectedly high (82%) prevalence of tapeworm infection represents a

particular presentation of neurocysticercosis is different from the previously described syndromes of cysticercotic encephalitis and disseminated systemic cysticercosis according with Garcia et al. [51] In our series we did not find remarkable differences excepting a fatal prognosis when more than one thousand of cystic lesions are present [14,16] but without doubt it is another uncommon clinical presentation of NCC. (Figure 1)

Uncommon Clinical Manifestations of Cysticercosis 203

presence of associated smaller and more typical cysticercal larval forms. Identification of a scolex in a cystic lesion with CSF intensity plays a key role in the diagnosis of NCC and the

Giant cysticercal cysts resolve remarkably rapidly on medical therapy. A therapeutic trial of praziquantel can, in suspected giant cysticercal cysts, obviate the need for exploratory craniotomy. [9, 56] Other authors [60] suggest repeating anthelmintic treatment to provide good response, when it is necessary, and we fully agree on that. However, surgical treatment may be required when it is associated with elevated intracranial pressure, [62]

**Figure 2.** In upper right and lower left white arrow show identification of active viable cyst in vesicular

The posterior cranial fossa is part of the intracranial cavity, located between the foramen magnum and tentorium cerebelli. It contains the brainstem and cerebellum. This is the most inferior of the fossa. It houses the cerebellum, medulla and pons. Anteriorly it extends to the apex of the petrous temporal. Posteriorly it is enclosed by the occipital bone. Laterally portions of the squamous temporal and mastoid part of the temporal bone from its walls.

In the posterior fossa, NCC can involve the fourth ventricle, cerebellopontine angle cistern, cisterna magna and rarely, the cerebellum [64, 65] .Cysticercosis affecting one of the abovementioned anatomical structures are grouped under the name of NCC of the posterior fossa

One of the first articles about clinical manifestations was done by Bickerstaff et al., in 1956 [66] and about clinical and surgical management by Kla in 1964. [67] Other modalities of management including the diagnostic importance of laboratory and serological analysis of blood and cerebro-spinal fluid in cysticercosis of the posterior cranial fossa and description

stage with an eccentric scolex. Giant cyst measuring 106 mm on the left occipital lobe is seen.

presence of internal septations is an atypical feature. [61]

mainly if the pre-operatory diagnosis is not clear enough.

*Neurocysticercosis in the posterior cranial fossa* 

(PFNCC).

**Figure 1.** CT scan of the brain showing massive neurocysticercosis (more that 100 cysts)

#### *Giant intraparenchymal/Giant subarachnoid neurocysticercosis*

Parenchymal cysts usually involve the gray white matter junction, however giant cyst is usually located at the Sylvia fissure or deeper in the cerebral lobe.

Giant NCC is a rare condition defined by various authors as measuring more than five cm in its largest dimension [52] usually associated with intracranial hypertension but giant cysts may not always be associated with elevated intracranial pressure, probably due to slow growing of the cyst.

Almost all reported patients presenting intraparenchymal giant cyst had similar clinical manifestations at the beginning characterized by headache, altered mental status, hemiparesis mainly on the right side [9, 53-62] or uncontrolled epilepsy. [63] Between 1990 and 2006, only five patients have been reported in South Africa. All had associated epilepsy, one patient also presented visual hallucinations, dysnomia in blue and red, dyslexia and palinopsia, and the cyst (106mm) was located in the left occipital lobe. [9] (See figure 2)

All patients were treated with a 31-day course of praziquantel 50 mg/kg daily. Two patients had corticosteroids administered simultaneously. All five patients had anticonvulsant cover. In all cases there was rapid and complete resolution of the cysts. In three patients this occurred within 30 days. The computed tomographic features of giant cysticercal cysts in our series were: thin walled cystic lesions containing clear fluid, localized thickening of the wall with an eccentric scale in three patients, the absence of pericyst edema, and the presence of associated smaller and more typical cysticercal larval forms. Identification of a scolex in a cystic lesion with CSF intensity plays a key role in the diagnosis of NCC and the presence of internal septations is an atypical feature. [61]

Giant cysticercal cysts resolve remarkably rapidly on medical therapy. A therapeutic trial of praziquantel can, in suspected giant cysticercal cysts, obviate the need for exploratory craniotomy. [9, 56] Other authors [60] suggest repeating anthelmintic treatment to provide good response, when it is necessary, and we fully agree on that. However, surgical treatment may be required when it is associated with elevated intracranial pressure, [62] mainly if the pre-operatory diagnosis is not clear enough.

**Figure 2.** In upper right and lower left white arrow show identification of active viable cyst in vesicular stage with an eccentric scolex. Giant cyst measuring 106 mm on the left occipital lobe is seen.

#### *Neurocysticercosis in the posterior cranial fossa*

202 Novel Aspects on Cysticercosis and Neurocysticercosis

particular presentation of neurocysticercosis is different from the previously described syndromes of cysticercotic encephalitis and disseminated systemic cysticercosis according with Garcia et al. [51] In our series we did not find remarkable differences excepting a fatal prognosis when more than one thousand of cystic lesions are present [14,16] but without

doubt it is another uncommon clinical presentation of NCC. (Figure 1)

**Figure 1.** CT scan of the brain showing massive neurocysticercosis (more that 100 cysts)

Parenchymal cysts usually involve the gray white matter junction, however giant cyst is

Giant NCC is a rare condition defined by various authors as measuring more than five cm in its largest dimension [52] usually associated with intracranial hypertension but giant cysts may not always be associated with elevated intracranial pressure, probably due to slow

Almost all reported patients presenting intraparenchymal giant cyst had similar clinical manifestations at the beginning characterized by headache, altered mental status, hemiparesis mainly on the right side [9, 53-62] or uncontrolled epilepsy. [63] Between 1990 and 2006, only five patients have been reported in South Africa. All had associated epilepsy, one patient also presented visual hallucinations, dysnomia in blue and red, dyslexia and palinopsia, and the cyst (106mm) was located in the left occipital lobe. [9] (See figure 2)

All patients were treated with a 31-day course of praziquantel 50 mg/kg daily. Two patients had corticosteroids administered simultaneously. All five patients had anticonvulsant cover. In all cases there was rapid and complete resolution of the cysts. In three patients this occurred within 30 days. The computed tomographic features of giant cysticercal cysts in our series were: thin walled cystic lesions containing clear fluid, localized thickening of the wall with an eccentric scale in three patients, the absence of pericyst edema, and the

*Giant intraparenchymal/Giant subarachnoid neurocysticercosis* 

growing of the cyst.

usually located at the Sylvia fissure or deeper in the cerebral lobe.

The posterior cranial fossa is part of the intracranial cavity, located between the foramen magnum and tentorium cerebelli. It contains the brainstem and cerebellum. This is the most inferior of the fossa. It houses the cerebellum, medulla and pons. Anteriorly it extends to the apex of the petrous temporal. Posteriorly it is enclosed by the occipital bone. Laterally portions of the squamous temporal and mastoid part of the temporal bone from its walls.

In the posterior fossa, NCC can involve the fourth ventricle, cerebellopontine angle cistern, cisterna magna and rarely, the cerebellum [64, 65] .Cysticercosis affecting one of the abovementioned anatomical structures are grouped under the name of NCC of the posterior fossa (PFNCC).

One of the first articles about clinical manifestations was done by Bickerstaff et al., in 1956 [66] and about clinical and surgical management by Kla in 1964. [67] Other modalities of management including the diagnostic importance of laboratory and serological analysis of blood and cerebro-spinal fluid in cysticercosis of the posterior cranial fossa and description of the pseudotumoral form of Cysticercus cellulose to be remembered when one attempts to characterize the type of neoplasms of the posterior fossa as detected by brain scan in the infantile population were reported from 1971 and 1978. [68-71]

Uncommon Clinical Manifestations of Cysticercosis 205

the spinal cord or into the cisterns of the subarachnoid space via three small foramina: the central foramen of Magendie and the two lateral foramina of Luschka. The fluid then flows around the superior sagittal sinus to be reabsorbed via the arachnoid villi into the venous system. CSF within the spinal cord can flow all the way down to the lumbar cistern at the

The aqueduct between the third and fourth ventricles is very small, as are the foramina, which means that they can be easily blocked, causing high pressure in the lateral ventricles. This is a common cause of hydrocephalus (known colloquially as "water on the brain"), which is an extremely serious condition due to both the damage caused by the pressure as well as the nature of whatever caused the block (e.g. a tumour, cyst or inflammatory swelling). The cavities of the cerebral hemispheres are called lateral ventricles, or 1st & 2nd ventricles. These two ventricles open commonly into the 3rd ventricle by a common opening

The presence of T solium in the ventricular system may be seen in a 7-30% of patients with NCC [45] and although it has been reported less frequently (6 %) by other authors [46] everything seems to indicate that its frequency is much greater than had been thought previously. [45] Of all forms of presentation of the NCC, the IVNCC is sometimes associated with a fatal prognosis. The diagnosis of certainty is achieved by methods of neuroimaging, particularly the nuclear magnetic resonance imaging (MRI), which is also indispensable as prior checking to surgery, the choice of treatment and assessment of the response to the

Some reports related to the clinical characteristics and the management of this entity confirm their increasing frequency in countries such as the Unite States of America. [83, 84] In 1906, Ludwig Bruns described a phenomenon characterized by sudden headache associated with acute vestibular syndrome, triggered by sudden movements of the head, or, at times, it could have a disastrous outcome, by leading to coma and even death. [85] A form of presentation of the NCCIv may be syndrome Bruns characterized by episodes of loss of consciousness to head movements and hydrocephalus due to a cyst in the IV ventricle their movement obstructs the exit of CSF through the holes of Luchka and Magendie, involuntary movements or intracranial hypertension. [86] Other clinical manifestations of the NCCIv have been reported according to the location of the cysticerci in the ventricular system [87] or when passing through the same. [88] The NCCIv of the lateral ventricles has not been reported that constitutes a diagnostic or therapeutic problem however the NCCIv of the III and IV ventricles constitute a major challenge to resolve. [80,89] The treatment of forms Iv is geared toward case by case, according to the pathogenic substrate. The first thing to define is the presence or risk of acute hydrocephalus, and, therefore, the option of a ventriculostomy emergency or making a ventriculoperitoneal shunt (DVP), with or without fenestration of the septum, according to the location of the Ct. To take the decision to remove the cysts must be taken into account: the number, location stage of cysticercus, general clinical conditions of the patient, abilities, and experience of the surgical team to perform open or endoscopic neurosurgical approach. [90, 91] Also add to the list other criteria such as: the mass effect, the ventricular obstruction, the dysfunction of the valve and

end of the cord around the cauda equina where lumbar punctures are performed.

called the foramen of Monro.

same. [80-82]

Anecdotal clinical presentations in patients with posterior fossa involvement may include signs of bilateral fourth nerve palsy, facial myokymia, upbeat nystagmus, periodic alternating nystagmus, and rhythmic oculopalatal myoclonus. [72] In patients presenting huge cystic lesions due to PFNCC then intermittent severe headache, epilepsy, and signs of raised intracranial pressure, cerebellar signs and cranial nerves signs can be seen. [63,73] Studies post-mortem have confirmed this correlation between signs of increased intracranial pressure, localizing cerebellar signs and even hearing loss in patients presenting PFNCC in the cerebellopontine cistern and obstructive hydrocephalus. [74,75]

Sang-Wook et al. [76] reported a case presenting headache, dizziness, drowsiness and ataxic gait. The magnetic resonance imaging showed hydrocephalus and an ill-defined, multicystic cerebellar mass with hypersignal on T2-weighted images, hyposignal on T1-weighted images and rim enhancement after gadolinium injection (the differential diagnoses of include abscess, tuberculosis, metastasis and other parasitic diseases). The patient underwent endoscopic third ventriculostomy and the cyst resection was done through a craniotomy. In surgical field, cysts were conglomerated in a dense collagen capsule that were severely adherent to surrounding cerebellar tissue, and transparent cysts contained white, milky fluid. Histological findings confirmed the diagnosis of cysticercosis. These authors concluded that racemose cysticercosis is rare in the cerebellar hemisphere but neurocysticercosis should be taken into consideration as a differential diagnosis of multiple cystic lesions in the cerebellum and we agree on that. As has been mentioned, T solium cysticercosis spread through the bloodstream and it may locate almost anywhere in the CNS, most frequently involves the cerebral hemispheres and sometimes ventricles, basal cisterns, subarachnoid space, and spinal cord. Cerebellar cysticercosis has been rarely reported [77-78]. Anecdotal presentations include multiple cranial nerve involvement (V, VI, VII, VIII) and cerebellar signs resembling anterior inferior cerebellar artery (AICA) syndrome. [79]

Other uncommon clinical locations of NCC (UNCC) are: intraventricular (IV), intra-spinal (IS), subarachnoid (SA) and others that we will explain below.

## *2.1.1. Neurocysticercosis intraventricular (IVNCC)*

The ventricular system is a set of structures containing cerebro-spinal fluid in the brain. It is continuous with the central canal of the spinal cord. The ventricles are filled with cerebrospinal fluid (CSF) which bathes and cushions the brain and spinal cord within their bony confines. Cerebro-spinal fluid is produced by modifying ependymal cells of the choroid plexus found in all components of the ventricular system except for the cerebral aqueduct and the posterior and anterior horns of the lateral ventricles. CSF flows from the lateral ventricles via the foramina of Monro into the third ventricle, and then the fourth ventricle via the cerebral aqueduct in the brainstem. From there it can pass into the central canal of the spinal cord or into the cisterns of the subarachnoid space via three small foramina: the central foramen of Magendie and the two lateral foramina of Luschka. The fluid then flows around the superior sagittal sinus to be reabsorbed via the arachnoid villi into the venous system. CSF within the spinal cord can flow all the way down to the lumbar cistern at the end of the cord around the cauda equina where lumbar punctures are performed.

204 Novel Aspects on Cysticercosis and Neurocysticercosis

syndrome. [79]

infantile population were reported from 1971 and 1978. [68-71]

the cerebellopontine cistern and obstructive hydrocephalus. [74,75]

(IS), subarachnoid (SA) and others that we will explain below.

*2.1.1. Neurocysticercosis intraventricular (IVNCC)* 

of the pseudotumoral form of Cysticercus cellulose to be remembered when one attempts to characterize the type of neoplasms of the posterior fossa as detected by brain scan in the

Anecdotal clinical presentations in patients with posterior fossa involvement may include signs of bilateral fourth nerve palsy, facial myokymia, upbeat nystagmus, periodic alternating nystagmus, and rhythmic oculopalatal myoclonus. [72] In patients presenting huge cystic lesions due to PFNCC then intermittent severe headache, epilepsy, and signs of raised intracranial pressure, cerebellar signs and cranial nerves signs can be seen. [63,73] Studies post-mortem have confirmed this correlation between signs of increased intracranial pressure, localizing cerebellar signs and even hearing loss in patients presenting PFNCC in

Sang-Wook et al. [76] reported a case presenting headache, dizziness, drowsiness and ataxic gait. The magnetic resonance imaging showed hydrocephalus and an ill-defined, multicystic cerebellar mass with hypersignal on T2-weighted images, hyposignal on T1-weighted images and rim enhancement after gadolinium injection (the differential diagnoses of include abscess, tuberculosis, metastasis and other parasitic diseases). The patient underwent endoscopic third ventriculostomy and the cyst resection was done through a craniotomy. In surgical field, cysts were conglomerated in a dense collagen capsule that were severely adherent to surrounding cerebellar tissue, and transparent cysts contained white, milky fluid. Histological findings confirmed the diagnosis of cysticercosis. These authors concluded that racemose cysticercosis is rare in the cerebellar hemisphere but neurocysticercosis should be taken into consideration as a differential diagnosis of multiple cystic lesions in the cerebellum and we agree on that. As has been mentioned, T solium cysticercosis spread through the bloodstream and it may locate almost anywhere in the CNS, most frequently involves the cerebral hemispheres and sometimes ventricles, basal cisterns, subarachnoid space, and spinal cord. Cerebellar cysticercosis has been rarely reported [77-78]. Anecdotal presentations include multiple cranial nerve involvement (V, VI, VII, VIII) and cerebellar signs resembling anterior inferior cerebellar artery (AICA)

Other uncommon clinical locations of NCC (UNCC) are: intraventricular (IV), intra-spinal

The ventricular system is a set of structures containing cerebro-spinal fluid in the brain. It is continuous with the central canal of the spinal cord. The ventricles are filled with cerebrospinal fluid (CSF) which bathes and cushions the brain and spinal cord within their bony confines. Cerebro-spinal fluid is produced by modifying ependymal cells of the choroid plexus found in all components of the ventricular system except for the cerebral aqueduct and the posterior and anterior horns of the lateral ventricles. CSF flows from the lateral ventricles via the foramina of Monro into the third ventricle, and then the fourth ventricle via the cerebral aqueduct in the brainstem. From there it can pass into the central canal of The aqueduct between the third and fourth ventricles is very small, as are the foramina, which means that they can be easily blocked, causing high pressure in the lateral ventricles. This is a common cause of hydrocephalus (known colloquially as "water on the brain"), which is an extremely serious condition due to both the damage caused by the pressure as well as the nature of whatever caused the block (e.g. a tumour, cyst or inflammatory swelling). The cavities of the cerebral hemispheres are called lateral ventricles, or 1st & 2nd ventricles. These two ventricles open commonly into the 3rd ventricle by a common opening called the foramen of Monro.

The presence of T solium in the ventricular system may be seen in a 7-30% of patients with NCC [45] and although it has been reported less frequently (6 %) by other authors [46] everything seems to indicate that its frequency is much greater than had been thought previously. [45] Of all forms of presentation of the NCC, the IVNCC is sometimes associated with a fatal prognosis. The diagnosis of certainty is achieved by methods of neuroimaging, particularly the nuclear magnetic resonance imaging (MRI), which is also indispensable as prior checking to surgery, the choice of treatment and assessment of the response to the same. [80-82]

Some reports related to the clinical characteristics and the management of this entity confirm their increasing frequency in countries such as the Unite States of America. [83, 84] In 1906, Ludwig Bruns described a phenomenon characterized by sudden headache associated with acute vestibular syndrome, triggered by sudden movements of the head, or, at times, it could have a disastrous outcome, by leading to coma and even death. [85] A form of presentation of the NCCIv may be syndrome Bruns characterized by episodes of loss of consciousness to head movements and hydrocephalus due to a cyst in the IV ventricle their movement obstructs the exit of CSF through the holes of Luchka and Magendie, involuntary movements or intracranial hypertension. [86] Other clinical manifestations of the NCCIv have been reported according to the location of the cysticerci in the ventricular system [87] or when passing through the same. [88] The NCCIv of the lateral ventricles has not been reported that constitutes a diagnostic or therapeutic problem however the NCCIv of the III and IV ventricles constitute a major challenge to resolve. [80,89] The treatment of forms Iv is geared toward case by case, according to the pathogenic substrate. The first thing to define is the presence or risk of acute hydrocephalus, and, therefore, the option of a ventriculostomy emergency or making a ventriculoperitoneal shunt (DVP), with or without fenestration of the septum, according to the location of the Ct. To take the decision to remove the cysts must be taken into account: the number, location stage of cysticercus, general clinical conditions of the patient, abilities, and experience of the surgical team to perform open or endoscopic neurosurgical approach. [90, 91] Also add to the list other criteria such as: the mass effect, the ventricular obstruction, the dysfunction of the valve and

the diagnostic doubt. [90] Some authors have argued that the confirmation of contrast in the wall cysticercus by MRI is a sign of a poor prognosis independent of the applied surgical treatment, as it translates the possibility of a process of ependimitis that will tend to chronicity with obstruction or entrapment, independent of the presence of the Ct. [90] The treatment of forms Iv includes not only surgical options, but also with anti-parasitic treatment (APT), corticosteroids [87, 90-93] and VPS when necessary. It is important to mention that the APT of the NCCIv should be indicated if there is a possibility to make an emergency-relief through a VPS in the case of acute obstruction. Variations in the number, volume and involution of the Ct, the secondary reactions, the eventual hydrocephalus, as well as the low number of patients who have this form of presentation, make it unlikely to carried out randomized clinical trials with an acceptable confidence. [94] On the other hand, Ramirez-Ceballos and Marquez [97] argue that the NCCIv requires an individualized treatment, medical, surgical, or a combination of both and in their experience Iv neuroendoscopic has provided a good surgical approach allowing to explore properly the ventricular cavities with either the rigid or flexible endoscope. According to these authors, this treatment improves the prognosis and clinical course of patients, since in most of them it avoids the placement of derivatives systems and in those cases that require, decreases the risk of re-surgical interventions. In this regards, we did not find articles related to the NCCIv and infection by HIV/AIDS, its clinical features and treatment options therefore it will be another aspect to be discussed below from our results.

Uncommon Clinical Manifestations of Cysticercosis 207

should be assessed carefully. The ABZ is the drug of choice, [2,3] if removal of the Ct by

Despite fourth ventricle is located in the posterior fossa, we decided to place apart here for didactic purposes, and because the fourth ventricle is the most frequent site of intraventricular infestation, a location that carries a higher risk of raise intracranial pressure due to cerebro-spinal fluid (CSF) blockage. We will comment a little bit more about this

The fourth ventricle is one of the four connected fluid-filled cavities within the human brain. These cavities, known collectively as the ventricular system, consist of the left and right lateral ventricles, the third ventricle, and the fourth ventricle. The fourth ventricle extends from the cerebral aqueduct (*aqueduct of Sylvius*) to the obex, and is filled with CSF. The fourth ventricle has a characteristic diamond shape in cross-sections of the human brain. It is located within the pons or in the upper part of the medulla. CSF entering the fourth ventricle through the cerebral aqueduct can exit to the subarachnoid space of the spinal cord

through two lateral foramina of Luschka and a single, midline foramen of Magendie.

Bruns syndrome, [110] positional vomiting [111] and comatose state [112]

literature we can conclude that the best choices for treatment of IVNCC are:

Clinical and imagenological aspects on FVNCC has been described in the medical literature [99- 108] and can be sumarized as follow: Symptoms and sings of intracraneal hypertension due to obstructive hydrocephalus [99,100-107] , blurred vision, loss of consciousness, [100] sudden death, [108] reversible parkinsonism following ventriculoperitoneal shunt, [109]

From the point of view of imagenology, the magnetic resonance imaging (MRI) is the investigation of choice which can also show: cerebrospinal fluid (CSF) signal intensity (on all pulse sequences), intra fourth ventricular cyst with scolex, and wall enhancement. On T1 weighted and Fluid Attenuation Inversion Recovery images (FAIR), the cyst wall and scolex which are not seen in other routine sequences, and the CSF flow study can show the intraluminal nature of the cyst [113] and the even imagenological appearance of multicystic

Most studies have been addressed to review the therapeutic approaches of IVNCC, as can be seen in our review. [99,110,115-127] and has been well established that available treatment options are: medical, external cerebro-spinal fluid diversion, microsurgical, or endoscopic removal alone or in combination. [122] Summarizing the reports in the medical

1. Albendazol (15 mg/kg/day for 2 weeks): A regimen of albendazole is the treatment of choice for IVNCC even after surgical procedures [122], although praziquantel may also

2. Praziquantel (100 mg/kg/day for 2 weeks): Rapid regression of a large cyst of the fourth ventricle after oral praziquantel, avoiding the need for surgery has been reported. [116]

endoscopic is not possible. [15,16,90, 91]

*Fourth ventricular neurocysticercosis (FVNCC)* 

particular location of T solium cysticercosis.

tumor. [114]

be useful. [117]

**Figure 3.** CT scan of the brain showing asymmetrical hydrocephalus due to intraventricular neurocysticercosis blocking foramen of Monro on the right side and calcified NCC.

Patients presenting intraventricular NCC (IvNCC) can have a different clinical presentation if they are HIV positive [2,3], although they have in common that the diagnosis must be confirmed by MRI as other have also pointed outr. [80, 97, 98] Should be emphasized that in HIV patients must be ruled out the presence an associated chronic meningitis by favoring the ventricular dilatation, (See figure 3) and in which case the serial lumbar punctures should be assessed carefully. The ABZ is the drug of choice, [2,3] if removal of the Ct by endoscopic is not possible. [15,16,90, 91]

#### *Fourth ventricular neurocysticercosis (FVNCC)*

206 Novel Aspects on Cysticercosis and Neurocysticercosis

will be another aspect to be discussed below from our results.

**Figure 3.** CT scan of the brain showing asymmetrical hydrocephalus due to intraventricular

Patients presenting intraventricular NCC (IvNCC) can have a different clinical presentation if they are HIV positive [2,3], although they have in common that the diagnosis must be confirmed by MRI as other have also pointed outr. [80, 97, 98] Should be emphasized that in HIV patients must be ruled out the presence an associated chronic meningitis by favoring the ventricular dilatation, (See figure 3) and in which case the serial lumbar punctures

neurocysticercosis blocking foramen of Monro on the right side and calcified NCC.

the diagnostic doubt. [90] Some authors have argued that the confirmation of contrast in the wall cysticercus by MRI is a sign of a poor prognosis independent of the applied surgical treatment, as it translates the possibility of a process of ependimitis that will tend to chronicity with obstruction or entrapment, independent of the presence of the Ct. [90] The treatment of forms Iv includes not only surgical options, but also with anti-parasitic treatment (APT), corticosteroids [87, 90-93] and VPS when necessary. It is important to mention that the APT of the NCCIv should be indicated if there is a possibility to make an emergency-relief through a VPS in the case of acute obstruction. Variations in the number, volume and involution of the Ct, the secondary reactions, the eventual hydrocephalus, as well as the low number of patients who have this form of presentation, make it unlikely to carried out randomized clinical trials with an acceptable confidence. [94] On the other hand, Ramirez-Ceballos and Marquez [97] argue that the NCCIv requires an individualized treatment, medical, surgical, or a combination of both and in their experience Iv neuroendoscopic has provided a good surgical approach allowing to explore properly the ventricular cavities with either the rigid or flexible endoscope. According to these authors, this treatment improves the prognosis and clinical course of patients, since in most of them it avoids the placement of derivatives systems and in those cases that require, decreases the risk of re-surgical interventions. In this regards, we did not find articles related to the NCCIv and infection by HIV/AIDS, its clinical features and treatment options therefore it

Despite fourth ventricle is located in the posterior fossa, we decided to place apart here for didactic purposes, and because the fourth ventricle is the most frequent site of intraventricular infestation, a location that carries a higher risk of raise intracranial pressure due to cerebro-spinal fluid (CSF) blockage. We will comment a little bit more about this particular location of T solium cysticercosis.

The fourth ventricle is one of the four connected fluid-filled cavities within the human brain. These cavities, known collectively as the ventricular system, consist of the left and right lateral ventricles, the third ventricle, and the fourth ventricle. The fourth ventricle extends from the cerebral aqueduct (*aqueduct of Sylvius*) to the obex, and is filled with CSF. The fourth ventricle has a characteristic diamond shape in cross-sections of the human brain. It is located within the pons or in the upper part of the medulla. CSF entering the fourth ventricle through the cerebral aqueduct can exit to the subarachnoid space of the spinal cord through two lateral foramina of Luschka and a single, midline foramen of Magendie.

Clinical and imagenological aspects on FVNCC has been described in the medical literature [99- 108] and can be sumarized as follow: Symptoms and sings of intracraneal hypertension due to obstructive hydrocephalus [99,100-107] , blurred vision, loss of consciousness, [100] sudden death, [108] reversible parkinsonism following ventriculoperitoneal shunt, [109] Bruns syndrome, [110] positional vomiting [111] and comatose state [112]

From the point of view of imagenology, the magnetic resonance imaging (MRI) is the investigation of choice which can also show: cerebrospinal fluid (CSF) signal intensity (on all pulse sequences), intra fourth ventricular cyst with scolex, and wall enhancement. On T1 weighted and Fluid Attenuation Inversion Recovery images (FAIR), the cyst wall and scolex which are not seen in other routine sequences, and the CSF flow study can show the intraluminal nature of the cyst [113] and the even imagenological appearance of multicystic tumor. [114]

Most studies have been addressed to review the therapeutic approaches of IVNCC, as can be seen in our review. [99,110,115-127] and has been well established that available treatment options are: medical, external cerebro-spinal fluid diversion, microsurgical, or endoscopic removal alone or in combination. [122] Summarizing the reports in the medical literature we can conclude that the best choices for treatment of IVNCC are:


3. Direct surgical excision of the parasite after a shunt procedure is performed, [115] but the complication rate is high due to obstruction or material infection, which may justify other procedures such s endoscopic third ventriculostomy, [122] as you can read below. Resection of the fourth ventricle lesion through a sub occipital approach allowed for restoration of normal cerebro-spinal fluid (CSF) flow and relief of midbrain compression. [112]

Uncommon Clinical Manifestations of Cysticercosis 209

*2.1.2. Subarachnoid NCC (SANCC)* 

bridges across them from gyrus to gyrus.

active in the blood brain barrier.

In the central nervous system, the subarachnoid space (SAE) is the interval between the arachnoid membrane and pia mater. It is occupied by spongy tissue consisting of trabeculae (delicate connective tissue filaments that extend from the arachnoid mater and blend into

This cavity is small on the surface of the hemispheres of the brain. On the summit of each gyrus the pia mater and the arachnoid are in close contact, but in the sulci between the gyri, triangular spaces are left, in which the subarachnoid trabecular tissue is found. Whilst the pia mater closely follows the surface of the brain and dips into the sulci, the arachnoid

At certain parts of the base of the brain, the arachnoid is separated from the pia mater by wide intervals, which communicate freely with each other and are named subarachnoid cisternæ; in these the subarachnoid tissue is less abundant. The subarachnoid space is the location of the interface between the vascular tissue and the cerebro-spinal fluid and is

The arachnoid mater continues down the spinal cord, and the subarachnoid layer with it. It

Despite SANCC is an uncommon location compare with intraparenchymal NCC, the

In 2004, Wallin and col. [128] reported the prevalence of SANCC in 2% while other authors reported a higher prevalence (3.5 %) in patients with SANCC with visual and hormonal disturbances secondary to a direct compression of the pituitary stalk and the optic nerve, [129,130] in some studies it was not clear whether refers to the frequency of complications by compression of the optic nerve and the pituitary stalk within the group of patients with SANCC or within a group of patients with NCC in general. As in the above situations, the treatment of choice for the SANCC is individual. [94] There are numerous reports of cases and a series of cases treated with cesticidas and corticosteroids to repetition, but not compared with a control group, to confirm this hypothesis with the additional benefit of reducing the size of the cysts or of the inflammatory leptomeningeal process. [131,132] Despite the lack of evidence which originated in randomized clinical trials, there is consensus for this option [133] and other authors combine prolonged treatments (sometimes for several months) with cesticidas and corticosteroids. [134] According to some authors the reason why the clinical manifestations of ischemia with or without infarction, due to cerebral vasculitis infectious by NCC, were not detected in some patients is due to the age group with the highest incidence have no aggregated vascular risk factors. The cerebrovascular complications are predominantly associated with the prevalence of the CC especially in the basal cisterns and associated arachnoiditis of variable degree [133, 135-139] later we will discuss this aspect based on our experience and results. It is known that when cisticerci are confined to a small area with associated arachnoiditis and few changes in the CSF, these affect small blood vessels predominantly penetrating arterioles causing

commonest location in the subarachnoid space is over the cerebral hemispheres.

serves a similar function in the spinal cord as it does in the brain.

the pia mater) and intercommunicating channels in which the CSF is contained.


## *2.1.2. Subarachnoid NCC (SANCC)*

208 Novel Aspects on Cysticercosis and Neurocysticercosis

secondary to mass effect.[119]

placement of ventriculoperitoneal shunt. [110]

compression. [112]

3. Direct surgical excision of the parasite after a shunt procedure is performed, [115] but the complication rate is high due to obstruction or material infection, which may justify other procedures such s endoscopic third ventriculostomy, [122] as you can read below. Resection of the fourth ventricle lesion through a sub occipital approach allowed for restoration of normal cerebro-spinal fluid (CSF) flow and relief of midbrain

4. If pericystic enhancement is present on MRI, shunt surgery should be performed, and craniotomy reserved for treatment of those patients with symptomatic lesions

5. Endoscopy approach: Neuroendoscopy provides a safe and effective management modality for the treatment of a variety of intracranial disorders, either temporal or nontumoral, congenital, developmental, and degenerative, and its knowledge, indications, and limits are fundamental for the armamentarium of the modern neurosurgeon. [126] In patients presenting symptomatic obstructive hydrocephalus, aqueductal and foramen of Monro dilatations, endoscopic neurosurgery is a minimally invasive technique enabling removal of intraventricular cyticercal cysts from all locations, avoiding major craniotomies/posterior fossa explorations and shunts. The flexible fiberoptic scope is used for excising cysts in the fourth ventricle, through a transaqueductal route and patients should be treated with albendazole in the postoperative period. [118, 120, 122] Endoscopic third ventriculostomy for patients with ventricular outlet obstruction and a prior history of FVNCC has been reported as a successful management and no patient has required subsequent surgery. [121, 122] Endoscopic transaqueductal removal of fourth ventricular neurocysticercosis with an angiographic catheter has been used and there are good experiences from two series of 10 and 21 patients with IVNCC with obstructive hydrocephalus that underwent endoscopic removal along with endoscopic third ventriculostomy through a frontal precoronal burr hole. The author concluded that a 30-degree rigid telescope provide excellent image quality, with the ability to address intra-FVNCC through the dilated aqueduct with a curved-tip catheter without significant operative or postoperative complications. They concluded that neuro-endoscopic surgery is an effective treatment modality for patients with intraventricular NCC. It effectively restores CSF flow and is capable of removing cysts completely or partially from accessible locations causing mass effect. Partial removal or rupture of the cyst does not affect the clinical outcome of the patients. [123, 124] Other series of patients (n-13) presenting FVNCC with complete excised of cyst by using a transventricular, transaqueductal "scope-in-scope" endoscopic technique was reported and their author also concluded that endoscopic fourth ventricular cysticercal cyst excision along with internal cerebrospinal fluid diversion via endoscopic third ventriculostomy is an effective alternative to open microneurosurgical procedures and avoids shunt placement and its related complications [125] such as: Parkinsonism with evidence of midbrain dysfunction due to aqueductal stenosis after In the central nervous system, the subarachnoid space (SAE) is the interval between the arachnoid membrane and pia mater. It is occupied by spongy tissue consisting of trabeculae (delicate connective tissue filaments that extend from the arachnoid mater and blend into the pia mater) and intercommunicating channels in which the CSF is contained.

This cavity is small on the surface of the hemispheres of the brain. On the summit of each gyrus the pia mater and the arachnoid are in close contact, but in the sulci between the gyri, triangular spaces are left, in which the subarachnoid trabecular tissue is found. Whilst the pia mater closely follows the surface of the brain and dips into the sulci, the arachnoid bridges across them from gyrus to gyrus.

At certain parts of the base of the brain, the arachnoid is separated from the pia mater by wide intervals, which communicate freely with each other and are named subarachnoid cisternæ; in these the subarachnoid tissue is less abundant. The subarachnoid space is the location of the interface between the vascular tissue and the cerebro-spinal fluid and is active in the blood brain barrier.

The arachnoid mater continues down the spinal cord, and the subarachnoid layer with it. It serves a similar function in the spinal cord as it does in the brain.

Despite SANCC is an uncommon location compare with intraparenchymal NCC, the commonest location in the subarachnoid space is over the cerebral hemispheres.

In 2004, Wallin and col. [128] reported the prevalence of SANCC in 2% while other authors reported a higher prevalence (3.5 %) in patients with SANCC with visual and hormonal disturbances secondary to a direct compression of the pituitary stalk and the optic nerve, [129,130] in some studies it was not clear whether refers to the frequency of complications by compression of the optic nerve and the pituitary stalk within the group of patients with SANCC or within a group of patients with NCC in general. As in the above situations, the treatment of choice for the SANCC is individual. [94] There are numerous reports of cases and a series of cases treated with cesticidas and corticosteroids to repetition, but not compared with a control group, to confirm this hypothesis with the additional benefit of reducing the size of the cysts or of the inflammatory leptomeningeal process. [131,132] Despite the lack of evidence which originated in randomized clinical trials, there is consensus for this option [133] and other authors combine prolonged treatments (sometimes for several months) with cesticidas and corticosteroids. [134] According to some authors the reason why the clinical manifestations of ischemia with or without infarction, due to cerebral vasculitis infectious by NCC, were not detected in some patients is due to the age group with the highest incidence have no aggregated vascular risk factors. The cerebrovascular complications are predominantly associated with the prevalence of the CC especially in the basal cisterns and associated arachnoiditis of variable degree [133, 135-139] later we will discuss this aspect based on our experience and results. It is known that when cisticerci are confined to a small area with associated arachnoiditis and few changes in the CSF, these affect small blood vessels predominantly penetrating arterioles causing

infarctions very similar to the lacunar infarctions of the basal ganglia which did not differ from those caused by arteriopatías or microateromas. [94] The most severe form of vasculopathy infectious for NCC is observed when the CC spreads through the SAE with major alterations of the LCR and a chronic evolution of the disease. The neurovascular syndrome (SNv) is usually the first manifestation of the focal SANCC that produces unique infarcts (90 %), which often have a good prognosis. [94] In contrast, many patients have nonvascular complications (including intracranial hypertension and a progressive neurological impairment) when the NCC is diffusely extended in the CNS, whereas the SNv occurs only late in the course of the disease and has a grim forecast. [94] We have not found in the reviewed medical literature references related to the SANCC (See figure 4) and cerebrovascular complications in patients with HIV/AIDS.

Uncommon Clinical Manifestations of Cysticercosis 211

medical literature. Its frequency varies between 7 and 5.8 % and its incidence is very low but six times more on that in axial medullary. [140, 141] Other authors in 2002 reviewed of

Its clinical manifestations are the result of the presence of CC in the intra-spinal canal i.e. the space between the meninges of the spinal canal.[143] Other authors have found up to 200 cases reported in the international medical literature before the year 2001, [144] this low prevalence of the IMNCC is explained by the fact that the blood supply to the spinal cord is 100 times less than the blood flow to the brain [145, 146] and the Ct usually comes to the region's best irrigated. Most of the times the symptoms of the ISNCC are secondary to the mass effect or the secondary edema, and among the most common can be found: spastic paraplegia, bladder incontinence, radicular pain or sexual impotence; the degree of commitment depends on the site which locate the lesion. [147] When there is an associated arachnoiditis (EMNCC) dominate the radicular pain and muscle weakness and when it affects the axial medullary then the predominant clinical feature is similar to the transverse myelitis characterized by sphincter dysfunction with motor and sensory signs below the

In ISNCC the subarachnoid damage can be secondary to its spread through the SAE, and the more usual imaging presentation is the EMNCC and arachnoiditis. The IMNCC presentation has been associated with hematogenous spread or through the spinal canal; the latter is seen as expansive lesions with a ring enhancement after contrast injection. [141] This presentation is extremely rare, and the lesions may be single or multiple, or even with the appearance of clusters. [149] When there is a degenerative process of the cystic lesion can also be seen this thickening in the ring of the cystic wall after the administration of contrast during a CT scan of the thoracic spine. [141,143, 147,150-152] It is equally unusual the damage of the spinal canal and the most frequent space occupies lesion to level EM. As we before-mentioned, on very rare occasions IMNCC is confirmed. [153] The differential diagnosis for the IMNCC presentation includes the IM abscess [154], other parasitic diseases such as hydatid cysts, neoplasias (ependymomas), traumatic lesions, sarcoidosis [155], arachnoid cysts [156], ependymal cysts [157] and neuroenteric cysts. [158] In the presentation EM include congenital cystic lesions (dermoides cysts); infectious diseases, demyelinating disorders, inflammatory or granulomatous, such as tuberculosis and sarcoidosis, and neoplasms. The region of provenance of the patient and the characteristics of the MRI images help to establish the difference. [159,160] For this type of presentation there is a general consensus on the poor knowledge and limited experience of the problem. Surgical treatment is recommended alone or combined with APD and corticoids [133, 161], although others believe that surgery is the best treatment option for NCCIm counting with current techniques of microsurgery. [141] The recovery of patients with IMNCC with surgical treatment is fast, with ranges of a week with maximum recovery of 3 months after surgery. [162] Although they have also reported irreversible spinal injuries by NCCIm that logically do not recover with surgical intervention. [141] Finally, there are individual differences from the clinical point of view, imaging and of response to treatment according

95 published cases of medullary cysticercosis since 1856. [142]

level of the lesion. [148]

**Figure 4.** Racemose cystic lesions in left frontal and both temporal lobes. Intraventricular cyst causing obstructive hydrocephalus.

The SANCC also has some different features in the South African patient due to their frequent association with HIV infection. As is known, scattered Ct in the SAE cause the most severe forms of infectious vasculitis when evolve toward the chronicity [ 94], although no one knows the exact prevalence of this problem. [ 16] In our study we were able to determine that the risk to present an ischemic infarct is almost three times higher in patient with SANCC compared with the patient with intraparenchymal NCC and this risk increase more than seven times if the patient is HIV-positive. Therefore you have to exhaust all the possibilities of deworming to avoid cerebrovascular complications. [16]

### *2.1.3. Intraspinal NCC*

The intra-spinal NCC (ISNCC) presentation include: Intra-medullary NCC (IMNCC) and extra-medullary NCC (EMNCC). The ISNCC is rare and there are only 45 cases reported in medical literature. Its frequency varies between 7 and 5.8 % and its incidence is very low but six times more on that in axial medullary. [140, 141] Other authors in 2002 reviewed of 95 published cases of medullary cysticercosis since 1856. [142]

210 Novel Aspects on Cysticercosis and Neurocysticercosis

obstructive hydrocephalus.

*2.1.3. Intraspinal NCC* 

cerebrovascular complications in patients with HIV/AIDS.

infarctions very similar to the lacunar infarctions of the basal ganglia which did not differ from those caused by arteriopatías or microateromas. [94] The most severe form of vasculopathy infectious for NCC is observed when the CC spreads through the SAE with major alterations of the LCR and a chronic evolution of the disease. The neurovascular syndrome (SNv) is usually the first manifestation of the focal SANCC that produces unique infarcts (90 %), which often have a good prognosis. [94] In contrast, many patients have nonvascular complications (including intracranial hypertension and a progressive neurological impairment) when the NCC is diffusely extended in the CNS, whereas the SNv occurs only late in the course of the disease and has a grim forecast. [94] We have not found in the reviewed medical literature references related to the SANCC (See figure 4) and

**Figure 4.** Racemose cystic lesions in left frontal and both temporal lobes. Intraventricular cyst causing

The SANCC also has some different features in the South African patient due to their frequent association with HIV infection. As is known, scattered Ct in the SAE cause the most severe forms of infectious vasculitis when evolve toward the chronicity [ 94], although no one knows the exact prevalence of this problem. [ 16] In our study we were able to determine that the risk to present an ischemic infarct is almost three times higher in patient with SANCC compared with the patient with intraparenchymal NCC and this risk increase more than seven times if the patient is HIV-positive. Therefore you have to exhaust all the

The intra-spinal NCC (ISNCC) presentation include: Intra-medullary NCC (IMNCC) and extra-medullary NCC (EMNCC). The ISNCC is rare and there are only 45 cases reported in

possibilities of deworming to avoid cerebrovascular complications. [16]

Its clinical manifestations are the result of the presence of CC in the intra-spinal canal i.e. the space between the meninges of the spinal canal.[143] Other authors have found up to 200 cases reported in the international medical literature before the year 2001, [144] this low prevalence of the IMNCC is explained by the fact that the blood supply to the spinal cord is 100 times less than the blood flow to the brain [145, 146] and the Ct usually comes to the region's best irrigated. Most of the times the symptoms of the ISNCC are secondary to the mass effect or the secondary edema, and among the most common can be found: spastic paraplegia, bladder incontinence, radicular pain or sexual impotence; the degree of commitment depends on the site which locate the lesion. [147] When there is an associated arachnoiditis (EMNCC) dominate the radicular pain and muscle weakness and when it affects the axial medullary then the predominant clinical feature is similar to the transverse myelitis characterized by sphincter dysfunction with motor and sensory signs below the level of the lesion. [148]

In ISNCC the subarachnoid damage can be secondary to its spread through the SAE, and the more usual imaging presentation is the EMNCC and arachnoiditis. The IMNCC presentation has been associated with hematogenous spread or through the spinal canal; the latter is seen as expansive lesions with a ring enhancement after contrast injection. [141] This presentation is extremely rare, and the lesions may be single or multiple, or even with the appearance of clusters. [149] When there is a degenerative process of the cystic lesion can also be seen this thickening in the ring of the cystic wall after the administration of contrast during a CT scan of the thoracic spine. [141,143, 147,150-152] It is equally unusual the damage of the spinal canal and the most frequent space occupies lesion to level EM. As we before-mentioned, on very rare occasions IMNCC is confirmed. [153] The differential diagnosis for the IMNCC presentation includes the IM abscess [154], other parasitic diseases such as hydatid cysts, neoplasias (ependymomas), traumatic lesions, sarcoidosis [155], arachnoid cysts [156], ependymal cysts [157] and neuroenteric cysts. [158] In the presentation EM include congenital cystic lesions (dermoides cysts); infectious diseases, demyelinating disorders, inflammatory or granulomatous, such as tuberculosis and sarcoidosis, and neoplasms. The region of provenance of the patient and the characteristics of the MRI images help to establish the difference. [159,160] For this type of presentation there is a general consensus on the poor knowledge and limited experience of the problem. Surgical treatment is recommended alone or combined with APD and corticoids [133, 161], although others believe that surgery is the best treatment option for NCCIm counting with current techniques of microsurgery. [141] The recovery of patients with IMNCC with surgical treatment is fast, with ranges of a week with maximum recovery of 3 months after surgery. [162] Although they have also reported irreversible spinal injuries by NCCIm that logically do not recover with surgical intervention. [141] Finally, there are individual differences from the clinical point of view, imaging and of response to treatment according

to the number and location of the lesions, that determine the characteristics of the presentations described [117,161] and which are by describing such as: medullary central cord syndrome, medullary conus and the horsetail, when this happens. In the meantime, report of isolated cases continues to unfold with more or less variations between the same. [162,163] We report a HIV patient presenting intraspinal calcified NCC and an associated tuberculosis of the spinal. [11] The clinical and imagenological features of NCC and TB of the spine in HIV-positive patients have not been previously reported. (See figure 5)

Uncommon Clinical Manifestations of Cysticercosis 213

**Figure 6.** Multiplanar, multisequence MRI of the spine with IV contrast: Shows intramedullary ring enhancing lesion at the level of T11/T12 with enhancement of the meninges and nerve roots due to tuberculous radiculomyelitis, tuberculoma and calcified cysticercosis in the paravertebral muscles.

In the chapter entitled: "What is a low frequency of the disseminated cysticercosis suggests that neurocysticercosis is going to disappear?" Our readership will find detailed information about this uncommon presentation of cysticercosis. Therefore in this chapter we

Disseminated cysticercosis (DCC) was reported in 1912 by doctors of the English Navy stationed in India. An intensive search in PubMed revealed 22 cases reported by Wadia and collaborators in 1988 and only 16 additional cases were reported until 2006. [167] Two years later Bhalla et al., (2008) argued that fewer than 50 cases have been reported in the international medical literature most of them from India. [168] The characteristic features of the DCC is epilepsy, subcutaneous cysticercosis, the pseudo-hypertrophy in the four members and the allocation of any other organ or system. [164] However, a manifestation fairly typical of the DCC may be a subcutaneous cyst that occurs as an asymptomatic nodule. These subcutaneous nodules can be sliders in the muscle and measure up to 1.5 cm. (See figure 6) In the case of patients who have had for more than 5 years cysticerci, these cysts are calcified. [169] Finally, there are individual differences from the clinical point of view, imaging and of response to treatment according to the number and location of the lesions, that determine the characteristics of the described presentations. [117,160]

The unusual location of the cysts may result in uncommon manifestations mimicking a host of neurological disorders. Thus, a high level of suspicion should be kept for NCC, especially

*Disseminated cysticercosis* 

are going to mention only some remarkable issues.

Neurocysticercosis is always present in patients with DCC.

**3. Other uncommon presentations of neurocysticercosis** 

We have also seen patients with intramedullary granulomatous lesions and associated paravertebral calcified cysticercosis confirmed by biopsy and antigen/antibody cysticercosis serology test. (See figure 6)

**Figure 5.** Shows calcified intramedullary calcified lesion and tuberculosis of the spine.

We will not discuss this observation in this chapter because we consider that this is just an anecdotal case that provides only casual interesting information. Some author thinks that EMNCC is explained by the downward migration of larvae from the brain to the spinal subarachnoid space and most larvae are expected to be stopped in the upper portions of the spinal canal due to peculiarities of the anatomy of the spinal cord; because it is hard to image cysticerci passing throughout foramen of Luscka and Magendie others authors support the hematogenous route. [11, 142, 163] Spinal intramedullary cysticercosis involving the conus medullaris is an extremelly uncommon clinical condition that may mimic an intramedullary tumor. It should be kept in mind in the differential diagnosis of intramedullary conus lesions and to rememeber that it can lead to irreversible neurological deficits if untreated.[164] Another clinical presentation is spastic paraplegia in patients presenting dorsal IMNCC but in some of them the neurological deficit may persist even after successful cyst excision. [165] Coexisting intramedullary schwannoma with intramedullary cysticercus has been reported. [166]

**Figure 6.** Multiplanar, multisequence MRI of the spine with IV contrast: Shows intramedullary ring enhancing lesion at the level of T11/T12 with enhancement of the meninges and nerve roots due to tuberculous radiculomyelitis, tuberculoma and calcified cysticercosis in the paravertebral muscles.

#### *Disseminated cysticercosis*

212 Novel Aspects on Cysticercosis and Neurocysticercosis

serology test. (See figure 6)

to the number and location of the lesions, that determine the characteristics of the presentations described [117,161] and which are by describing such as: medullary central cord syndrome, medullary conus and the horsetail, when this happens. In the meantime, report of isolated cases continues to unfold with more or less variations between the same. [162,163] We report a HIV patient presenting intraspinal calcified NCC and an associated tuberculosis of the spinal. [11] The clinical and imagenological features of NCC and TB of

We have also seen patients with intramedullary granulomatous lesions and associated paravertebral calcified cysticercosis confirmed by biopsy and antigen/antibody cysticercosis

the spine in HIV-positive patients have not been previously reported. (See figure 5)

**Figure 5.** Shows calcified intramedullary calcified lesion and tuberculosis of the spine.

intramedullary cysticercus has been reported. [166]

We will not discuss this observation in this chapter because we consider that this is just an anecdotal case that provides only casual interesting information. Some author thinks that EMNCC is explained by the downward migration of larvae from the brain to the spinal subarachnoid space and most larvae are expected to be stopped in the upper portions of the spinal canal due to peculiarities of the anatomy of the spinal cord; because it is hard to image cysticerci passing throughout foramen of Luscka and Magendie others authors support the hematogenous route. [11, 142, 163] Spinal intramedullary cysticercosis involving the conus medullaris is an extremelly uncommon clinical condition that may mimic an intramedullary tumor. It should be kept in mind in the differential diagnosis of intramedullary conus lesions and to rememeber that it can lead to irreversible neurological deficits if untreated.[164] Another clinical presentation is spastic paraplegia in patients presenting dorsal IMNCC but in some of them the neurological deficit may persist even after successful cyst excision. [165] Coexisting intramedullary schwannoma with In the chapter entitled: "What is a low frequency of the disseminated cysticercosis suggests that neurocysticercosis is going to disappear?" Our readership will find detailed information about this uncommon presentation of cysticercosis. Therefore in this chapter we are going to mention only some remarkable issues.

Disseminated cysticercosis (DCC) was reported in 1912 by doctors of the English Navy stationed in India. An intensive search in PubMed revealed 22 cases reported by Wadia and collaborators in 1988 and only 16 additional cases were reported until 2006. [167] Two years later Bhalla et al., (2008) argued that fewer than 50 cases have been reported in the international medical literature most of them from India. [168] The characteristic features of the DCC is epilepsy, subcutaneous cysticercosis, the pseudo-hypertrophy in the four members and the allocation of any other organ or system. [164] However, a manifestation fairly typical of the DCC may be a subcutaneous cyst that occurs as an asymptomatic nodule. These subcutaneous nodules can be sliders in the muscle and measure up to 1.5 cm. (See figure 6) In the case of patients who have had for more than 5 years cysticerci, these cysts are calcified. [169] Finally, there are individual differences from the clinical point of view, imaging and of response to treatment according to the number and location of the lesions, that determine the characteristics of the described presentations. [117,160] Neurocysticercosis is always present in patients with DCC.

## **3. Other uncommon presentations of neurocysticercosis**

The unusual location of the cysts may result in uncommon manifestations mimicking a host of neurological disorders. Thus, a high level of suspicion should be kept for NCC, especially in endemic zones and developing countries. Uncommon clinical presentations that demonstrate the spectrum of pleomorphism of NCC include extrapyramidal disease (parkinsonism and focal dystonia), Kluver-Bucy syndrome, Weber's syndrome, dementia and cortical blindness,[170] other authors include dorsal midbrain syndrome, isolated bilateral ptosis, papillitis, cerebral hemorrhage, painful cervical radiculopathy, progressive swelling of arm, paraplegia due to intramedullary cyst, third ventricular cyst, dystonia and nominal aphasia masquerading as transient ischaemic attacks, [171] and an associated pachymeningitis of the cervical spinal cord.[172]

Uncommon Clinical Manifestations of Cysticercosis 215

Anatomically, it comprises the tectum (or corpora quadrigemina), tegmentum and the cerebral peduncles, as well as several nuclei and fasciculi. Caudally the mesencephalon adjoins the pons (metencephalon) and rostrally it adjoins the diencephalon (Thalamus, hypothalamus, etc.). The midbrain is located below the cerebral cortex, and above the hindbrain placing it near the center of the brain. The clinical features of the midbrain

The pretectal area, or pretectum, is a midbrain structure composed of seven nuclei and comprises part of the subcortical visual system. Through reciprocal bilateral projections to the retina, it is primarily involved in mediating pupillary light reflex, the optokinetic reflex, the accommodation reflex, and temporary changes to the circadian rhythm. In addition to the pretectum's role in the visual system, the anterior pretectal nucleus has been found to mediate somatosensory and nociceptive information. Parinaud's Syndrome, also known as a dorsal midbrain syndrome (Pretectal syndrome) is a group of abnormalities of eye movement and pupil dysfunction. It is caused by lesions of the upper brain stem and is named for Henri Parinaud (1844–1905), considered to be the father of French

As before-mentioned, isolated brainstem involvement in patients who have neurocysticercosis is rare and symptoms and signs of MIDNCC can vary from one reported

The symptoms of NCC of the pretectal area are nonspecific, but abnormal pupils, vertical gaze limitation, disjunctive horizontal and vertical eye position, lid retraction, and

Superior alternating hemiplegia is usually a form of stroke characterized by the presence of an oculomotor nerve palsy and contralateral hemiparesis or hemiplegia but cases secondary to MIDNCC (acute onset right 3rd nerve palsy with left hemiplegia) has been reported. [176]

Oculomotor nerve palsy is an eye condition resulting from damage to the third cranial nerve or a branch thereof. As the name suggests, the oculomotor nerve supplies the majority of the muscles controlling eye movements. Thus, damage to this nerve will result in the affected individual being unable to move their eyes normally. In addition, the nerve also supplies the upper eyelid muscle (Levator palpebrae superioris) and the muscles responsible for pupil constriction (sphincter pupillae). The limitations of eye movements resulting from the condition are generally so severe that the affected individual is unable to maintain normal alignment of their eyes when looking straight ahead, leading to strabismus and, as a consequence, double vision (diplopia). In the chapter entitled: "Orbital cysticercosis.A challenger for neurologists, ophthalmologists, neuro-ophthalmologists, and general practitioner" more detailed information

patient to another according to the exact location of *T solium* NCC. [174,175,181,182]

neurocysticercosis (MIDNCC) could be summarized as follows:

convergence-retraction nystagmus can be seen.[181]

about extraocular muscle palsy due to cysticercosis is available.

*1. Pretectal syndrome* 

ophthalmology.

*2. Weber syndrome* 

*3. Third cranial nerve palsy* 

## **3.1. Brainstem neurocysticercosis**

## *3.1.1. Midbrain, pons and medulla oblongata NCC*

In vertebrate anatomy the brainstem (or brain stem) is the posterior part of the brain, adjoining and structurally continuous with the spinal cord. The brain stem provides the main motor and sensory innervation to the face and neck via the cranial nerves. This is an extremely important part of the brain as the nerve connections of the motor and sensory systems from the main part of the brain to the rest of the body pass through the brain stem. This includes the corticospinal tract (motor), the posterior column-medial lemniscus pathway (fine touch, vibration sensation and proprioception) and the spinothalamic tract (pain, temperature, itch and crude touch). The brain stem also plays an important role in the regulation of cardiac and respiratory function. It also regulates the central nervous system, and is pivotal in maintaining consciousness and regulating the sleep cycle. The brain stem has many basic functions including heart rate, breathing, sleeping and eating. It is usually described as including the medulla oblongata (myelencephalon), pons (part of metencephalon), and midbrain (mesencephalon).Less frequently, parts of the diencephalon are included.

Isolated brainstem involvement in patients who have neurocysticercosis is rare, usually, it occurs in association with disseminated forms of cysticercosis and some patients can present acute midbrain syndrome responding well to corticosteroids [173,178-180] although tapering of corticosteroids should be performed very slowly in such cases [174] good response to albendazol therapy [175, 177, 178,179] and spontaneous resolution have been reported. The importance of recognizing this entity allows to avoid unnecessary surgical intervention, empirical anti-tuberculous chemotherapy, [176] and to recommend a conservative approach to case management, including observation. [175] The segment of the brainstem most frequently affected by the larval stage of the *T solium* is the midbrain, followed by the pons and in some anecdotal cases the medulla oblongata. We will comment a little bit more about this presentation.

#### *3.1.2. Midbrain neurocysticercosis*

The midbrain or mesencephalon is a portion of the central nervous system associated with vision, hearing, motor control, sleep/wake, arousal (alertness), and temperature regulation. Anatomically, it comprises the tectum (or corpora quadrigemina), tegmentum and the cerebral peduncles, as well as several nuclei and fasciculi. Caudally the mesencephalon adjoins the pons (metencephalon) and rostrally it adjoins the diencephalon (Thalamus, hypothalamus, etc.). The midbrain is located below the cerebral cortex, and above the hindbrain placing it near the center of the brain. The clinical features of the midbrain neurocysticercosis (MIDNCC) could be summarized as follows:

#### *1. Pretectal syndrome*

214 Novel Aspects on Cysticercosis and Neurocysticercosis

pachymeningitis of the cervical spinal cord.[172]

*3.1.1. Midbrain, pons and medulla oblongata NCC* 

**3.1. Brainstem neurocysticercosis** 

a little bit more about this presentation.

*3.1.2. Midbrain neurocysticercosis* 

are included.

in endemic zones and developing countries. Uncommon clinical presentations that demonstrate the spectrum of pleomorphism of NCC include extrapyramidal disease (parkinsonism and focal dystonia), Kluver-Bucy syndrome, Weber's syndrome, dementia and cortical blindness,[170] other authors include dorsal midbrain syndrome, isolated bilateral ptosis, papillitis, cerebral hemorrhage, painful cervical radiculopathy, progressive swelling of arm, paraplegia due to intramedullary cyst, third ventricular cyst, dystonia and nominal aphasia masquerading as transient ischaemic attacks, [171] and an associated

In vertebrate anatomy the brainstem (or brain stem) is the posterior part of the brain, adjoining and structurally continuous with the spinal cord. The brain stem provides the main motor and sensory innervation to the face and neck via the cranial nerves. This is an extremely important part of the brain as the nerve connections of the motor and sensory systems from the main part of the brain to the rest of the body pass through the brain stem. This includes the corticospinal tract (motor), the posterior column-medial lemniscus pathway (fine touch, vibration sensation and proprioception) and the spinothalamic tract (pain, temperature, itch and crude touch). The brain stem also plays an important role in the regulation of cardiac and respiratory function. It also regulates the central nervous system, and is pivotal in maintaining consciousness and regulating the sleep cycle. The brain stem has many basic functions including heart rate, breathing, sleeping and eating. It is usually described as including the medulla oblongata (myelencephalon), pons (part of metencephalon), and midbrain (mesencephalon).Less frequently, parts of the diencephalon

Isolated brainstem involvement in patients who have neurocysticercosis is rare, usually, it occurs in association with disseminated forms of cysticercosis and some patients can present acute midbrain syndrome responding well to corticosteroids [173,178-180] although tapering of corticosteroids should be performed very slowly in such cases [174] good response to albendazol therapy [175, 177, 178,179] and spontaneous resolution have been reported. The importance of recognizing this entity allows to avoid unnecessary surgical intervention, empirical anti-tuberculous chemotherapy, [176] and to recommend a conservative approach to case management, including observation. [175] The segment of the brainstem most frequently affected by the larval stage of the *T solium* is the midbrain, followed by the pons and in some anecdotal cases the medulla oblongata. We will comment

The midbrain or mesencephalon is a portion of the central nervous system associated with vision, hearing, motor control, sleep/wake, arousal (alertness), and temperature regulation. The pretectal area, or pretectum, is a midbrain structure composed of seven nuclei and comprises part of the subcortical visual system. Through reciprocal bilateral projections to the retina, it is primarily involved in mediating pupillary light reflex, the optokinetic reflex, the accommodation reflex, and temporary changes to the circadian rhythm. In addition to the pretectum's role in the visual system, the anterior pretectal nucleus has been found to mediate somatosensory and nociceptive information. Parinaud's Syndrome, also known as a dorsal midbrain syndrome (Pretectal syndrome) is a group of abnormalities of eye movement and pupil dysfunction. It is caused by lesions of the upper brain stem and is named for Henri Parinaud (1844–1905), considered to be the father of French ophthalmology.

As before-mentioned, isolated brainstem involvement in patients who have neurocysticercosis is rare and symptoms and signs of MIDNCC can vary from one reported patient to another according to the exact location of *T solium* NCC. [174,175,181,182]

The symptoms of NCC of the pretectal area are nonspecific, but abnormal pupils, vertical gaze limitation, disjunctive horizontal and vertical eye position, lid retraction, and convergence-retraction nystagmus can be seen.[181]

*2. Weber syndrome* 

Superior alternating hemiplegia is usually a form of stroke characterized by the presence of an oculomotor nerve palsy and contralateral hemiparesis or hemiplegia but cases secondary to MIDNCC (acute onset right 3rd nerve palsy with left hemiplegia) has been reported. [176]

*3. Third cranial nerve palsy* 

Oculomotor nerve palsy is an eye condition resulting from damage to the third cranial nerve or a branch thereof. As the name suggests, the oculomotor nerve supplies the majority of the muscles controlling eye movements. Thus, damage to this nerve will result in the affected individual being unable to move their eyes normally. In addition, the nerve also supplies the upper eyelid muscle (Levator palpebrae superioris) and the muscles responsible for pupil constriction (sphincter pupillae). The limitations of eye movements resulting from the condition are generally so severe that the affected individual is unable to maintain normal alignment of their eyes when looking straight ahead, leading to strabismus and, as a consequence, double vision (diplopia). In the chapter entitled: "Orbital cysticercosis.A challenger for neurologists, ophthalmologists, neuro-ophthalmologists, and general practitioner" more detailed information about extraocular muscle palsy due to cysticercosis is available.

Isolated third cranial nerve palsy is usually due to vascular causes like posterior communicating artery aneurysm and it is rare in NCC and is usually caused by supratentorial or sub-arachnoid lesions with accompanying hydrocephalus or meningitis however patients presenting third cranial nerve palsy caused by neurocysticercosis involving the midbrain has been reported. [174, 183]

Uncommon Clinical Manifestations of Cysticercosis 217

Clinical features of pontine neurocyticercosis (PNCC) also depends of different locations of

Internuclear ophthalmoplegia (INO) is a disorder of conjugate lateral gaze in which the affected eye shows impairment of adduction. When an attempt is made to gaze contralaterally (relative to the affected eye), the affected eye adducts minimally, if at all. The contralateral eye abducts, however with nystagmus. Additionally, the divergence of the eyes leads to horizontal diplopia. That is, if the right eye is affected the patient will "see double" when looking to the left, seeing two images side-by-side. Convergence is generally preserved. Clinical presentations of INO can be divided into: unilateral INO, bilateral INO, and one-and-a-half syndrome in a similar descending frequency. [185] Perhaps the more difficult clinical diagnosis to perform is the One-and-a-half syndrome (OAHS). It is a neurological disorder of the extra ocular movements characterized by conjugate horizontal gaze palsy in one direction and internuclear ophthalmoplegia (INO) in the other. The commonest cause of the syndrome is a vascular lesion on the basilar territory followed by demyelinating lesions of the brainstem usually affecting the paramedian pontine reticular formation unilaterally or due to lesions on the abducens nucleus on one side, with interruption of internuclear fibbers of the ipsilateral medial longitudinal fasciculus after it has crossed the midline from its side of origin in the contra lateral abducens nucleus causing

In 2004, we proposed a new classification for OAHS based on our finding in patients with NCC and cerebrovascular diseases and we described the clinical features of One-and-a-half "plus" syndrome. We found patients with other combinations that can be called as: Fiveand-a-half syndrome (1 ½ + 4-throclear nerve), Seven-and-a-half syndrome (1 ½ + 6 abducens) or Eleven-and-a-half syndrome (1 ½ + 7-facial nerve + 3-oculomotor nerve) due to racemose NCC, and midbrain lesions related to NCC. However we honestly considered at that time and even today that a generation of novel arithmetic combinations will bring more confusion than clarity to this problem and will increase unnecessarily the long list of different names of neuro-ophthalmological disorders, therefore we concluded that adding the term "plus" was good enough to distinguish these conditions from the typical OAHS. [186] Five years later Ranjith et al [178] studied an 18-year-old male presented with a history of sudden-onset diplopia of 1-week duration and blurring of vision. He had no history of headache, fever, seizures, walking difficulty, previous head injury, diabetes or hypertension. There was no history of a similar episode in the past. Diplopia was found to increase while looking to the left. Findings from general examination were normal. The left eye was deviated outward, and the right eye was central in primary gaze. There was a weakness of adduction in the left eye and paralysis of all conjugate horizontal eye movements in the right eye. The left eye also showed nystagmus while the patient attempted left lateral gaze. Vertical eye movements were normal, and no ocular bobbing was observed. There was no long tract sign, and the cerebellar system was normal. They confirmed OAHS secondary to

the T solium at the pontine level such as:

failure of adduction of the ipsilateral eye. [186]

PNCC.

*1. Internuclear ophthalmoplegia* 

#### 4. *Claude's syndrome*

Claude's syndrome is a distinctive brainstem syndrome characterized by ipsilateral third cranial nerve palsy with contralateral hemiataxia and is due to an intrinsic or extrinsic lesion in the midbrain. The most common cause of Claude's syndrome is ischemic stroke on the midbrain however one patient presenting Claude's syndrome secondary to MIDNCC is reported. Therefore, if we encounter Claude's syndrome, we should consider neurocysticercosis infection as one of the etiologic factors [179]

#### *5. Superior divisional oculomotor nerve palsy*

Superior divisional oculomotor nerve palsy caused by intrinsic brain stem disease occurs rarely. The first case of midbrain neurocysticercosis presenting as isolated superior divisional paresis of the oculomotor nerve was communicated by Chotmongkol et al,. in 2006. [177] They reported a 25-year-old woman presented with acute onset of headache and ptosis-upgaze palsy of the right eye. This is an anecdotal case but serve to keep in mind MIDNCC for the differential diagnosis in similar cases.

*6. Sudden-onset ptosis [184]* 

Sudden onset of ptosis secondary to MIDNCC was identified in 2008 and the first two children were informed to the medical literature by Singhi et al. [184] Recently Naphade et al., [180] reported the case of a 45-year-old woman who presented with acute onset of bilateral symmetrical ptosis with no other neurological deficit. Her imaging revealed a single cystic ring-enhancing lesion with perilesional oedema in the midbrain suggestive of NCC.

### *3.1.3. Pontine neurocysticercosis*

The pons is a structure located on the brain stem, named after the Latin word for "bridge" or the 16th-century Italian anatomist and surgeon Costanzo Varolio (Pons Varolii). It is cranial to the medulla oblongata, caudal to the midbrain, and ventral to the cerebellum. In humans and other bipeds this means it is above the medulla, below the midbrain, and anterior to the cerebellum. This white matter includes tracts that conduct signals from the cerebrum down to the cerebellum and medulla, and tracts that carry the sensory signals up into the thalamus. The pons measures about 2.5 cm in length. Most of it appears as a broad anterior bulge rostral to the medulla. Posteriorly, it consists mainly of two pairs of thick stalks called cerebellar peduncles which connect the cerebellum to the pons and midbrain. The pons contains nuclei that relay signals from the forebrain to the cerebellum, along with nuclei that deal primarily with sleep, respiration, swallowing, bladder control, hearing, equilibrium, taste, eye movement, facial expressions, facial sensation, and posture. The pons is implicated in sleep paralysis, and also plays a role in generating dreams.

Clinical features of pontine neurocyticercosis (PNCC) also depends of different locations of the T solium at the pontine level such as:

#### *1. Internuclear ophthalmoplegia*

216 Novel Aspects on Cysticercosis and Neurocysticercosis

4. *Claude's syndrome*

involving the midbrain has been reported. [174, 183]

*5. Superior divisional oculomotor nerve palsy* 

*6. Sudden-onset ptosis [184]* 

*3.1.3. Pontine neurocysticercosis* 

neurocysticercosis infection as one of the etiologic factors [179]

MIDNCC for the differential diagnosis in similar cases.

in sleep paralysis, and also plays a role in generating dreams.

Isolated third cranial nerve palsy is usually due to vascular causes like posterior communicating artery aneurysm and it is rare in NCC and is usually caused by supratentorial or sub-arachnoid lesions with accompanying hydrocephalus or meningitis however patients presenting third cranial nerve palsy caused by neurocysticercosis

Claude's syndrome is a distinctive brainstem syndrome characterized by ipsilateral third cranial nerve palsy with contralateral hemiataxia and is due to an intrinsic or extrinsic lesion in the midbrain. The most common cause of Claude's syndrome is ischemic stroke on the midbrain however one patient presenting Claude's syndrome secondary to MIDNCC is reported. Therefore, if we encounter Claude's syndrome, we should consider

Superior divisional oculomotor nerve palsy caused by intrinsic brain stem disease occurs rarely. The first case of midbrain neurocysticercosis presenting as isolated superior divisional paresis of the oculomotor nerve was communicated by Chotmongkol et al,. in 2006. [177] They reported a 25-year-old woman presented with acute onset of headache and ptosis-upgaze palsy of the right eye. This is an anecdotal case but serve to keep in mind

Sudden onset of ptosis secondary to MIDNCC was identified in 2008 and the first two children were informed to the medical literature by Singhi et al. [184] Recently Naphade et al., [180] reported the case of a 45-year-old woman who presented with acute onset of bilateral symmetrical ptosis with no other neurological deficit. Her imaging revealed a single cystic

The pons is a structure located on the brain stem, named after the Latin word for "bridge" or the 16th-century Italian anatomist and surgeon Costanzo Varolio (Pons Varolii). It is cranial to the medulla oblongata, caudal to the midbrain, and ventral to the cerebellum. In humans and other bipeds this means it is above the medulla, below the midbrain, and anterior to the cerebellum. This white matter includes tracts that conduct signals from the cerebrum down to the cerebellum and medulla, and tracts that carry the sensory signals up into the thalamus. The pons measures about 2.5 cm in length. Most of it appears as a broad anterior bulge rostral to the medulla. Posteriorly, it consists mainly of two pairs of thick stalks called cerebellar peduncles which connect the cerebellum to the pons and midbrain. The pons contains nuclei that relay signals from the forebrain to the cerebellum, along with nuclei that deal primarily with sleep, respiration, swallowing, bladder control, hearing, equilibrium, taste, eye movement, facial expressions, facial sensation, and posture. The pons is implicated

ring-enhancing lesion with perilesional oedema in the midbrain suggestive of NCC.

Internuclear ophthalmoplegia (INO) is a disorder of conjugate lateral gaze in which the affected eye shows impairment of adduction. When an attempt is made to gaze contralaterally (relative to the affected eye), the affected eye adducts minimally, if at all. The contralateral eye abducts, however with nystagmus. Additionally, the divergence of the eyes leads to horizontal diplopia. That is, if the right eye is affected the patient will "see double" when looking to the left, seeing two images side-by-side. Convergence is generally preserved. Clinical presentations of INO can be divided into: unilateral INO, bilateral INO, and one-and-a-half syndrome in a similar descending frequency. [185] Perhaps the more difficult clinical diagnosis to perform is the One-and-a-half syndrome (OAHS). It is a neurological disorder of the extra ocular movements characterized by conjugate horizontal gaze palsy in one direction and internuclear ophthalmoplegia (INO) in the other. The commonest cause of the syndrome is a vascular lesion on the basilar territory followed by demyelinating lesions of the brainstem usually affecting the paramedian pontine reticular formation unilaterally or due to lesions on the abducens nucleus on one side, with interruption of internuclear fibbers of the ipsilateral medial longitudinal fasciculus after it has crossed the midline from its side of origin in the contra lateral abducens nucleus causing failure of adduction of the ipsilateral eye. [186]

In 2004, we proposed a new classification for OAHS based on our finding in patients with NCC and cerebrovascular diseases and we described the clinical features of One-and-a-half "plus" syndrome. We found patients with other combinations that can be called as: Fiveand-a-half syndrome (1 ½ + 4-throclear nerve), Seven-and-a-half syndrome (1 ½ + 6 abducens) or Eleven-and-a-half syndrome (1 ½ + 7-facial nerve + 3-oculomotor nerve) due to racemose NCC, and midbrain lesions related to NCC. However we honestly considered at that time and even today that a generation of novel arithmetic combinations will bring more confusion than clarity to this problem and will increase unnecessarily the long list of different names of neuro-ophthalmological disorders, therefore we concluded that adding the term "plus" was good enough to distinguish these conditions from the typical OAHS. [186] Five years later Ranjith et al [178] studied an 18-year-old male presented with a history of sudden-onset diplopia of 1-week duration and blurring of vision. He had no history of headache, fever, seizures, walking difficulty, previous head injury, diabetes or hypertension. There was no history of a similar episode in the past. Diplopia was found to increase while looking to the left. Findings from general examination were normal. The left eye was deviated outward, and the right eye was central in primary gaze. There was a weakness of adduction in the left eye and paralysis of all conjugate horizontal eye movements in the right eye. The left eye also showed nystagmus while the patient attempted left lateral gaze. Vertical eye movements were normal, and no ocular bobbing was observed. There was no long tract sign, and the cerebellar system was normal. They confirmed OAHS secondary to PNCC.

#### *2. Simulating cystic tumor*

Characterized by focal neurological signs and clinical manifestation of intracranial hypertension including abducens palsy among other features. Cases have been reported [187]

Uncommon Clinical Manifestations of Cysticercosis 219

*3.1.5. Insula lobe neurocysticercosis* 

involved in psychopathology.

neural source of feeling experiences. [192]

rhythm or paroxysmal activity.

of the QT Segment.

disorders, cardiovascular problems, and epileptic seizures.

In each hemisphere of the mammalian brain the insular cortex (often called insula, insulary cortex, *Island of Reil* or the insular lobe) is a portion of the cerebral cortex folded deep within the lateral sulcus between the temporal lobe and the frontal lobe. The insula is believed to be involved in consciousness and play a role in diverse functions usually linked to emotion or the regulation of the body's homeostasis. These functions include perception, motor control, self-awareness, cognitive functioning, and interpersonal experience. In relation to these it is

The insular cortex is divided into two parts: the larger anterior insula and the smaller posterior insula in which more than a dozen field areas have been identified. The cortical area overlying the insula towards the lateral surface of the brain is the operculum (meaning "lid"). The Operculum, derived from Latin, meaning "little lid", refers to the cerebral cortex that covers the insula and is formed from parts of the enclosing frontal, temporal and parietal lobes. Operculum-insular cortex plays a role in the processing of nociceptive input. It has been convincingly established, over the past decade, that the human insular cortices are involved in processing both body feelings (such as pain) and feelings of emotion. Recently, however, an interpretation of this finding has emerged suggesting that the insular cortices are the necessary and sufficient platform for human feelings, in effect, the sole

Between the years 1999 and 2005 we studied a series of patients with NCC located in the lobe of the insula (INCC) confirmed by imagenology. All came from the epilepsy/NCC clinic and the neurology clinic of the Mthatha Hospital Complex. This study was designed for the screening of symptoms and clinical signs such as: dysphagia, dysarthria, attention deficit

All the patients underwent a CT scan of the brain and/or MRI, an ELISA test for the determination of antigens and antibodies of CC in serum and an electrocardiogram. For all patients a digital interictal EEG was performed based on, the 10-20 system and each study lasted about 20 minutes looking for focal or generalized alterations of the background

It was considered that the patient had difficulty swallowing liquids when they were choking or coughing after swallowing five cubic centimeters of water. In the first six month period of this study we explored the ability of the patients to differentiate between expressions of fear or disgust in photos or drawings that we show them for that purpose. Unfortunately many patients did not have the capacity to differentiate an expression of joy, fear or sadness; however we erroneously believe that this test did not work and it was eliminated from this study. In this research, special attention was given in the exclusion of patients with a history of other neurological diseases in addition to epilepsy, metabolic diseases of any type of ischemic heart disease or diseases causing difficulty in swallowing, the syndromes of Jervell-Lang -Nielsen, Romano-Ward and other causes of prolongation

#### *3. Hemifacial spasm*

Ipsilateral hemifacial spasm and associated bladder incontinence and confusional state secondary to PNCC has been reported [173]

#### *4. Isolated facial myokymia*

In patients presenting a history of continuous rippling and quivering movements of their hemiface suggestive of myokymia, a diagnosis of PNCC should be taking into account. The first well documented patient was reported in the medical literature by Bhatia et al in 2008 [188]

## *3.1.4. Medulla oblongata neurocysticercosis*

The medulla oblongata is the lower half of the brainstem. In discussions of neurology and similar contexts where no ambiguity will result, it is often referred to as simply the medulla. The medulla contains the cardiac, respiratory, vomiting and vasomotor centers and deals with autonomic, involuntary functions, such as breathing, heart rate and blood pressure.

The medulla neurocysticercosis (MENCC) is an extremely rare presentation of NCC and the main affected area is the lateral aspect of the medulla leading to: Lateral medullary syndrome (also called Wallenberg syndrome and posterior inferior cerebellar artery syndrome) which is characterized by sensory deficits affecting the trunk and extremities on the opposite side of the MEDNCC and sensory deficits affecting the face and cranial nerves on the same side of the lesion. Specifically, there is a loss of pain and temperature sensation on the *contralateral* (opposite) side of the body due to damage of the spinothalamic tract and *ipsilateral* side of the face including an absence of corneal reflex due to lesion of the spinal trigeminal nucleus. Clinical symptoms include ataxia secondary to damage of the inferior cerebellar peduncle, facial pain, vertigo and nystagmus due to lesion of Deiters' nucleus and the inferior vestibular nucleus, and possibly palatal myoclonus may be observed due to disruption of the central tegmental tract. Damage to the hypothalamospinal fibers disrupts sympathetic nervous system relay and gives symptoms analogous to Horner syndrome. If patients also start vomiting then the extension of the damage toward the area postrema (dorsal vagal complex) should be considered. Remember that the area postrema is located outside of the blood–brain barrier and connects to the nucleus of the solitary tract and other autonomic control centers in the brainstem, which may serve as the beginning of the pathway triggering vomiting in response to various emetic inputs. The MEDNCC causing Wallenber syndrome has been communicated by Garg in 1999. [189]

Other uncommon presentations of NCC may include: Bilateral trigeminal neuralgia secondary to racemous cysticercus of the cerebellopontine angle, [190] and bradycardia due to right cerebellar peduncle neurocysticercosis, [191]

## *3.1.5. Insula lobe neurocysticercosis*

218 Novel Aspects on Cysticercosis and Neurocysticercosis

secondary to PNCC has been reported [173]

*3.1.4. Medulla oblongata neurocysticercosis* 

Characterized by focal neurological signs and clinical manifestation of intracranial hypertension including abducens palsy among other features. Cases have been reported

Ipsilateral hemifacial spasm and associated bladder incontinence and confusional state

In patients presenting a history of continuous rippling and quivering movements of their hemiface suggestive of myokymia, a diagnosis of PNCC should be taking into account. The first well documented patient was reported in the medical literature by Bhatia et al in 2008

The medulla oblongata is the lower half of the brainstem. In discussions of neurology and similar contexts where no ambiguity will result, it is often referred to as simply the medulla. The medulla contains the cardiac, respiratory, vomiting and vasomotor centers and deals with autonomic, involuntary functions, such as breathing, heart rate and blood pressure.

The medulla neurocysticercosis (MENCC) is an extremely rare presentation of NCC and the main affected area is the lateral aspect of the medulla leading to: Lateral medullary syndrome (also called Wallenberg syndrome and posterior inferior cerebellar artery syndrome) which is characterized by sensory deficits affecting the trunk and extremities on the opposite side of the MEDNCC and sensory deficits affecting the face and cranial nerves on the same side of the lesion. Specifically, there is a loss of pain and temperature sensation on the *contralateral* (opposite) side of the body due to damage of the spinothalamic tract and *ipsilateral* side of the face including an absence of corneal reflex due to lesion of the spinal trigeminal nucleus. Clinical symptoms include ataxia secondary to damage of the inferior cerebellar peduncle, facial pain, vertigo and nystagmus due to lesion of Deiters' nucleus and the inferior vestibular nucleus, and possibly palatal myoclonus may be observed due to disruption of the central tegmental tract. Damage to the hypothalamospinal fibers disrupts sympathetic nervous system relay and gives symptoms analogous to Horner syndrome. If patients also start vomiting then the extension of the damage toward the area postrema (dorsal vagal complex) should be considered. Remember that the area postrema is located outside of the blood–brain barrier and connects to the nucleus of the solitary tract and other autonomic control centers in the brainstem, which may serve as the beginning of the pathway triggering vomiting in response to various emetic inputs. The MEDNCC causing

Other uncommon presentations of NCC may include: Bilateral trigeminal neuralgia secondary to racemous cysticercus of the cerebellopontine angle, [190] and bradycardia due

Wallenber syndrome has been communicated by Garg in 1999. [189]

to right cerebellar peduncle neurocysticercosis, [191]

*2. Simulating cystic tumor* 

*4. Isolated facial myokymia* 

*3. Hemifacial spasm* 

[187]

[188]

In each hemisphere of the mammalian brain the insular cortex (often called insula, insulary cortex, *Island of Reil* or the insular lobe) is a portion of the cerebral cortex folded deep within the lateral sulcus between the temporal lobe and the frontal lobe. The insula is believed to be involved in consciousness and play a role in diverse functions usually linked to emotion or the regulation of the body's homeostasis. These functions include perception, motor control, self-awareness, cognitive functioning, and interpersonal experience. In relation to these it is involved in psychopathology.

The insular cortex is divided into two parts: the larger anterior insula and the smaller posterior insula in which more than a dozen field areas have been identified. The cortical area overlying the insula towards the lateral surface of the brain is the operculum (meaning "lid"). The Operculum, derived from Latin, meaning "little lid", refers to the cerebral cortex that covers the insula and is formed from parts of the enclosing frontal, temporal and parietal lobes. Operculum-insular cortex plays a role in the processing of nociceptive input. It has been convincingly established, over the past decade, that the human insular cortices are involved in processing both body feelings (such as pain) and feelings of emotion. Recently, however, an interpretation of this finding has emerged suggesting that the insular cortices are the necessary and sufficient platform for human feelings, in effect, the sole neural source of feeling experiences. [192]

Between the years 1999 and 2005 we studied a series of patients with NCC located in the lobe of the insula (INCC) confirmed by imagenology. All came from the epilepsy/NCC clinic and the neurology clinic of the Mthatha Hospital Complex. This study was designed for the screening of symptoms and clinical signs such as: dysphagia, dysarthria, attention deficit disorders, cardiovascular problems, and epileptic seizures.

All the patients underwent a CT scan of the brain and/or MRI, an ELISA test for the determination of antigens and antibodies of CC in serum and an electrocardiogram. For all patients a digital interictal EEG was performed based on, the 10-20 system and each study lasted about 20 minutes looking for focal or generalized alterations of the background rhythm or paroxysmal activity.

It was considered that the patient had difficulty swallowing liquids when they were choking or coughing after swallowing five cubic centimeters of water. In the first six month period of this study we explored the ability of the patients to differentiate between expressions of fear or disgust in photos or drawings that we show them for that purpose. Unfortunately many patients did not have the capacity to differentiate an expression of joy, fear or sadness; however we erroneously believe that this test did not work and it was eliminated from this study. In this research, special attention was given in the exclusion of patients with a history of other neurological diseases in addition to epilepsy, metabolic diseases of any type of ischemic heart disease or diseases causing difficulty in swallowing, the syndromes of Jervell-Lang -Nielsen, Romano-Ward and other causes of prolongation of the QT Segment.

Two patients died and in the postmortem studies *T solium* cystic lesions were confirmed in the insular cortex. (See figure 7) The problems most frequently encountered were functional dysphagia, the visual and somato-sensory inattention, ECG alterations such as ST-segment depression greater than 0.1 millivolts by more than 80 milliseconds and elongation of the QT Segment (greater than 0.44 seconds).

Uncommon Clinical Manifestations of Cysticercosis 221

**Figure 7.** Right insular lobe with T solium cysticercosis in different stages. Unfortunately some cyst was

In brief: patient with INCC may submit a functional dysphagia with visual and somatosensory inattention, signs of neurogenic heart and, ECG alterations such as ST-segment depression greater than 0.1 millivolts for longer than 80 milliseconds and elongation of the QT segment (greater than 0.44 seconds) and that special attention should be given to this mode of presentation by the serious complications that can be involved, including death.

The clinical features of epileptic seizures secondary to NCCI are characterized by paroxysmal feeling of laryngeal constriction, the functional dysphagia, and peribucal paresthesia. We must insist on the patient's interview about these symptoms if there is evidence of INCC in imagenology. Frequently, neither the patient nor the family or friends give importance to these symptoms and the situation is even worse when the patient is assisted by other doctor, another diagnosis is made and do not to pay too much attention is suggested especially if the seizures are sporadic and apparently banal. The diagnosis is based on the clinical confirmation of these manifestations as the conventional EEG with

Apart from subarachnoid NCC, *T solium* can also affect directly or indirectly midline anatomical structures such as: optic quiasm, hypothalamus, pituitary gland, and sella

The optic chiasm (OCh) is the part of the brain where the optic nerves partially cross. Several important structures are located adjacent to the OCh. The supraclinoid branches of the internal carotid artery flank the OCh. The cavernous sinuses are lateral and inferior to

damaged during disserting procedures.

surface electrode is normal. [7,15]

*3.1.7. Optic quiasm NCC* 

turcica.

*3.1.6. Middle fossa neurocysticercosis* 

In our series, disturbances of the language due to the NCC are very uncommon excepting some patients presenting ischemic stroke due to cysticercotic /HIV/AIDS vasculitis. It is even rare in patients presenting T solium cystic lesions in the motor or sensory language cortical regions. In patients with lesions of the insula with involvement of the superior temporal gyrus and the inferior parietal region, conductive afasias have been reported, [193] but we have not been able to identify any single patient with isolated disorders of the speech or spoken languages without stroke.

The insula of the right cerebral hemisphere may play an important role in the autonomic control of the heart and the electrocardiographic abnormalities of the heart are often transient, but represent a diagnostic problem sometimes indistinguishable from a myocardial infarction. Some alterations of the T waves may be suggestive of cardiac pathology, but are not specific so it is important to consider this situation to avoid inappropriate treatment.

In the year 2004, Colivichi et al. [194] designed a study to establish the relationship between the vascular ischemic brain damage at the level of the right insular lobe and cardiac arrhythmias and they concluded that this area of the brain exerts a controlling function of the visceral autonomic activity on the heart with prognostic implications. However, the cardiac signs found in our series such as: asystole and bradycardia may also be suggestive of cardiac dysfunction secondary to left insula as has previously been reported in patients with temporal lobe epilepsy [195-198] and confirmed in 2009 by Koseoglu et al.[199] The epileptic activity spreads through the limbic system with the involvement of the amygdala, hypothalamus, and thalamus. These, in turn stimulate the autonomic system at medullar level including the nucleus tractus solitarius and the nucleus ambiguous which generate sympathetic and parasympathetic discharges.[200]

In our first study the ictal manifestations found were: laryngeal constriction, oppression of the chest, paresthesia of peri-oral region, the feeling of suspension or floating and some taste disorders. The characteristics of the epileptic seizures in these patients could not be defined convincingly at that time, therefore few years later, the realization of a methodological design addressed primarily to achieve that goal was made. However as noted earlier in this study could not arrive at confident conclusions. Later we concluded that the INCC is characterized by a functional dysphagia, ECG alterations such as: ST-segment depression and especially a prolongation of the QT segment, atypical epileptic seizures, a slight spastic dysarthria, sensory lapses and cardiovascular disorders. [7, 15]

Finally, we would like to highlight that even when the clinical features of the lesions in the insula are known mainly from neurophysiological studies and by electrical stimulation of the insular cortex, our clinical findings were not previously reported in the international medical literature although some of them were confirmed later by other authors. [201-203]

**Figure 7.** Right insular lobe with T solium cysticercosis in different stages. Unfortunately some cyst was damaged during disserting procedures.

In brief: patient with INCC may submit a functional dysphagia with visual and somatosensory inattention, signs of neurogenic heart and, ECG alterations such as ST-segment depression greater than 0.1 millivolts for longer than 80 milliseconds and elongation of the QT segment (greater than 0.44 seconds) and that special attention should be given to this mode of presentation by the serious complications that can be involved, including death.

The clinical features of epileptic seizures secondary to NCCI are characterized by paroxysmal feeling of laryngeal constriction, the functional dysphagia, and peribucal paresthesia. We must insist on the patient's interview about these symptoms if there is evidence of INCC in imagenology. Frequently, neither the patient nor the family or friends give importance to these symptoms and the situation is even worse when the patient is assisted by other doctor, another diagnosis is made and do not to pay too much attention is suggested especially if the seizures are sporadic and apparently banal. The diagnosis is based on the clinical confirmation of these manifestations as the conventional EEG with surface electrode is normal. [7,15]

## *3.1.6. Middle fossa neurocysticercosis*

Apart from subarachnoid NCC, *T solium* can also affect directly or indirectly midline anatomical structures such as: optic quiasm, hypothalamus, pituitary gland, and sella turcica.

## *3.1.7. Optic quiasm NCC*

220 Novel Aspects on Cysticercosis and Neurocysticercosis

Segment (greater than 0.44 seconds).

speech or spoken languages without stroke.

sympathetic and parasympathetic discharges.[200]

dysarthria, sensory lapses and cardiovascular disorders. [7, 15]

Two patients died and in the postmortem studies *T solium* cystic lesions were confirmed in the insular cortex. (See figure 7) The problems most frequently encountered were functional dysphagia, the visual and somato-sensory inattention, ECG alterations such as ST-segment depression greater than 0.1 millivolts by more than 80 milliseconds and elongation of the QT

In our series, disturbances of the language due to the NCC are very uncommon excepting some patients presenting ischemic stroke due to cysticercotic /HIV/AIDS vasculitis. It is even rare in patients presenting T solium cystic lesions in the motor or sensory language cortical regions. In patients with lesions of the insula with involvement of the superior temporal gyrus and the inferior parietal region, conductive afasias have been reported, [193] but we have not been able to identify any single patient with isolated disorders of the

The insula of the right cerebral hemisphere may play an important role in the autonomic control of the heart and the electrocardiographic abnormalities of the heart are often transient, but represent a diagnostic problem sometimes indistinguishable from a myocardial infarction. Some alterations of the T waves may be suggestive of cardiac pathology, but are not specific so

In the year 2004, Colivichi et al. [194] designed a study to establish the relationship between the vascular ischemic brain damage at the level of the right insular lobe and cardiac arrhythmias and they concluded that this area of the brain exerts a controlling function of the visceral autonomic activity on the heart with prognostic implications. However, the cardiac signs found in our series such as: asystole and bradycardia may also be suggestive of cardiac dysfunction secondary to left insula as has previously been reported in patients with temporal lobe epilepsy [195-198] and confirmed in 2009 by Koseoglu et al.[199] The epileptic activity spreads through the limbic system with the involvement of the amygdala, hypothalamus, and thalamus. These, in turn stimulate the autonomic system at medullar level including the nucleus tractus solitarius and the nucleus ambiguous which generate

In our first study the ictal manifestations found were: laryngeal constriction, oppression of the chest, paresthesia of peri-oral region, the feeling of suspension or floating and some taste disorders. The characteristics of the epileptic seizures in these patients could not be defined convincingly at that time, therefore few years later, the realization of a methodological design addressed primarily to achieve that goal was made. However as noted earlier in this study could not arrive at confident conclusions. Later we concluded that the INCC is characterized by a functional dysphagia, ECG alterations such as: ST-segment depression and especially a prolongation of the QT segment, atypical epileptic seizures, a slight spastic

Finally, we would like to highlight that even when the clinical features of the lesions in the insula are known mainly from neurophysiological studies and by electrical stimulation of the insular cortex, our clinical findings were not previously reported in the international medical literature although some of them were confirmed later by other authors. [201-203]

it is important to consider this situation to avoid inappropriate treatment.

The optic chiasm (OCh) is the part of the brain where the optic nerves partially cross. Several important structures are located adjacent to the OCh. The supraclinoid branches of the internal carotid artery flank the OCh. The cavernous sinuses are lateral and inferior to

the OCh. The frontal lobe of the brain lies above. The pituitary gland sits below in the sella turcica. The sella turcica is bounded in front by the tuberculum sellae and behind of the dorsum sellae. Behind the chiasm lies the floor of the third ventricle. Chiasmal syndrome is the medical term used to describe the various signs and symptoms that are associated with lesions of the OCh, manifesting as various types of blindness or impairment in the sufferer's visual field according on the location of the lesions along the optic nerve.

Uncommon Clinical Manifestations of Cysticercosis 223

tumor, but this probably represented enlargement and an erosion of the sella secondary to increased intra cranial pressure. [210] Therefore the fourth well documented patient with sella turcica neurocysticercosis (STNCC) was studied by Prosser et al in 1978. They reported *a* 42-year-old woman from El Salvador presented with arthralgias and headache. She was found to have an enlarged sella with an intrasellar mass. Transsphenoidal exploration of the sella revealed a cysticercus, the cyst was successfully removed and normal pituitary function was retained. These authors recommend that cysticercosis be considered in the differential diagnosis of any patient from an endemic area who presents with a mass lesion anywhere in the CNS, including intrasellar masses. [210] Few years later other authors reported cases presenting disturbances in both visual acuity and visual fields and other

As a general rule, for patients from endemic areas presenting chiasmatic syndrome and hormonal disturbances to rule out STNCC is mandatory. Other authors have similar

Although NCC damages sellar structures with direct compression by large cysts, extension through the basal cisterns and third ventricle with focal arachnoiditis can result as an inflammatory response. Taking into consideration that the sellar region and the basal cisterns contain many vascular and CNS structures that are separated by only a few millimeters increasing the possibility that different pathologic entities could affect them simultaneously then analysis of imaging findings and knowledge of the varied clinical presentations of NCC are important to detect cases of sellar involvement in patients with unexplained visual loss or atypical cystic lesions in the sella, especially in endemic countries. [213] As in other locations in the brain, the parasite can affect the sellar structures through diverse mechanisms, ranging from direct or indirect invasion to a severe local inflammatory response [217-218] Although direct invasion of the sella turcica by the NCC vesicle affecting the hypophysis is the most commonly described form in the literature [206-212] and is often confused with the most common tumors of the area (eg, cystic adenoma and craniopharyngioma). The absence of clinical suspicion of STNCC usually results in surgical treatment of the lesions and

A 7-year-old child presented with polyuria and polydipsia for 1 year due to a small cystic lesion in the proximal pituitary stalk was also reported in the medical literature. [214] And three patients presenting hypopituitarism secondary to the NCC have been reported as well.

The hypothalamus is a portion of the brain that contains a number of small nuclei with a variety of functions. One of the most important functions of the hypothalamus is to link the nervous system to the endocrine system via the pituitary gland (hypophysis). The hypothalamus is located below the thalamus, just above the brain stem. In the terminology of neuroanatomy, it forms the ventral part of the diencephalon. All vertebrate brains contain

postoperative diagnosis of the NCC with variable clinical results. [213]

a hypothalamus. In humans, it is roughly the size of an almond.

considerations were made. [211,212]

*3.1.9. Hypothalamic neurocysticercosis* 

opinions. [210, 213-216]

[216]

Optic NCC has been well described in the past and all interested readers can find more information in the chapter entitled: **"**Orbital cysticercosis. A challenger for neurologists, ophthalmologists, neuro-ophthalmologists, and general practtioners" in this book. However optic chiasm NCC (OChNCC) caused by intra-chiasmatic cysticercosis has not been reported up to date. It's known that the clinical features of the OCh are caused by inflammatory processes secondary to cysticercotic vasculitis [204] or by released pro-inflammatory elements when close by cysticerci are dying. Neurocysticercosis may cause bitemporal hemianopsia due to chiasmatic compression secondary to obstructive hydrocephalus. [205]

## *3.1.8. Intra-Sellar turcica neurocysticercosis*

The sella turcica is a midline depression in the sphenoid bone which contains the anterior and posterior lobes of the pituitary gland and the distal portion of the pituitary stalk. It is covered by a dural reflection, the diaphragm sellae. Above this lies the suprasellar cistern, which contains the supraclinoid carotid arteries and the optic tract, OCh, and nerves, and through which travels the pituitary stalk, Lateral to the sella turcica are the cavernous sinuses containing the carotid arteries, cranial nerves III, IV, and VI, and the first two divisions of the fifth cranial nerve. Anteriorly, the sella turcica is bound by the tuberculum sellae and anterolaterally by the anterior clinoid processes, Anteroinferiorly, the foramen rotundum conducts the maxillary branch of cranial nerve V. Posteriorly, there are the smaller posterior clinoid processes, the dorsum sellae, and the interpeduncular cistern containing cranial nerves III and IV. Inferiorly, the sella turcica has a thin floor of cortical bone, below which lies the air-containing sphenoid sinus. The sinus is extremely variable in size. Adjacent to the posteroinferior aspect of the cavernous sinus lies a Meckel's cave, containing the gasserian ganglion. Immediately below and lateral to the gasserian ganglion, the third branch of the fifth cranial nerve exits through the foramen ovale.

In 1915, Kufs described a woman in whom destruction of the pituitary by one of multiple basilar cysts was found at autopsy. The diagnosis was not suspected antemortem, and the patient had findings that may have been related to pituitary insufficiency. [206] In 1955, panhypopituitarism was diagnosed by Dickinson [207] in a British soldier who had served in India and developed epilepsy with X-ray evidence of cysticerci in the legs and a distorted pituitary phase CT scan of the brain was not available at that time. The case reported by Briceflo et al. (1961) was one of 97 cases of cysticercosis found among 2 ,767 routine autopsies over a 6-year period in a large Mexican hospital. Clinical data were not presented, and the authors did not record whether the patient had other lesions elsewhere in the CNS. [209] A fourth report describes a patient with cysticercosis masquerading as a pituitary tumor, but this probably represented enlargement and an erosion of the sella secondary to increased intra cranial pressure. [210] Therefore the fourth well documented patient with sella turcica neurocysticercosis (STNCC) was studied by Prosser et al in 1978. They reported *a* 42-year-old woman from El Salvador presented with arthralgias and headache. She was found to have an enlarged sella with an intrasellar mass. Transsphenoidal exploration of the sella revealed a cysticercus, the cyst was successfully removed and normal pituitary function was retained. These authors recommend that cysticercosis be considered in the differential diagnosis of any patient from an endemic area who presents with a mass lesion anywhere in the CNS, including intrasellar masses. [210] Few years later other authors reported cases presenting disturbances in both visual acuity and visual fields and other considerations were made. [211,212]

As a general rule, for patients from endemic areas presenting chiasmatic syndrome and hormonal disturbances to rule out STNCC is mandatory. Other authors have similar opinions. [210, 213-216]

Although NCC damages sellar structures with direct compression by large cysts, extension through the basal cisterns and third ventricle with focal arachnoiditis can result as an inflammatory response. Taking into consideration that the sellar region and the basal cisterns contain many vascular and CNS structures that are separated by only a few millimeters increasing the possibility that different pathologic entities could affect them simultaneously then analysis of imaging findings and knowledge of the varied clinical presentations of NCC are important to detect cases of sellar involvement in patients with unexplained visual loss or atypical cystic lesions in the sella, especially in endemic countries. [213] As in other locations in the brain, the parasite can affect the sellar structures through diverse mechanisms, ranging from direct or indirect invasion to a severe local inflammatory response [217-218] Although direct invasion of the sella turcica by the NCC vesicle affecting the hypophysis is the most commonly described form in the literature [206-212] and is often confused with the most common tumors of the area (eg, cystic adenoma and craniopharyngioma). The absence of clinical suspicion of STNCC usually results in surgical treatment of the lesions and postoperative diagnosis of the NCC with variable clinical results. [213]

A 7-year-old child presented with polyuria and polydipsia for 1 year due to a small cystic lesion in the proximal pituitary stalk was also reported in the medical literature. [214] And three patients presenting hypopituitarism secondary to the NCC have been reported as well. [216]

## *3.1.9. Hypothalamic neurocysticercosis*

222 Novel Aspects on Cysticercosis and Neurocysticercosis

*3.1.8. Intra-Sellar turcica neurocysticercosis* 

the OCh. The frontal lobe of the brain lies above. The pituitary gland sits below in the sella turcica. The sella turcica is bounded in front by the tuberculum sellae and behind of the dorsum sellae. Behind the chiasm lies the floor of the third ventricle. Chiasmal syndrome is the medical term used to describe the various signs and symptoms that are associated with lesions of the OCh, manifesting as various types of blindness or impairment in the sufferer's

Optic NCC has been well described in the past and all interested readers can find more information in the chapter entitled: **"**Orbital cysticercosis. A challenger for neurologists, ophthalmologists, neuro-ophthalmologists, and general practtioners" in this book. However optic chiasm NCC (OChNCC) caused by intra-chiasmatic cysticercosis has not been reported up to date. It's known that the clinical features of the OCh are caused by inflammatory processes secondary to cysticercotic vasculitis [204] or by released pro-inflammatory elements when close by cysticerci are dying. Neurocysticercosis may cause bitemporal hemianopsia due

The sella turcica is a midline depression in the sphenoid bone which contains the anterior and posterior lobes of the pituitary gland and the distal portion of the pituitary stalk. It is covered by a dural reflection, the diaphragm sellae. Above this lies the suprasellar cistern, which contains the supraclinoid carotid arteries and the optic tract, OCh, and nerves, and through which travels the pituitary stalk, Lateral to the sella turcica are the cavernous sinuses containing the carotid arteries, cranial nerves III, IV, and VI, and the first two divisions of the fifth cranial nerve. Anteriorly, the sella turcica is bound by the tuberculum sellae and anterolaterally by the anterior clinoid processes, Anteroinferiorly, the foramen rotundum conducts the maxillary branch of cranial nerve V. Posteriorly, there are the smaller posterior clinoid processes, the dorsum sellae, and the interpeduncular cistern containing cranial nerves III and IV. Inferiorly, the sella turcica has a thin floor of cortical bone, below which lies the air-containing sphenoid sinus. The sinus is extremely variable in size. Adjacent to the posteroinferior aspect of the cavernous sinus lies a Meckel's cave, containing the gasserian ganglion. Immediately below and lateral to the gasserian ganglion,

In 1915, Kufs described a woman in whom destruction of the pituitary by one of multiple basilar cysts was found at autopsy. The diagnosis was not suspected antemortem, and the patient had findings that may have been related to pituitary insufficiency. [206] In 1955, panhypopituitarism was diagnosed by Dickinson [207] in a British soldier who had served in India and developed epilepsy with X-ray evidence of cysticerci in the legs and a distorted pituitary phase CT scan of the brain was not available at that time. The case reported by Briceflo et al. (1961) was one of 97 cases of cysticercosis found among 2 ,767 routine autopsies over a 6-year period in a large Mexican hospital. Clinical data were not presented, and the authors did not record whether the patient had other lesions elsewhere in the CNS. [209] A fourth report describes a patient with cysticercosis masquerading as a pituitary

visual field according on the location of the lesions along the optic nerve.

to chiasmatic compression secondary to obstructive hydrocephalus. [205]

the third branch of the fifth cranial nerve exits through the foramen ovale.

The hypothalamus is a portion of the brain that contains a number of small nuclei with a variety of functions. One of the most important functions of the hypothalamus is to link the nervous system to the endocrine system via the pituitary gland (hypophysis). The hypothalamus is located below the thalamus, just above the brain stem. In the terminology of neuroanatomy, it forms the ventral part of the diencephalon. All vertebrate brains contain a hypothalamus. In humans, it is roughly the size of an almond.

The hypothalamus is responsible for certain metabolic processes and other activities of the autonomic nervous system. It synthesizes and secretes certain neurohormones, often called hypothalamic-releasing hormones, and these in turn stimulate or inhibit the secretion of pituitary hormones. The hypothalamus controls body temperature, hunger, thirst, fatigue, sleep, and circadian cycles. In mammals, the axons of magnocellular neurosecretory cells of the paraventricular nucleus and the supraoptic nucleus, which contain oxytocin and vasopressin (also called antidiuretic hormone), comprise the posterior pituitary. Parvocellular neurons of the paraventricular nucleus contain neurons that release corticotropin-releasing hormone and other hormones into the hypophyseal portal system where these hormones diffuse to the anterior pituitary.

Uncommon Clinical Manifestations of Cysticercosis 225

neuroimaging abnormality seen as: less than 20 mm ring-enhancing lesion in patients with new-onset epilepsy. [223] However, in our region SCG is not the most frequent presentation of NCC and compared with the prevalence of multiple active and the calcified intraparenchymal NCC which is highest, SCG is relatively uncommon probable related to

Only one patient presenting lateral sinus thrombosis secondary to NCC has been reported in

Taenia solium cysticercosis can invade almost all extracraneal anatomical structures in the

When a temporalis muscle is involved, clinical presentation can be a temporal headache. Two woman who complained of subacute onset of left temporal pain and another complaining headache due to cysticercosis of the temporalis muscle have been reported.

Ocular cysts are mostly vitreous or may be found in subretinal locations. Cysts can impair vision by floating in the eye and can lead to blindness by causing retinal detachment.

We dedicated a complete article for this issue and interested readers can find detailed information in the chapter entitled: **"**Orbital Cysticercosis. A challenger for neurologists,

We found facial cysticercosis in a patient presenting disseminate subcutaneous and muscular cysticercosis with cardiac involvement and NCC. [10] A cystic lesion on the right cheek and in the maxillofacial region have been also reported. [229, 230] A photo of patient showing a nodular lesion in the left hemiface at the zigomatic arch level that was removed by surgical procedures and histological examination confirmed T solium cysticercosis can

As other before-cited locations of cysticercosis this one, is also extremely uncommon. Patients presenting lesion in the lips, [231, 232] oral cavity, [233] oral mucosa, [232] and in

head excepting skin, nasal cavity, paranasal sinus, bone tissue and the periostium.

Visualization of cysts via fundoscopy may be diagnostic of the disease.

ophthalmologists, neurophthalmologists, and general practitioners"

unknown factors.

[227, 228]

*3.1.11. Sinus thrombosis* 

the medical literature up to date. [226]

**4. Extracraneal cysticercosis** 

**4.1. Ocular cysticercosis** 

**4.2. Facial cysticercosis** 

be seen in a previous chapter.

*4.3.1. Oral cysticercosis* 

**4.3. Buco-lingua-pharyngeal cysticercosis** 

The hypothalamus is thus richly connected to many parts of the central nervous system, including the brainstem reticular formation and autonomic zones, the limbic forebrain (particularly the amygdala, septum, diagonal band of Broca, and the olfactory bulbs, and the cerebral cortex). The hypothalamic-pituitary-adrenal axis (HPA or HTPA axis), also known as the limbic-hypothalamic-pituitary-adrenal axis (LHPA axis) and, occasionally, as the hypothalamus-pituitary-adrenal-gonadotropic axis, is a complex set of direct influences and feedback interactions among the hypothalamus, the pituitary gland (a pea-shaped structure located below the hypothalamus), and the adrenal glands.

Hypothalamic neurocysticercosis (HTNCC) is another extremelly uncommon presentation of NCC and the most common clinical features of best documented patient are characterized by: obesity, galactorrhea and central diabetes insipidus (polyuria without solute diuresis), and panhypopituitarism due to an associated pituitary gland involvement can also be present.[207,217, 219-222, 224] It has been established that intracranial hypertension "per se" does not generate changes in the hypothalamopituitary axis function. [219]

At a Uberaba Teaching Hospital in Brazil, a total of 1.596 autopsies performed between 1974 and 1997 were studied. The study found a diagnosis of cysticercosis in 53 autopsies (3.3%) and in two obese patients with NCC the lesions were located in the ventromedial nucleus of the hypothalamus. [220]

Other authors also divided the unusual manifestations of neurocysticercosis into: intraventricular, subarachnoid, spinal, orbital, intraparenchymatous, and reactivation of previously calcified lesions and they reported frequencies of 15.69% of intraventricular presentations, subarachnoid: 11.63%; spinal: 3.49%; orbital: 0.58% and reactivated lesion: 0.58% and the frequencies for association of intraventricular and intraparenchymatous: 6.98%; association of subarachnoid and intraparenchymatous: 3.49; and association of subarachnoid and intraventricular: 2.32%. [225] but they did not include other presentations already mentioned in this chapter.

#### *3.1.10. Solitary cysticercus granuloma*

The solitary cysticercus granuloma (SCG) is considered the most frequent type of NCC in India and many other Southeast Asian countries and these lesions are the most frequent neuroimaging abnormality seen as: less than 20 mm ring-enhancing lesion in patients with new-onset epilepsy. [223] However, in our region SCG is not the most frequent presentation of NCC and compared with the prevalence of multiple active and the calcified intraparenchymal NCC which is highest, SCG is relatively uncommon probable related to unknown factors.

## *3.1.11. Sinus thrombosis*

224 Novel Aspects on Cysticercosis and Neurocysticercosis

where these hormones diffuse to the anterior pituitary.

located below the hypothalamus), and the adrenal glands.

the hypothalamus. [220]

already mentioned in this chapter.

*3.1.10. Solitary cysticercus granuloma* 

The hypothalamus is responsible for certain metabolic processes and other activities of the autonomic nervous system. It synthesizes and secretes certain neurohormones, often called hypothalamic-releasing hormones, and these in turn stimulate or inhibit the secretion of pituitary hormones. The hypothalamus controls body temperature, hunger, thirst, fatigue, sleep, and circadian cycles. In mammals, the axons of magnocellular neurosecretory cells of the paraventricular nucleus and the supraoptic nucleus, which contain oxytocin and vasopressin (also called antidiuretic hormone), comprise the posterior pituitary. Parvocellular neurons of the paraventricular nucleus contain neurons that release corticotropin-releasing hormone and other hormones into the hypophyseal portal system

The hypothalamus is thus richly connected to many parts of the central nervous system, including the brainstem reticular formation and autonomic zones, the limbic forebrain (particularly the amygdala, septum, diagonal band of Broca, and the olfactory bulbs, and the cerebral cortex). The hypothalamic-pituitary-adrenal axis (HPA or HTPA axis), also known as the limbic-hypothalamic-pituitary-adrenal axis (LHPA axis) and, occasionally, as the hypothalamus-pituitary-adrenal-gonadotropic axis, is a complex set of direct influences and feedback interactions among the hypothalamus, the pituitary gland (a pea-shaped structure

Hypothalamic neurocysticercosis (HTNCC) is another extremelly uncommon presentation of NCC and the most common clinical features of best documented patient are characterized by: obesity, galactorrhea and central diabetes insipidus (polyuria without solute diuresis), and panhypopituitarism due to an associated pituitary gland involvement can also be present.[207,217, 219-222, 224] It has been established that intracranial hypertension "per se"

At a Uberaba Teaching Hospital in Brazil, a total of 1.596 autopsies performed between 1974 and 1997 were studied. The study found a diagnosis of cysticercosis in 53 autopsies (3.3%) and in two obese patients with NCC the lesions were located in the ventromedial nucleus of

Other authors also divided the unusual manifestations of neurocysticercosis into: intraventricular, subarachnoid, spinal, orbital, intraparenchymatous, and reactivation of previously calcified lesions and they reported frequencies of 15.69% of intraventricular presentations, subarachnoid: 11.63%; spinal: 3.49%; orbital: 0.58% and reactivated lesion: 0.58% and the frequencies for association of intraventricular and intraparenchymatous: 6.98%; association of subarachnoid and intraparenchymatous: 3.49; and association of subarachnoid and intraventricular: 2.32%. [225] but they did not include other presentations

The solitary cysticercus granuloma (SCG) is considered the most frequent type of NCC in India and many other Southeast Asian countries and these lesions are the most frequent

does not generate changes in the hypothalamopituitary axis function. [219]

Only one patient presenting lateral sinus thrombosis secondary to NCC has been reported in the medical literature up to date. [226]

## **4. Extracraneal cysticercosis**

Taenia solium cysticercosis can invade almost all extracraneal anatomical structures in the head excepting skin, nasal cavity, paranasal sinus, bone tissue and the periostium.

When a temporalis muscle is involved, clinical presentation can be a temporal headache. Two woman who complained of subacute onset of left temporal pain and another complaining headache due to cysticercosis of the temporalis muscle have been reported. [227, 228]

### **4.1. Ocular cysticercosis**

Ocular cysts are mostly vitreous or may be found in subretinal locations. Cysts can impair vision by floating in the eye and can lead to blindness by causing retinal detachment. Visualization of cysts via fundoscopy may be diagnostic of the disease.

We dedicated a complete article for this issue and interested readers can find detailed information in the chapter entitled: **"**Orbital Cysticercosis. A challenger for neurologists, ophthalmologists, neurophthalmologists, and general practitioners"

## **4.2. Facial cysticercosis**

We found facial cysticercosis in a patient presenting disseminate subcutaneous and muscular cysticercosis with cardiac involvement and NCC. [10] A cystic lesion on the right cheek and in the maxillofacial region have been also reported. [229, 230] A photo of patient showing a nodular lesion in the left hemiface at the zigomatic arch level that was removed by surgical procedures and histological examination confirmed T solium cysticercosis can be seen in a previous chapter.

## **4.3. Buco-lingua-pharyngeal cysticercosis**

#### *4.3.1. Oral cysticercosis*

As other before-cited locations of cysticercosis this one, is also extremely uncommon. Patients presenting lesion in the lips, [231, 232] oral cavity, [233] oral mucosa, [232] and in the gums [232] due to cysticercosis have been well documented. In all cases clinical manifestations are related to the size of the nodule lesion and good response to antiparasitic medication is the rule.

Uncommon Clinical Manifestations of Cysticercosis 227

great vessels and the trachea but up to date, tracheal cysticercosis is seen only in porcine cysticercosis. [265] Esophageal cysticercosis has not been described; T solium thymic cysticercosis has not been reported as yet. In the other hand; vagus nerves, the paired sympathetic chains, lymphatic and the thoracic duct cysticercosis have not been well documented in the medical literature neither. Finally we would like to emphasize that pericardial cysticercosis is also extremely uncommon and has been mentioned in the international medical literature occasionally. [264,266] Other intra-thoracic anatomical structures are: lungs where pulmonary cysticercosis has been communicated. [240, 249, 250] Pulmonary infiltration with eosinophilia is a disease entity characterized by lung disease and eosinophilia its commonest causes are tuberculosis, parasitic infestations, malignancy and connective tissue disorders. The first report about pleural effusion and subcutaneous cysticercosis in a 2 year old child is made by Salaria et al. in 2001. [268] In adults pleural

In women, the breasts overlay the pectoralis major muscles and usually extend from the level of the second rib to the level of the sixth rib in the front of the human rib cage; thus, the breasts cover much of the chest area and the chest walls. At the front of the chest, the breast tissue can extend from the clavicle (collarbone) to the middle of the sternum (breastbone). At the sides of the chest, the breast tissue can extend into the axilla (armpit), and can reach as far to the back as the latissimus dorsi muscle, extending from the lower back to the humerus bone (the longest bone of the upper arm). As a mammary gland, the breast is an homogeneous anatomic structure composed of layers of different types of tissue, among which predominate two types, adipose tissue and glandular tissue, which affects the lactation functions of the breasts. The blood supply to the breast is derived from 3 sources. The predominant supply of blood comes from the perforating branches of the internal mammary arteries, derived from the internal thoracic artery. The breast is supplied by the lateral thoracic and thoracoacromial arteries (branches of the axillary artery) as well as posterior intercostal arteries (branches of the thoracic aorta). Apart from breast pain, fibrocystic change, nipple discharge, tumor, bacterial and fungal infection and Paget's disease; breast can also be infected by T solium cysticercosis. Some patients can present from mastalgia, hyperprolactinaemia [271] and painless mobile swelling (lump) in the breast [272]

In places where cysticercosis is frequent the before mentioned presentations are rare while in countries where cysticercosis had been exceedingly rare (i.e. Malaysia), with only one case of neurocysticercosis diagnosed during post-mortem in 1934 and another one

Limbs cysticercosis may be isolated in the biceps brachii, deltoid, thigh, elbow, forearm, triceps, gluteal muscles [275-290] or as part of disseminated cysticercosis. [254, 291-296] The

effusion is the commonest presentation of pleural cysticercosis. [269, 270]

**5.2. Breast cysticercosis** 

to no clinical manifestations. [273]

**5.3. Limbs cysticercosis** 

diagnosed ante-mortem [274] any presentation is uncommon.

Cysticercus in the tongue can be present up to 50% in porcine cysticercosis. However in human beings only anecdotal cases have been reported. [232, 234]

## **4.4. Parotid gland cysticercosis**

Involvement of the parotid gland is manifest by a mass effect, masquerading as a salivary gland neoplasm or mild swelling with occasional aching sensation and headache, and features resembling acute parotitis [235-238] Parotid gland cysticercosis can be associated with NCC [237] or presenting as a solitary extracranial cysticercosis of the parotid gland. [238] In solitary lesions final diagnosis is based on fine needle aspiration cytology and immunological investigations and in patients with an associated NCC antigen/antibody tests for T solium cysticercosis can be useful.

## **4.5. Neck cysticercosis**

Subcutaneous cysticercosis in the neck is usually part of the disseminated cysticercosis and it does not constitute an independent clinical entity by itself because its clinical features are similar to another subcutaneous location. [10] We studied one patient with this condition who is cited in the chapter entitled: "Orbital cysticercosis. A challenger for a neurologist, ophthalmologist, neurophthalmologists, and general practitioners". Other uncommon presentations of cysticercosis around the neck are: thyroid cysticercosis (one patient is from our region), [239, 240] cricothyroid muscle cysticercosis, [241] pharyngeal cysticercosis, first described in South Africa, [242] submandibular lymphadenopathy cysticercosis in a 7-y-old girl presented with seizures and a right submandibular lymph node enlargement confirmed by fine needle aspiration cytology [243] and cervical lymphadenopathy cysticercosis [244] cysticercosis of submandibular duct [245] and isolated cysticercosis of the sternocleidomastoid [246] were also reported.

## **5. Truncal cysticercosis**

## **5.1. Thoracic cysticercosis**

The thoracic cavity (or chest cavity) is the chamber of the human body (and other animal bodies) that is protected by the thoracic wall (thoracic cage and associated skin, muscle, and fascia) which can be infected by isolated T solium cysticercosis or disseminated cysticercosis as it has been reported in South African patients. [10, 240, 242, 247, 251 ] and others. [241, 252-255] Anatomical structures within the thoracic cavity include: structures of the cardiovascular system, including the heart where cardiac cysticercosis can be seen. [10,247,248, 256-267] Parasitic diseases may involve various anatomic structures of the heart which can be manifested clinically as myocarditis, cardiomyopathies, pericarditis, or pulmonary hypertension in many resource-constrained settings. [263] Other structures are: great vessels and the trachea but up to date, tracheal cysticercosis is seen only in porcine cysticercosis. [265] Esophageal cysticercosis has not been described; T solium thymic cysticercosis has not been reported as yet. In the other hand; vagus nerves, the paired sympathetic chains, lymphatic and the thoracic duct cysticercosis have not been well documented in the medical literature neither. Finally we would like to emphasize that pericardial cysticercosis is also extremely uncommon and has been mentioned in the international medical literature occasionally. [264,266] Other intra-thoracic anatomical structures are: lungs where pulmonary cysticercosis has been communicated. [240, 249, 250] Pulmonary infiltration with eosinophilia is a disease entity characterized by lung disease and eosinophilia its commonest causes are tuberculosis, parasitic infestations, malignancy and connective tissue disorders. The first report about pleural effusion and subcutaneous cysticercosis in a 2 year old child is made by Salaria et al. in 2001. [268] In adults pleural effusion is the commonest presentation of pleural cysticercosis. [269, 270]

## **5.2. Breast cysticercosis**

226 Novel Aspects on Cysticercosis and Neurocysticercosis

**4.4. Parotid gland cysticercosis** 

tests for T solium cysticercosis can be useful.

sternocleidomastoid [246] were also reported.

**5. Truncal cysticercosis** 

**5.1. Thoracic cysticercosis** 

medication is the rule.

**4.5. Neck cysticercosis** 

the gums [232] due to cysticercosis have been well documented. In all cases clinical manifestations are related to the size of the nodule lesion and good response to antiparasitic

Cysticercus in the tongue can be present up to 50% in porcine cysticercosis. However in

Involvement of the parotid gland is manifest by a mass effect, masquerading as a salivary gland neoplasm or mild swelling with occasional aching sensation and headache, and features resembling acute parotitis [235-238] Parotid gland cysticercosis can be associated with NCC [237] or presenting as a solitary extracranial cysticercosis of the parotid gland. [238] In solitary lesions final diagnosis is based on fine needle aspiration cytology and immunological investigations and in patients with an associated NCC antigen/antibody

Subcutaneous cysticercosis in the neck is usually part of the disseminated cysticercosis and it does not constitute an independent clinical entity by itself because its clinical features are similar to another subcutaneous location. [10] We studied one patient with this condition who is cited in the chapter entitled: "Orbital cysticercosis. A challenger for a neurologist, ophthalmologist, neurophthalmologists, and general practitioners". Other uncommon presentations of cysticercosis around the neck are: thyroid cysticercosis (one patient is from our region), [239, 240] cricothyroid muscle cysticercosis, [241] pharyngeal cysticercosis, first described in South Africa, [242] submandibular lymphadenopathy cysticercosis in a 7-y-old girl presented with seizures and a right submandibular lymph node enlargement confirmed by fine needle aspiration cytology [243] and cervical lymphadenopathy cysticercosis [244] cysticercosis of submandibular duct [245] and isolated cysticercosis of the

The thoracic cavity (or chest cavity) is the chamber of the human body (and other animal bodies) that is protected by the thoracic wall (thoracic cage and associated skin, muscle, and fascia) which can be infected by isolated T solium cysticercosis or disseminated cysticercosis as it has been reported in South African patients. [10, 240, 242, 247, 251 ] and others. [241, 252-255] Anatomical structures within the thoracic cavity include: structures of the cardiovascular system, including the heart where cardiac cysticercosis can be seen. [10,247,248, 256-267] Parasitic diseases may involve various anatomic structures of the heart which can be manifested clinically as myocarditis, cardiomyopathies, pericarditis, or pulmonary hypertension in many resource-constrained settings. [263] Other structures are:

human beings only anecdotal cases have been reported. [232, 234]

In women, the breasts overlay the pectoralis major muscles and usually extend from the level of the second rib to the level of the sixth rib in the front of the human rib cage; thus, the breasts cover much of the chest area and the chest walls. At the front of the chest, the breast tissue can extend from the clavicle (collarbone) to the middle of the sternum (breastbone). At the sides of the chest, the breast tissue can extend into the axilla (armpit), and can reach as far to the back as the latissimus dorsi muscle, extending from the lower back to the humerus bone (the longest bone of the upper arm). As a mammary gland, the breast is an homogeneous anatomic structure composed of layers of different types of tissue, among which predominate two types, adipose tissue and glandular tissue, which affects the lactation functions of the breasts. The blood supply to the breast is derived from 3 sources. The predominant supply of blood comes from the perforating branches of the internal mammary arteries, derived from the internal thoracic artery. The breast is supplied by the lateral thoracic and thoracoacromial arteries (branches of the axillary artery) as well as posterior intercostal arteries (branches of the thoracic aorta). Apart from breast pain, fibrocystic change, nipple discharge, tumor, bacterial and fungal infection and Paget's disease; breast can also be infected by T solium cysticercosis. Some patients can present from mastalgia, hyperprolactinaemia [271] and painless mobile swelling (lump) in the breast [272] to no clinical manifestations. [273]

In places where cysticercosis is frequent the before mentioned presentations are rare while in countries where cysticercosis had been exceedingly rare (i.e. Malaysia), with only one case of neurocysticercosis diagnosed during post-mortem in 1934 and another one diagnosed ante-mortem [274] any presentation is uncommon.

#### **5.3. Limbs cysticercosis**

Limbs cysticercosis may be isolated in the biceps brachii, deltoid, thigh, elbow, forearm, triceps, gluteal muscles [275-290] or as part of disseminated cysticercosis. [254, 291-296] The

clinical features depend on the location of the cyst, the cyst burden, and the host reaction but most of patients' present painless, rubbery nodules around 2 cm in diameter, which do not carry any risk to the patient's health or can present pseudotumor, mass, pseudohypertrophy [295-298] and myalgias. [298] A positive diagnosis is established solely by incision and examination of the interior, where the parasite will be found. In the other hand , the role of ultrasonographic (USG) studies in the diagnosis of isolated soft tissue cysticercosis has been also confirmed. [295] The most common USG appearance is that of a cyst containing a scolex within and with surrounding abscess. The second most common appearance reported is that of a cyst containing a scolex within and with surrounding edema and the least common appearance is that of an irregular cyst with no scolex within and with surrounding edema. [295] Fine needle aspiration cytology [296] and even mitochondrial DNA analysis of extremely calcified cysts have demonstrated a good efficacy. [288]

Uncommon Clinical Manifestations of Cysticercosis 229

The most common location for soft tissue cysticercosis is in the skeletal muscles of the upper extremities (33. %) as we show in the next figure. , and the least common sites for isolated muscular cysticercosis are the back, buttock and cheek, which accounted for 3% each [287].

A joint is the location at which two or more bones make contact. They are constructed to allow movement (except for skull bones) and provide mechanical support to the body. A joint disorder is termed an arthropathy, and when involving inflammation of one or more

Arthropathies are called *polyarticular* when involving many joints and *monoarticular* when involving only one single joint how can happens cysticercotic arthropathy. Fortunately it is another very rare condition. Kurrein and Vickers [303] reported a former regular soldier who had served in India before the Second World War. He was in 1953-1955 diagnosed as suffering from cysticercosis affecting the brain and spinal cord as well as the muscles. Twenty years later he was found to have sustained severe damage to the lumbar spine, eventually proved due to cysticercosis involving the bones, **joints** and psoas muscles. The problem of why and how the disease remained active 40 years after the initial exposure remains unexplained. Searches of the literature and enquiries from appropriate specialists have failed to produce any record of similar cases. Kumar e al., [304] studied a 62-years old man presented with complaints of pain in the large peripheral joints with limitation of movement for two months. Fine needle aspiration cytology of the nodular eruptions over joints demonstrated negatively birefringent needle shaped crystals while that of calcific lesion in soft tissue demonstrated *cysticercus cellulose* with scolex. However the majority of the researchers that we have consulted, agree that the diagnosis of joint cysticercosis in this

The last report is made by Banu and Veen. [305] They studied an immunocompetent patient with disseminated cysticercosis who had involvement of the brain, subcutaneous tissues, lungs and skeletal muscles and presented with arthritis. This patient was otherwise

Unfortunately we have come to the final part of this chapter faced severe space restrictions by what we have to reduce the content of each paragraph without sacrificing the quality of

The abdominopelvic cavity (APC) is the cavity of the human body that holds the bulk of the viscera including include the stomach, liver, gallbladder, spleen, pancreas, small intestine, kidneys, large intestine and reproductive organs, the urinary bladder, the pelvic colon, and the rectum. The APC is lined with a protective membrane termed the peritoneum. The kidneys are located in the abdominal cavity behind the peritoneum, in the retroperitoneum. The viscera are also covered, in the front, with a layer of peritoneum called the greater

**5.4. Monoarticular cysticercosis** 

joints the disorder is called: arthritis.

patient demonstrates some doubts though cannot be rejected.

**6. Abdominopelvic cysticercosis** 

this chapter.

asymptomatic in spite of the extensive involvement of multiple organs.

We found a good response to 100 mg/kg of praziquantel for a day [292] but in most patients albendazole is the medication of choice.

Cysticercosis of the flexordigitorum profundus muscle producing flexion deformity of the fingers and cysticercosis of the tendon sheath of Tendoachilles have been reported. [300, 301]

Only one patient with cysticercosis presenting as an ulnar entrapment neuropathy due to a compressive mass in the flexor digitorum profundus has been reported. In this case magnetic resonance imaging suggested an inflammatory mass compressing the neurovascular bundle and the final diagnosis was confirmed by histologic examination. [302]

**Figure 8.** Subcutaneous and intramuscular cysticercosis in the proximal region of the limbs predominantly

The most common location for soft tissue cysticercosis is in the skeletal muscles of the upper extremities (33. %) as we show in the next figure. , and the least common sites for isolated muscular cysticercosis are the back, buttock and cheek, which accounted for 3% each [287].

## **5.4. Monoarticular cysticercosis**

228 Novel Aspects on Cysticercosis and Neurocysticercosis

albendazole is the medication of choice.

[288]

301]

[302]

predominantly

clinical features depend on the location of the cyst, the cyst burden, and the host reaction but most of patients' present painless, rubbery nodules around 2 cm in diameter, which do not carry any risk to the patient's health or can present pseudotumor, mass, pseudohypertrophy [295-298] and myalgias. [298] A positive diagnosis is established solely by incision and examination of the interior, where the parasite will be found. In the other hand , the role of ultrasonographic (USG) studies in the diagnosis of isolated soft tissue cysticercosis has been also confirmed. [295] The most common USG appearance is that of a cyst containing a scolex within and with surrounding abscess. The second most common appearance reported is that of a cyst containing a scolex within and with surrounding edema and the least common appearance is that of an irregular cyst with no scolex within and with surrounding edema. [295] Fine needle aspiration cytology [296] and even mitochondrial DNA analysis of extremely calcified cysts have demonstrated a good efficacy.

We found a good response to 100 mg/kg of praziquantel for a day [292] but in most patients

Cysticercosis of the flexordigitorum profundus muscle producing flexion deformity of the fingers and cysticercosis of the tendon sheath of Tendoachilles have been reported. [300,

Only one patient with cysticercosis presenting as an ulnar entrapment neuropathy due to a compressive mass in the flexor digitorum profundus has been reported. In this case magnetic resonance imaging suggested an inflammatory mass compressing the neurovascular bundle and the final diagnosis was confirmed by histologic examination.

**Figure 8.** Subcutaneous and intramuscular cysticercosis in the proximal region of the limbs

A joint is the location at which two or more bones make contact. They are constructed to allow movement (except for skull bones) and provide mechanical support to the body. A joint disorder is termed an arthropathy, and when involving inflammation of one or more joints the disorder is called: arthritis.

Arthropathies are called *polyarticular* when involving many joints and *monoarticular* when involving only one single joint how can happens cysticercotic arthropathy. Fortunately it is another very rare condition. Kurrein and Vickers [303] reported a former regular soldier who had served in India before the Second World War. He was in 1953-1955 diagnosed as suffering from cysticercosis affecting the brain and spinal cord as well as the muscles. Twenty years later he was found to have sustained severe damage to the lumbar spine, eventually proved due to cysticercosis involving the bones, **joints** and psoas muscles. The problem of why and how the disease remained active 40 years after the initial exposure remains unexplained. Searches of the literature and enquiries from appropriate specialists have failed to produce any record of similar cases. Kumar e al., [304] studied a 62-years old man presented with complaints of pain in the large peripheral joints with limitation of movement for two months. Fine needle aspiration cytology of the nodular eruptions over joints demonstrated negatively birefringent needle shaped crystals while that of calcific lesion in soft tissue demonstrated *cysticercus cellulose* with scolex. However the majority of the researchers that we have consulted, agree that the diagnosis of joint cysticercosis in this patient demonstrates some doubts though cannot be rejected.

The last report is made by Banu and Veen. [305] They studied an immunocompetent patient with disseminated cysticercosis who had involvement of the brain, subcutaneous tissues, lungs and skeletal muscles and presented with arthritis. This patient was otherwise asymptomatic in spite of the extensive involvement of multiple organs.

## **6. Abdominopelvic cysticercosis**

Unfortunately we have come to the final part of this chapter faced severe space restrictions by what we have to reduce the content of each paragraph without sacrificing the quality of this chapter.

The abdominopelvic cavity (APC) is the cavity of the human body that holds the bulk of the viscera including include the stomach, liver, gallbladder, spleen, pancreas, small intestine, kidneys, large intestine and reproductive organs, the urinary bladder, the pelvic colon, and the rectum. The APC is lined with a protective membrane termed the peritoneum. The kidneys are located in the abdominal cavity behind the peritoneum, in the retroperitoneum. The viscera are also covered, in the front, with a layer of peritoneum called the greater

omentum. The peritoneal cavity is a potential space between the parietal peritoneum and visceral peritoneum, that is, the two membranes that separate the organs in the abdominal cavity from the abdominal wall. The peritoneal cavity is the largest serosa sac in the body and secretes approximately 50 ml of fluid per day. This fluid acts as a lubricant and has antiinflammatory properties.

Uncommon Clinical Manifestations of Cysticercosis 231

In this series patient with visceral cysticercosis, the cysts were localized in the heart, pleura,

Cysticercosis of the liver by T solium or other tapeworms has been reported in the animal population since long time ago, [312] and the first human being patient with hepatic cysticercosis and clinical manifestations of liver damage and not associated to disseminate cysticercosis was reported in the international medical literature in 1995. [313] Up to date only four patients with hepatic cysticercosis have been well documented.[313-316] Solitary nodular lesion remains asymptomatic while multinodular lesions may manifest as acute or chronic hepatitis. The experience of the last report highlights the need to use high resolution

In one of the previous chapter we mentioned that DCC is a rare form of cysticercosis caused by dissemination of the larval form of the pork tapeworm *T. solium* and fewer than 50 cases have been reported worldwide. Involvement of the pancreas and spleen is even rarer. The best of our knowledge, the first patient presenting splenic and pancreatic involvement by cysticercosis leading to pancreatitis was born and grew up at the former rural Transkei in South Africa (where we are living) and was reported in 1987 by Scholtz and Mentis [240] the second case is reported by Jain et al., [317] and their patient presented a history of headache and vomiting and was found to have DCC with pulmonary, cardiac , pancreatic, intraocular, and extradural spinal canal involvement. The third (last one) patient presented a disseminated cysticercosis involving the brain, orbit, myocardium, muscle, subcutaneous tissues, spleen and pancreas but without clinical or biochemical evidence of pancreatitis due

Cysticercosis of the spleen [318,319] and the kidneys [240, 311,319] have been reported in two and three occasions respectively, always as part of a disseminate cysticercosis. Clinical manifestations secondary to the spleen or renal damage caused by *T solium* are uncertain up to date. The first reported patient presenting renal cysticercosis came from our region.

Tenia solium cysticercosis can infect ovary and one anecdotal case has been without

There are several types of tapeworms such as *T pisiformis, T hydatigena, T saginata, Taenia crassiceps* and other parasites that can be found in the peritoneal cavity in several types of

ultrasonography of abdomen in patients with a high index of suspicion. [314]

kidney and ovary without clinical manifestations of importance.

**6.3. Hepatic cysticercosis** 

**6.4. Pancreatic cysticercosis** 

to infection by cysticerci. [318]

**6.6. Pelvic cysticercosis** 

**6.7. Peritoneal cysticercosis** 

**6.5. Spleen and Kidney cysticercosis** 

remarkable clinical manifestation has been reported. [311]

Cysticercosis of the whole APC is another preventable faeco-oral transmitted T solium infection but for didactic purposes we will divide this topic in extra-abdominal and intraabdominal cysticercosis.

## **6.1. Extra-abdominal cysticercosis**

In the abdominal, wall *T solium* cysticercosis can be found involving the anterior abdominal wall musculature [306-308] and diagnosis can be done by sonography which can reveal a well-defined cystic lesion with an eccentric hyperechoic area within it and an eccentric, echogenic, pedunculated structure is seen within the cystic area of the lesion without calcification but inflammation and hypervascularity in the surrounding muscle also can be noted or can be confirmed by histopathology. Clinical manifestations include vague abdominal discomfort, tender or painless nodules. This presentation is more commonly seen in patients presenting disseminated cysticercosis [283, 292, 293, 296-298, 300-303] rather than isolated cysticercosis. [277-282, 286-289, 294]

## **6.2. Intra-Abdominal cysticercosis**

In some anecdotal cases intra-abdominal cysticercosis is presenting as abdominal pain secondary to acute abdomen and reported cases have shown psoas muscle cysticercosis mimicking appendicitis [309] and mesenteric lymph node enlargement cysticercosis and associated appendicitis. [310] In this case is postulated that the larvae, after evagination in the small intestine, penetrated the mesenteric lymphatic, burrowing through the bowel wall, hence reaching the mesenteric lymph node. The before-cited case is the second case of isolated lymph node enlargement due to cysticercus infestation reported in the medical literature.

One study made by Vianna et al., [311] shows the frequency of cutaneous, muscular and visceral forms of cysticercosis in anatomopathological examinations and necropsies in the Federal District of Sao Paulo. In 64.911 protocols of anatomopathological examinations they found that 30 (0.05%) had the diagnosis of cysticercosis, 90% had cysticerci in the subcutaneous tissue, skeletal muscle or mucous membrane, 3.3% in lymph node and 6.7% in the central nervous system and two (7.4%) patients had cysticerci in the tongue. In 1520 autopsy protocols reviewed, 25 (1.6%) autopsies with cysticercosis were found. 96% had cysticerci in the central nervous system, 8% in the heart, 8% in the skeletal muscle and 4% in the liver, either isolated or associated with cysticerci in other viscera. A total of 1122 people were also examined and the muscular cysticercosis was found in 42.4%, subcutaneous cysticercosis was present in 20.3% and the visceral cysticercosis was found in 3.4% of cases. In this series patient with visceral cysticercosis, the cysts were localized in the heart, pleura, kidney and ovary without clinical manifestations of importance.

## **6.3. Hepatic cysticercosis**

230 Novel Aspects on Cysticercosis and Neurocysticercosis

**6.1. Extra-abdominal cysticercosis** 

isolated cysticercosis. [277-282, 286-289, 294]

**6.2. Intra-Abdominal cysticercosis** 

literature.

inflammatory properties.

abdominal cysticercosis.

omentum. The peritoneal cavity is a potential space between the parietal peritoneum and visceral peritoneum, that is, the two membranes that separate the organs in the abdominal cavity from the abdominal wall. The peritoneal cavity is the largest serosa sac in the body and secretes approximately 50 ml of fluid per day. This fluid acts as a lubricant and has anti-

Cysticercosis of the whole APC is another preventable faeco-oral transmitted T solium infection but for didactic purposes we will divide this topic in extra-abdominal and intra-

In the abdominal, wall *T solium* cysticercosis can be found involving the anterior abdominal wall musculature [306-308] and diagnosis can be done by sonography which can reveal a well-defined cystic lesion with an eccentric hyperechoic area within it and an eccentric, echogenic, pedunculated structure is seen within the cystic area of the lesion without calcification but inflammation and hypervascularity in the surrounding muscle also can be noted or can be confirmed by histopathology. Clinical manifestations include vague abdominal discomfort, tender or painless nodules. This presentation is more commonly seen in patients presenting disseminated cysticercosis [283, 292, 293, 296-298, 300-303] rather than

In some anecdotal cases intra-abdominal cysticercosis is presenting as abdominal pain secondary to acute abdomen and reported cases have shown psoas muscle cysticercosis mimicking appendicitis [309] and mesenteric lymph node enlargement cysticercosis and associated appendicitis. [310] In this case is postulated that the larvae, after evagination in the small intestine, penetrated the mesenteric lymphatic, burrowing through the bowel wall, hence reaching the mesenteric lymph node. The before-cited case is the second case of isolated lymph node enlargement due to cysticercus infestation reported in the medical

One study made by Vianna et al., [311] shows the frequency of cutaneous, muscular and visceral forms of cysticercosis in anatomopathological examinations and necropsies in the Federal District of Sao Paulo. In 64.911 protocols of anatomopathological examinations they found that 30 (0.05%) had the diagnosis of cysticercosis, 90% had cysticerci in the subcutaneous tissue, skeletal muscle or mucous membrane, 3.3% in lymph node and 6.7% in the central nervous system and two (7.4%) patients had cysticerci in the tongue. In 1520 autopsy protocols reviewed, 25 (1.6%) autopsies with cysticercosis were found. 96% had cysticerci in the central nervous system, 8% in the heart, 8% in the skeletal muscle and 4% in the liver, either isolated or associated with cysticerci in other viscera. A total of 1122 people were also examined and the muscular cysticercosis was found in 42.4%, subcutaneous cysticercosis was present in 20.3% and the visceral cysticercosis was found in 3.4% of cases. Cysticercosis of the liver by T solium or other tapeworms has been reported in the animal population since long time ago, [312] and the first human being patient with hepatic cysticercosis and clinical manifestations of liver damage and not associated to disseminate cysticercosis was reported in the international medical literature in 1995. [313] Up to date only four patients with hepatic cysticercosis have been well documented.[313-316] Solitary nodular lesion remains asymptomatic while multinodular lesions may manifest as acute or chronic hepatitis. The experience of the last report highlights the need to use high resolution ultrasonography of abdomen in patients with a high index of suspicion. [314]

## **6.4. Pancreatic cysticercosis**

In one of the previous chapter we mentioned that DCC is a rare form of cysticercosis caused by dissemination of the larval form of the pork tapeworm *T. solium* and fewer than 50 cases have been reported worldwide. Involvement of the pancreas and spleen is even rarer. The best of our knowledge, the first patient presenting splenic and pancreatic involvement by cysticercosis leading to pancreatitis was born and grew up at the former rural Transkei in South Africa (where we are living) and was reported in 1987 by Scholtz and Mentis [240] the second case is reported by Jain et al., [317] and their patient presented a history of headache and vomiting and was found to have DCC with pulmonary, cardiac , pancreatic, intraocular, and extradural spinal canal involvement. The third (last one) patient presented a disseminated cysticercosis involving the brain, orbit, myocardium, muscle, subcutaneous tissues, spleen and pancreas but without clinical or biochemical evidence of pancreatitis due to infection by cysticerci. [318]

### **6.5. Spleen and Kidney cysticercosis**

Cysticercosis of the spleen [318,319] and the kidneys [240, 311,319] have been reported in two and three occasions respectively, always as part of a disseminate cysticercosis. Clinical manifestations secondary to the spleen or renal damage caused by *T solium* are uncertain up to date. The first reported patient presenting renal cysticercosis came from our region.

#### **6.6. Pelvic cysticercosis**

Tenia solium cysticercosis can infect ovary and one anecdotal case has been without remarkable clinical manifestation has been reported. [311]

#### **6.7. Peritoneal cysticercosis**

There are several types of tapeworms such as *T pisiformis, T hydatigena, T saginata, Taenia crassiceps* and other parasites that can be found in the peritoneal cavity in several types of

animals, [320-324] but we are not aware of patients with peritoneal cysticercosis caused by T solium cysticercosis up to date.

Uncommon Clinical Manifestations of Cysticercosis 233

We wish to mention our eternal and deepest gratitude to Lorna María Foyaca García, Fátima

Hereby, we acknowledge financial support from the Directorate of Research Development, Walter Sisulu University in South Africa, South African Medical Research Council and NRF. The founder had no role in study design, data collection and analysis, decision to publish, or

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Susana Foyaca Ibañez and Thabo Humberto Foyaca Ibañez for their delight support.

**Acknowledgement** 

preparation of the manuscript.

Internet Journal of Neurology 2002;2):41-50.

**8. References** 

neurocys.htm

Number 2.

ature.html

## **6.8. Adrenal gland cysticercosis**

The adrenal glands (also known as suprarenal glands) are endocrine glands that sit atop the kidneys; in humans, the right adrenal gland is triangular shaped, while the left adrenal gland is semilunar shaped. On CT scan, the average measurements for the right adrenal gland were: maximum width 0.61 cm (S.D. 0.2), the width of the medial limb 0.28 cm (S.D. 0.08), and width of the lateral limb 0.28 cm (S.D. 0.06). The average measurements for the left adrenal gland were: maximum width 0.79 cm (S.D. 0.21), width of the medial limb 0.33 cm (S.D. 0.09), and width of the lateral limb 0.30 cm (S.D. 0.10), [325] typically, they are less than 4 cm in length and less than 1 cm in width.

Although variations of the blood supply to the adrenal glands are common, there are usually three arteries that supply each adrenal gland such as: the superior suprarenal artery, the middle suprarenal artery and the inferior suprarenal artery which represent three paths for cysticercosis to reach the adrenal glands. However due to the size of the adrenal glands the cysticerci cannot be accommodated there; therefore, the adrenal cistticercosis does not exist. Nevertheless, there are clinical manifestations of adrenal dysfunction related to T solium cysticercosis (even simulating pheocromocytoma) but secondary to NCC [326-328] when it is affecting the limbic-hypothalamic-pituitary-adrenal axis.

## **7. Conclusion**

After an extensive review of the medical literature and from our database, we concluded that the uncommon clinical manifestations of cysticercosis are related to the immune status of the patient, the location and numbers or cisticercus in any organ or tissue able to accommodate cysticerci depending of its size, consistency of the tissue, and the characteristics of its blood supply.

From the before-mentioned postulate is that there will never be cysticercosis in the skin, hair, nails, bones, and or organs, tissues and membranes with dimensions less than one centimeter. Outside of this rule, the cysticerci can be viewed in any organ of the human body causing direct or indirect damage with or without clinical manifestations. So any patient from an endemic area or not presenting clinical manifestation of unknown causes deserves an investigation in search of cysticercosis.

## **Author details**

Humberto Foyaca Sibat and Lourdes de Fátima Ibañez Valdés *Department of Neurology, Stroke Unit, Epilepsy and neurocysticercosis Clinic in Nelson Mandela Academic Hospital, Faculty of Health Sciences, Walter Sisulu University, Mthatha, South Africa* 

## **Acknowledgement**

232 Novel Aspects on Cysticercosis and Neurocysticercosis

solium cysticercosis up to date.

**7. Conclusion** 

**Author details** 

characteristics of its blood supply.

deserves an investigation in search of cysticercosis.

*Academic Hospital, Faculty of Health Sciences, Walter Sisulu University, Mthatha, South Africa* 

Humberto Foyaca Sibat and Lourdes de Fátima Ibañez Valdés

**6.8. Adrenal gland cysticercosis** 

than 4 cm in length and less than 1 cm in width.

when it is affecting the limbic-hypothalamic-pituitary-adrenal axis.

animals, [320-324] but we are not aware of patients with peritoneal cysticercosis caused by T

The adrenal glands (also known as suprarenal glands) are endocrine glands that sit atop the kidneys; in humans, the right adrenal gland is triangular shaped, while the left adrenal gland is semilunar shaped. On CT scan, the average measurements for the right adrenal gland were: maximum width 0.61 cm (S.D. 0.2), the width of the medial limb 0.28 cm (S.D. 0.08), and width of the lateral limb 0.28 cm (S.D. 0.06). The average measurements for the left adrenal gland were: maximum width 0.79 cm (S.D. 0.21), width of the medial limb 0.33 cm (S.D. 0.09), and width of the lateral limb 0.30 cm (S.D. 0.10), [325] typically, they are less

Although variations of the blood supply to the adrenal glands are common, there are usually three arteries that supply each adrenal gland such as: the superior suprarenal artery, the middle suprarenal artery and the inferior suprarenal artery which represent three paths for cysticercosis to reach the adrenal glands. However due to the size of the adrenal glands the cysticerci cannot be accommodated there; therefore, the adrenal cistticercosis does not exist. Nevertheless, there are clinical manifestations of adrenal dysfunction related to T solium cysticercosis (even simulating pheocromocytoma) but secondary to NCC [326-328]

After an extensive review of the medical literature and from our database, we concluded that the uncommon clinical manifestations of cysticercosis are related to the immune status of the patient, the location and numbers or cisticercus in any organ or tissue able to accommodate cysticerci depending of its size, consistency of the tissue, and the

From the before-mentioned postulate is that there will never be cysticercosis in the skin, hair, nails, bones, and or organs, tissues and membranes with dimensions less than one centimeter. Outside of this rule, the cysticerci can be viewed in any organ of the human body causing direct or indirect damage with or without clinical manifestations. So any patient from an endemic area or not presenting clinical manifestation of unknown causes

*Department of Neurology, Stroke Unit, Epilepsy and neurocysticercosis Clinic in Nelson Mandela* 

We wish to mention our eternal and deepest gratitude to Lorna María Foyaca García, Fátima Susana Foyaca Ibañez and Thabo Humberto Foyaca Ibañez for their delight support.

Hereby, we acknowledge financial support from the Directorate of Research Development, Walter Sisulu University in South Africa, South African Medical Research Council and NRF. The founder had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.

## **8. References**


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**Section 2** 

**Neurocysticercosis** 


**Neurocysticercosis** 

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**Chapter 9** 

© 2013 Flisser, licensee InTech. This is an open access chapter distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use,

distribution, and reproduction in any medium, provided the original work is properly cited.

© 2013 Flisser, licensee InTech. This is a paper distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

**Figure 1.** Images of cysticerci lodged in different locations. From top (left) to the bottom (right): diseminated sbcutaneous cysticercosis, confirmed by biopsy, lingual cysticercosis, intramuscular (pseudohypertrophy), intraocular (anterior chamber), intraventricular (IV ventricle), multiple locations

of cellulosae type and subarachnoid (racemose) neurocysticercosis.

**Epidemiology of Neurocysticercosis in Mexico:** 

Cysticercosis is a parasitic disease of humans caused by the establishment of the larval stage

**From a Public Health Problem to Its Control** 

Additional information is available at the end of the chapter

of the cestode *Taenia solium* in several organs (Figure 1).

Ana Flisser

http://dx.doi.org/10.5772/53839

**1. Introduction** 

## **Epidemiology of Neurocysticercosis in Mexico: From a Public Health Problem to Its Control**

Ana Flisser

Additional information is available at the end of the chapter

http://dx.doi.org/10.5772/53839

## **1. Introduction**

Cysticercosis is a parasitic disease of humans caused by the establishment of the larval stage of the cestode *Taenia solium* in several organs (Figure 1).

**Figure 1.** Images of cysticerci lodged in different locations. From top (left) to the bottom (right): diseminated sbcutaneous cysticercosis, confirmed by biopsy, lingual cysticercosis, intramuscular (pseudohypertrophy), intraocular (anterior chamber), intraventricular (IV ventricle), multiple locations of cellulosae type and subarachnoid (racemose) neurocysticercosis.

© 2013 Flisser, licensee InTech. This is an open access chapter distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. © 2013 Flisser, licensee InTech. This is a paper distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

The main clinical presentation is neurocysticercosis (NCC); it is due to the establishment of cysticerci in the Central Nervous System, where, depending on the specific location, can cause different symptoms, the main being epilepsy and hydrocephalus. Cysticercosis is a disease associated to poverty, lack of health education, inadequate hygienic habits and insufficient sanitary infrastructure; therefore it is common in underdeveloped countries. The life cycle of the parasite includes the human being, which is the only definitive host and, thus, lodges the adult tapeworm, which measures 1-4 meters long and is formed by a scolex, a neck and a long chain of proglottids that, as they are farther away from the neck, they develop into sexual maturity and, afterwards, gravidity.

Epidemiology of Neurocysticercosis in Mexico: From a Public Health Problem to Its Control 257

Cysticercosis has been known since ancient times; it was detected in an Egyptian mummy by paleoparasitologists [2]. In a popular Greek theater play "The Knights" by Aristophanes, the slave Demosthenes says "We will set his mouth open with a wooden stick as the cooks do with pigs; we will tear out his tongue, and, looking down his gaping throat, will see whether his inside has any pimples" [3]. Egyptian and Greek cultures also distinguished tapeworms, but the relation between both developmental stages was not known. Probably Egiptians had *Taenia saginata* because they did not eat pork meat; Hipocrates, Aristoteles y Teofrastus called the worms "flatworms" while the Romans, Celsus, Galenus and Pliny the elder, called them "lumbricus latus" that meant "wideworm". At the beggining of the Christian era, some Arab authors such as Serapio, considered that each proglottid was a different worm and were named "cucurbitinum" not only because of their resemblance to pumpkin seeds, but also because these seeds were used to release tapeworms, and,

The species T. solium was described by Villanovani in 1585 and reflects the common finding of a solitary tapeworm, *T. solium* was differentiated from *T. saginata* and *Diphyllobothrium latum* by Tyson in 1683 who discovered and described the different types of heads or scolices of tapeworms; Redi published the following year illustrations of the scolex of dog and cat tapeworms. Van Beneden 170 years later stated that a tapeworm was an animal formed by multiple individuals and that cysticerci in pigs develop after ingesting *T. solium* segments. At that time Kuchenmeister, in spte of severe ethical criticisms, identified that humans are the hosts of the adult stage of *T. solium*. In two occasions (1854 and 1859) he fed death convicts with 63 and 40 cysticerci each, and found developing tapeworms in the small intestine after necropsy. Interestingly the number of tapeworms found was 10 and 20 respectively and, since the second prisoner ingested the bladder worms much before death than the first convict (four months vs 4 days) 11 worms had mature proglottids and the largest reached five feet (1.5 meters) in length. This finding further demonstrates that *T. solium* is not necessarily a solitary parasite. He stated that the sheer number of tapeworms produced ought to convince even the most skeptical that they were derived from the cysticerci that the convicts ingested. Nevertheless he recommended that the experiment of bladder worm feeding should be allowed to be repetead on criminals under death sentences and, in the case of subsequent pardon, the tapeworms could be easily expelled, calming anxious souls and serving science at the same time [4]. Figure 3 shows pictures of some of the scientists that made outstanding contributions to the understanding of the life cycle of T. solium: Malpighi, Tyson, van Beneden and Kuchenmeister. Not until last century the precise knowledge of the complete anatomy of a tapeworm, as well as its organization and

Rumler was the first clinician that described a case of human cysticercosis in 1558 but considered it to be a tumor in the dura matter of a person with epilepsy. Panarolus, one hundred years later, reported similar cysts in the corpus callosum of the brain of an epileptic priest and Wharton found many cysticerci, that he considered to be glands in the adipose and muscle tissues of a soldier. The disease was not clearly identified as parasitic until

interestingly, are still in use nowadays [4].

individuality were defined, considering it a single animal.

Each gravid proglottid contains around 60,000 microscopic eggs; these segments are periodically released with feces. When outdoor defecation or improper latrines are found, pigs ingest feces; if contaminated with tapeworm eggs or proglottids, the embryos transform into the larval stage or cysticercus in the musculature and the central nervous system.

If humans eat insufficiently cooked or raw pork that is contaminated with cysticerci, the scolex evaginates, and with its doble row of hooks and four suckers in the rostellum, anchors to the intestinal mucosa and, after a few months, a new gravid tapeworm initiates the life cycle (Figure 2) [1].

**Figure 2.** Life cycle of *T solium*. A- Adult in the small intestine. B- gravid proglotid. C- infective egg containing an oncosphere. D- Human cysticercosis. E- Porcine cysticercosis an hexacanth oncosphere and an invaginated cysticercus are drawn.

Cysticercosis has been known since ancient times; it was detected in an Egyptian mummy by paleoparasitologists [2]. In a popular Greek theater play "The Knights" by Aristophanes, the slave Demosthenes says "We will set his mouth open with a wooden stick as the cooks do with pigs; we will tear out his tongue, and, looking down his gaping throat, will see whether his inside has any pimples" [3]. Egyptian and Greek cultures also distinguished tapeworms, but the relation between both developmental stages was not known. Probably Egiptians had *Taenia saginata* because they did not eat pork meat; Hipocrates, Aristoteles y Teofrastus called the worms "flatworms" while the Romans, Celsus, Galenus and Pliny the elder, called them "lumbricus latus" that meant "wideworm". At the beggining of the Christian era, some Arab authors such as Serapio, considered that each proglottid was a different worm and were named "cucurbitinum" not only because of their resemblance to pumpkin seeds, but also because these seeds were used to release tapeworms, and, interestingly, are still in use nowadays [4].

256 Novel Aspects on Cysticercosis and Neurocysticercosis

the life cycle (Figure 2) [1].

and an invaginated cysticercus are drawn.

develop into sexual maturity and, afterwards, gravidity.

The main clinical presentation is neurocysticercosis (NCC); it is due to the establishment of cysticerci in the Central Nervous System, where, depending on the specific location, can cause different symptoms, the main being epilepsy and hydrocephalus. Cysticercosis is a disease associated to poverty, lack of health education, inadequate hygienic habits and insufficient sanitary infrastructure; therefore it is common in underdeveloped countries. The life cycle of the parasite includes the human being, which is the only definitive host and, thus, lodges the adult tapeworm, which measures 1-4 meters long and is formed by a scolex, a neck and a long chain of proglottids that, as they are farther away from the neck, they

Each gravid proglottid contains around 60,000 microscopic eggs; these segments are periodically released with feces. When outdoor defecation or improper latrines are found, pigs ingest feces; if contaminated with tapeworm eggs or proglottids, the embryos transform into

If humans eat insufficiently cooked or raw pork that is contaminated with cysticerci, the scolex evaginates, and with its doble row of hooks and four suckers in the rostellum, anchors to the intestinal mucosa and, after a few months, a new gravid tapeworm initiates

**Figure 2.** Life cycle of *T solium*. A- Adult in the small intestine. B- gravid proglotid. C- infective egg containing an oncosphere. D- Human cysticercosis. E- Porcine cysticercosis an hexacanth oncosphere

the larval stage or cysticercus in the musculature and the central nervous system.

The species T. solium was described by Villanovani in 1585 and reflects the common finding of a solitary tapeworm, *T. solium* was differentiated from *T. saginata* and *Diphyllobothrium latum* by Tyson in 1683 who discovered and described the different types of heads or scolices of tapeworms; Redi published the following year illustrations of the scolex of dog and cat tapeworms. Van Beneden 170 years later stated that a tapeworm was an animal formed by multiple individuals and that cysticerci in pigs develop after ingesting *T. solium* segments. At that time Kuchenmeister, in spte of severe ethical criticisms, identified that humans are the hosts of the adult stage of *T. solium*. In two occasions (1854 and 1859) he fed death convicts with 63 and 40 cysticerci each, and found developing tapeworms in the small intestine after necropsy. Interestingly the number of tapeworms found was 10 and 20 respectively and, since the second prisoner ingested the bladder worms much before death than the first convict (four months vs 4 days) 11 worms had mature proglottids and the largest reached five feet (1.5 meters) in length. This finding further demonstrates that *T. solium* is not necessarily a solitary parasite. He stated that the sheer number of tapeworms produced ought to convince even the most skeptical that they were derived from the cysticerci that the convicts ingested. Nevertheless he recommended that the experiment of bladder worm feeding should be allowed to be repetead on criminals under death sentences and, in the case of subsequent pardon, the tapeworms could be easily expelled, calming anxious souls and serving science at the same time [4]. Figure 3 shows pictures of some of the scientists that made outstanding contributions to the understanding of the life cycle of T. solium: Malpighi, Tyson, van Beneden and Kuchenmeister. Not until last century the precise knowledge of the complete anatomy of a tapeworm, as well as its organization and individuality were defined, considering it a single animal.

Rumler was the first clinician that described a case of human cysticercosis in 1558 but considered it to be a tumor in the dura matter of a person with epilepsy. Panarolus, one hundred years later, reported similar cysts in the corpus callosum of the brain of an epileptic priest and Wharton found many cysticerci, that he considered to be glands in the adipose and muscle tissues of a soldier. The disease was not clearly identified as parasitic until

Malpighi in 1698 discovered the animal nature of these cysts and described their scolex. Goeze, not knowing this study, examined swine cysticerci and described their helminthic nature. The taxonomic clasification of "Cysticercus cellulosae" was given by Zeder and Rudoplphi [4]. The use of a scientific name for this parasite was abolished when it was demonstrated that cysticerci are larval stages of *T. solium*. Therefore, when mentioning cysticerci it should only be stated "celluosae type of *T. solium* cysticerci" or simply "*T. solium* cysticerci". Yoshino published detailed histological descriptions of the development of cysticerci, including the formation of the scolex and the size and appearance of its hooks, after he fed proglottids to pigs, released by him because he ingested cysticerci [5-8] and Rabiela demonstrated that there are intermediate forms between a cellulose and a recemose type cysticercus and that cysticerci evaginate through a pore. (Figure 4) [9, 10].

Epidemiology of Neurocysticercosis in Mexico: From a Public Health Problem to Its Control 259

**Figure 4.** Micrographs of the evagination pore of a cysticercus and an evaginated cysticercus as well as a photograph of a bilobulated cysticercus; its appearance suggests the beginning of transformation from

The first report regarding NCC in Mexico, probably lost in the old Mexican literature, was published in 1901; the author, Dr. Ignacio Gómez-Izquierdo, described a patient from Cuba who died in a psichiatric asylum with diagnosis of alcoholism or tuberculosis. During necropsy multiple cysticerci were found. The author stated his doubts regarding the diease: "Diagnosis is almost impossible, because, with the exception of those cases in which

a cellulosae to a racemos type cysticercus. Kindly donated by Dr. Maria Teresa Rabiela (RIP)

**Figure 3.** Photograps of M. Malpighi, E. Tyson, PJ van Beneden and F Kuchenmeister

Epidemiology of Neurocysticercosis in Mexico: From a Public Health Problem to Its Control 259

258 Novel Aspects on Cysticercosis and Neurocysticercosis

Malpighi in 1698 discovered the animal nature of these cysts and described their scolex. Goeze, not knowing this study, examined swine cysticerci and described their helminthic nature. The taxonomic clasification of "Cysticercus cellulosae" was given by Zeder and Rudoplphi [4]. The use of a scientific name for this parasite was abolished when it was demonstrated that cysticerci are larval stages of *T. solium*. Therefore, when mentioning cysticerci it should only be stated "celluosae type of *T. solium* cysticerci" or simply "*T. solium* cysticerci". Yoshino published detailed histological descriptions of the development of cysticerci, including the formation of the scolex and the size and appearance of its hooks, after he fed proglottids to pigs, released by him because he ingested cysticerci [5-8] and Rabiela demonstrated that there are intermediate forms between a cellulose and a recemose

type cysticercus and that cysticerci evaginate through a pore. (Figure 4) [9, 10].

**Figure 3.** Photograps of M. Malpighi, E. Tyson, PJ van Beneden and F Kuchenmeister

**Figure 4.** Micrographs of the evagination pore of a cysticercus and an evaginated cysticercus as well as a photograph of a bilobulated cysticercus; its appearance suggests the beginning of transformation from a cellulosae to a racemos type cysticercus. Kindly donated by Dr. Maria Teresa Rabiela (RIP)

The first report regarding NCC in Mexico, probably lost in the old Mexican literature, was published in 1901; the author, Dr. Ignacio Gómez-Izquierdo, described a patient from Cuba who died in a psichiatric asylum with diagnosis of alcoholism or tuberculosis. During necropsy multiple cysticerci were found. The author stated his doubts regarding the diease: "Diagnosis is almost impossible, because, with the exception of those cases in which cysticerci are found in superficial tissues or in the eye, symptomatology by itself does not provide enough information in order to establish its diagnosis, and, if diagnosis could have been done with precision, would prognosis stop to be fatal? Are there medical or surgical treatments to successfully fight this disease? Our answer, sadly to say, is negative" [11]. The questions posed by Dr. Gómez-Izquierdo 110 years ago have illuminated the path of knowledge and reflect major advances in the last 30 years because NCC is diagnosed and treated with high efficiency and its fatality rate has dramatically diminished [12,13].

Epidemiology of Neurocysticercosis in Mexico: From a Public Health Problem to Its Control 261

Because human cysticercosis is a disease related to underdevelopment, it is present in countries that lack proper sanitary infrastructure and hygiene as well as insufficient health education [15,18,19]. An outstanding example is the emergence of neurocysticercosis in West New Guinea among the Ekari population, to whom the disease was unknown prior to the entrance of cysticercotic pigs as official gifts. Some 18-20% of the population acquired cysticercosis. The disease was detected by an epidemic of severe burns resulting from convulsions manifested while the people were sleeping around house fires; individuals also had subcutaneous nodules [20]. Between the 1940's and 1970's several reports of human necropsies performed in Latin America revealed the presence of cysticerci in the brain with different frequencies, so high that NCC is considered a public health problem in some countries [15,21,22]. As it can be seen in table 1, necropsies performed in Mexico reported an average of 2% of NCC. In addition, hospital-based reports provided data on the magnitude of cysticercosis in patients; the National Institute of Neurology and Neurosurgery reported a prevalence of 6% among its neurological patients [23]. This prompted the development and standardization of immunodiagnostic assays, and the detection of specific serum antibodies facilitated the shift to epidemiological information instead of that coming from autopsies, because these do not strictly represent the Mexican population since they were performed in people dying in public government hospitals, while seroepidemiological surveys are

performed in open populations including both rural and urban settings [24].

**Table 1.** Frequency of neurocysticercosis in necropsies

Country Years reported % Peru 1961-1974 5.9 Mexico 1947-1957 2.8 Brazil 1960-1979 2.4 Brazil 1965-1970 2.2 Mexico 1963-1974 2.2 Brazil 1992-1997 1.5 Mexico 1963-1973 1.5 Mexico 1953-1970 1.3 Peru 1961-1974 0.99 Colombia 1944-1964 0.78 Chile 1939-1966 0.70 Venezuela 1967 0.49 Ecuador 1947-1968 0.47 Costa Rica 1967 0.45 Colombia 1955-1970 0.40 El Salvador 1961 0.40 Chile 1947-1979 0.09 Honduras 1951-1966 0.02

## **2. Epidemiology**

Human cysticercosis was considered in the past to be less frequent than taeniosis due to the possibility of finding a tapeworm in feces, while muscle cysticerci mostly do not cause clinical signs and neurocysticercosis generally could not be identified due to its pleomorphic symtomatology [14,15]. Interest in NCC arouse after Dixon and Lipscomb identified soldiers that acquired NCC when they were were stated in India [16]. This study is the only one that allowed identifying the duration of the disease and of the appearance of clinical manifestations, since it became clear when soldiers acquired NCC while stationed in India. Figure 5 shows a histogram of the time when soldiers presented the first convulsive crisis and/or subcutaneous nodules; as it can be seen in around 50% symptoms appeared two to four years after being in India, and cases were also identified as far as 30 years afterwards. MacArthur suggested that symptomatology was due to the death of parasites and suggested that the biological objetive of cysticerci, while in the tissues of the intermediate host, is to remain silent in order to be able to continue their life cycle. He also suggested that parasite death could be associated to toxin release that increases irritation [17].

**Figure 5.** Histogram showing the interval in years in which signs and symptoms related to cysticercosis appearaed in 450 soldiers after they returned from being stationed in India. Convulsive crisis (///), subcutaneous nodules (\\\), both (black) or other data (white), reproduced from [16].

Because human cysticercosis is a disease related to underdevelopment, it is present in countries that lack proper sanitary infrastructure and hygiene as well as insufficient health education [15,18,19]. An outstanding example is the emergence of neurocysticercosis in West New Guinea among the Ekari population, to whom the disease was unknown prior to the entrance of cysticercotic pigs as official gifts. Some 18-20% of the population acquired cysticercosis. The disease was detected by an epidemic of severe burns resulting from convulsions manifested while the people were sleeping around house fires; individuals also had subcutaneous nodules [20]. Between the 1940's and 1970's several reports of human necropsies performed in Latin America revealed the presence of cysticerci in the brain with different frequencies, so high that NCC is considered a public health problem in some countries [15,21,22]. As it can be seen in table 1, necropsies performed in Mexico reported an average of 2% of NCC. In addition, hospital-based reports provided data on the magnitude of cysticercosis in patients; the National Institute of Neurology and Neurosurgery reported a prevalence of 6% among its neurological patients [23]. This prompted the development and standardization of immunodiagnostic assays, and the detection of specific serum antibodies facilitated the shift to epidemiological information instead of that coming from autopsies, because these do not strictly represent the Mexican population since they were performed in people dying in public government hospitals, while seroepidemiological surveys are performed in open populations including both rural and urban settings [24].


**Table 1.** Frequency of neurocysticercosis in necropsies

260 Novel Aspects on Cysticercosis and Neurocysticercosis

**2. Epidemiology** 

cysticerci are found in superficial tissues or in the eye, symptomatology by itself does not provide enough information in order to establish its diagnosis, and, if diagnosis could have been done with precision, would prognosis stop to be fatal? Are there medical or surgical treatments to successfully fight this disease? Our answer, sadly to say, is negative" [11]. The questions posed by Dr. Gómez-Izquierdo 110 years ago have illuminated the path of knowledge and reflect major advances in the last 30 years because NCC is diagnosed and

Human cysticercosis was considered in the past to be less frequent than taeniosis due to the possibility of finding a tapeworm in feces, while muscle cysticerci mostly do not cause clinical signs and neurocysticercosis generally could not be identified due to its pleomorphic symtomatology [14,15]. Interest in NCC arouse after Dixon and Lipscomb identified soldiers that acquired NCC when they were were stated in India [16]. This study is the only one that allowed identifying the duration of the disease and of the appearance of clinical manifestations, since it became clear when soldiers acquired NCC while stationed in India. Figure 5 shows a histogram of the time when soldiers presented the first convulsive crisis and/or subcutaneous nodules; as it can be seen in around 50% symptoms appeared two to four years after being in India, and cases were also identified as far as 30 years afterwards. MacArthur suggested that symptomatology was due to the death of parasites and suggested that the biological objetive of cysticerci, while in the tissues of the intermediate host, is to remain silent in order to be able to continue their life cycle. He also suggested that parasite

**Figure 5.** Histogram showing the interval in years in which signs and symptoms related to cysticercosis appearaed in 450 soldiers after they returned from being stationed in India. Convulsive crisis (///),

subcutaneous nodules (\\\), both (black) or other data (white), reproduced from [16].

treated with high efficiency and its fatality rate has dramatically diminished [12,13].

death could be associated to toxin release that increases irritation [17].

For the first seroepidemiologic studies reported in Mexico, immunoelectrophoresis was used, initially in communities in the state of Chiapas: 1610 samples obtained from 9 communities, mostly rural, were analyzed. Interestingly, in spite of the low sensitivity of immunoelectrophoresis (50%), its high specificity (100% because practically no echinococcosis is found in Mexico) allowed to identify a clear correlation: communities with less than 4000 inhabitants had 1-8% seropositivity, while populations with more inhabitants (35,000 was the biggest) had 1% or less antibody frequency; indicating that the parasite was more prevalent in small, an thus less developed, towns [25]. Afterwards, using 20,000 samples from a national survey, a central area in Mexico, in which people had between 0.6 and 1% of anti-cysticercus antibodies, was identified; remarkably this geographic area, called "El Bajio", is the most important pig breeding area for national consumption of pork meat [26]. A few years later, in El Sotano, a small community in the state of Hidalgo, 6% of the 124 inhabitants had antibodies detected by ELISA, 25% of their pigs had cysticerci that were palpated in their tongues and 3% of the people had *Taenia* eggs. *Ascaris, Trichuris* and *Toxocara* eggs were found in soil samples but no *Taenia* eggs were identified in soil. The clustered distribution of infected pigs, tapeworm carriers and people with serologic or clinical evidence of cysticercosis suggested intra-household transmission. Furthermore, although the correlation of seropositivity and clinical history suggestive of NCC in individual residents was poor, there was an apparent spatial association between tapeworm carriers and persons with serologic or clinical histories associated to NCC [27]. This information was very interesting because it identified, for the first time ever, the main risk factor for NCC: the presence of a person infected with an adult tapeworm at home (which I consider my most important contribution to the control of NCC), instead of egg transmission through strawberries, lettuce, coriander and any other vegetable grown at floor level, eaten unpeeled, uncooked and probably unwashed, that could have been irrigated with sewage.

Epidemiology of Neurocysticercosis in Mexico: From a Public Health Problem to Its Control 263

means of hygienic and sanitary measures. The results obtained support that the prevalence of human cysticercosis in Mexico was, as indicated by previous necropsy findings, around 2%, while the prevalence of antibodies was much higher, suggesting exposure to the parasite but not current infection. In another national survey, using indirect hemaglutination, a similar antibody prevalence was found (15% of the 11,611 homes analyzed), and 2.2% had two or more seropositive members, pointing to the presence of an

Many epidemiological surveys were performed with ELISA in the 1980´s, supported by direct detection of cysticerci in pigs' tongue and tapeworm eggs in human feces [27, 28, 30- 34] and, in the 1990's, by western blot (WB) [27, 30, 35-41] using an enriched fraction of glycoproteins as antigen source [42] allowing the demonstration of the presence of all the components of the life cycle (Tables 2 and 3), [24]). Table 3 also shows that swine cysticercosis is higher in Peru, suggesting that Peruvian pigs have easier access to human feces. Furthermore, one survey was performed in two rural communities of Mexico in order to compare the performance of both assays; 2524 individuals were studied, 7.5% were positive by WB, 2.1% by ELISA and only 3.1% in both assays [43]. Reports of predictive values indicate that for screening purposes, WB would be the test of choice, and for diagnosis, performing WB and CT tests would yield the best results, although with clinical

data suggestive of NCC even ELISA is useful to support diagnosis [24].

**Year Number of** 

**samples** 

El Sótano, Hidalgo 1984 124 24 3.1 6 San Pedro Martir, DF 1985 928 0 0 0 El Salado, Sinaloa 1986 432 Present 1.2 12 Los Sauces, Guerrero 1987 440 6.6 3.0 2.3 La Curva, Sinaloa 1989 549 1.4 1.3 11 **Table 2.** Epidemiologic studies performed with ELISA for detection of people with anti-cysticercus

When data from table 1 are compared to those of tables 2 and 3, it is evident that the prevalence of anti-cysticercus antibodies in humans (2.3-24%) is much higher than the finding of the parasite in pathology studies (0.02-5.9%), indicating that antibodies reflect exposure and not necessary the presence of the parasite; its serologic confirmation can be obtained by detecting cysticercal antigens. One survey used monoclonal antibodies in a capture ELISA [44,45] in 900 inhabitants of the community of Cerritos, San Luis Potosí, 1% positive samples for antigens and 4.2% for antibodies were found, only one sample was positive in both tests. Interestingly, although antigen detection was lower, two of the three positive cases that accepted undergoing computed tomography (CT) had images compatible with cysticerci, while only two of the seven antibody positive individuals had CT images resembling cysticerci [46]. These data point to a higher correlation with the disease in open populations when parasite antigens are being searched than when antibodies are detected.

**% pigs with tongue cysticerci** 

**% people with eggs in feces** 

**% people positive in ELISA** 

intestinal tapeworm carrier [29].

**Community studied, state** 

antibodies

In a study undertaken in order to determine markers of *T. solium* transmission and risk factors in an urban community, 1000 soldiers from a military camp in Mexico City and their families were studied. Serum samples were used to detect cysticercus antigens and antibodies and fecal specimens were examined for *Taenia* coproantigens (CpAgs) and eggs. Antibodies were detected in 12.2% of soldiers and 5.8% and 10% of relatives of positive and negative soldiers, respectively. Antigens in serum were detected in 2.8% of the soldiers and in 4.2% of the relatives of antibody-positive soldiers. CpAgs were found in 0.5% and *Taenia* eggs in 0.1% of soldiers but were not found in their families. Interestingly, 12% of the family members of positive soldiers had had a history of proglottid release, compared to only 3.7% of the family members of negative soldiers. Lastly, 86% of the family members of positive soldiers had eaten in street food stores, compared to only 62.5% of those of negative soldiers. Both risk factors identified were statistically significant, indicating again, that the main risk factor was an association with the presence of a tapeworm carrier at home confirming its importance for the transmission of *T. solium* [28]. Therefore, a clinical history of taeniosis in a family member, defined as elimination of proglottids in feces, should be taken into account by health personnel in order to be treated in spite of being asymptomatic, and to prevent other members of the family from becoming infected. Likewise, public health authorities should control street food vendors to reduce new cases of *T. solium* infections by means of hygienic and sanitary measures. The results obtained support that the prevalence of human cysticercosis in Mexico was, as indicated by previous necropsy findings, around 2%, while the prevalence of antibodies was much higher, suggesting exposure to the parasite but not current infection. In another national survey, using indirect hemaglutination, a similar antibody prevalence was found (15% of the 11,611 homes analyzed), and 2.2% had two or more seropositive members, pointing to the presence of an intestinal tapeworm carrier [29].

262 Novel Aspects on Cysticercosis and Neurocysticercosis

For the first seroepidemiologic studies reported in Mexico, immunoelectrophoresis was used, initially in communities in the state of Chiapas: 1610 samples obtained from 9 communities, mostly rural, were analyzed. Interestingly, in spite of the low sensitivity of immunoelectrophoresis (50%), its high specificity (100% because practically no echinococcosis is found in Mexico) allowed to identify a clear correlation: communities with less than 4000 inhabitants had 1-8% seropositivity, while populations with more inhabitants (35,000 was the biggest) had 1% or less antibody frequency; indicating that the parasite was more prevalent in small, an thus less developed, towns [25]. Afterwards, using 20,000 samples from a national survey, a central area in Mexico, in which people had between 0.6 and 1% of anti-cysticercus antibodies, was identified; remarkably this geographic area, called "El Bajio", is the most important pig breeding area for national consumption of pork meat [26]. A few years later, in El Sotano, a small community in the state of Hidalgo, 6% of the 124 inhabitants had antibodies detected by ELISA, 25% of their pigs had cysticerci that were palpated in their tongues and 3% of the people had *Taenia* eggs. *Ascaris, Trichuris* and *Toxocara* eggs were found in soil samples but no *Taenia* eggs were identified in soil. The clustered distribution of infected pigs, tapeworm carriers and people with serologic or clinical evidence of cysticercosis suggested intra-household transmission. Furthermore, although the correlation of seropositivity and clinical history suggestive of NCC in individual residents was poor, there was an apparent spatial association between tapeworm carriers and persons with serologic or clinical histories associated to NCC [27]. This information was very interesting because it identified, for the first time ever, the main risk factor for NCC: the presence of a person infected with an adult tapeworm at home (which I consider my most important contribution to the control of NCC), instead of egg transmission through strawberries, lettuce, coriander and any other vegetable grown at floor level, eaten unpeeled,

uncooked and probably unwashed, that could have been irrigated with sewage.

In a study undertaken in order to determine markers of *T. solium* transmission and risk factors in an urban community, 1000 soldiers from a military camp in Mexico City and their families were studied. Serum samples were used to detect cysticercus antigens and antibodies and fecal specimens were examined for *Taenia* coproantigens (CpAgs) and eggs. Antibodies were detected in 12.2% of soldiers and 5.8% and 10% of relatives of positive and negative soldiers, respectively. Antigens in serum were detected in 2.8% of the soldiers and in 4.2% of the relatives of antibody-positive soldiers. CpAgs were found in 0.5% and *Taenia* eggs in 0.1% of soldiers but were not found in their families. Interestingly, 12% of the family members of positive soldiers had had a history of proglottid release, compared to only 3.7% of the family members of negative soldiers. Lastly, 86% of the family members of positive soldiers had eaten in street food stores, compared to only 62.5% of those of negative soldiers. Both risk factors identified were statistically significant, indicating again, that the main risk factor was an association with the presence of a tapeworm carrier at home confirming its importance for the transmission of *T. solium* [28]. Therefore, a clinical history of taeniosis in a family member, defined as elimination of proglottids in feces, should be taken into account by health personnel in order to be treated in spite of being asymptomatic, and to prevent other members of the family from becoming infected. Likewise, public health authorities should control street food vendors to reduce new cases of *T. solium* infections by Many epidemiological surveys were performed with ELISA in the 1980´s, supported by direct detection of cysticerci in pigs' tongue and tapeworm eggs in human feces [27, 28, 30- 34] and, in the 1990's, by western blot (WB) [27, 30, 35-41] using an enriched fraction of glycoproteins as antigen source [42] allowing the demonstration of the presence of all the components of the life cycle (Tables 2 and 3), [24]). Table 3 also shows that swine cysticercosis is higher in Peru, suggesting that Peruvian pigs have easier access to human feces. Furthermore, one survey was performed in two rural communities of Mexico in order to compare the performance of both assays; 2524 individuals were studied, 7.5% were positive by WB, 2.1% by ELISA and only 3.1% in both assays [43]. Reports of predictive values indicate that for screening purposes, WB would be the test of choice, and for diagnosis, performing WB and CT tests would yield the best results, although with clinical data suggestive of NCC even ELISA is useful to support diagnosis [24].


**Table 2.** Epidemiologic studies performed with ELISA for detection of people with anti-cysticercus antibodies

When data from table 1 are compared to those of tables 2 and 3, it is evident that the prevalence of anti-cysticercus antibodies in humans (2.3-24%) is much higher than the finding of the parasite in pathology studies (0.02-5.9%), indicating that antibodies reflect exposure and not necessary the presence of the parasite; its serologic confirmation can be obtained by detecting cysticercal antigens. One survey used monoclonal antibodies in a capture ELISA [44,45] in 900 inhabitants of the community of Cerritos, San Luis Potosí, 1% positive samples for antigens and 4.2% for antibodies were found, only one sample was positive in both tests. Interestingly, although antigen detection was lower, two of the three positive cases that accepted undergoing computed tomography (CT) had images compatible with cysticerci, while only two of the seven antibody positive individuals had CT images resembling cysticerci [46]. These data point to a higher correlation with the disease in open populations when parasite antigens are being searched than when antibodies are detected.

Another field study showed and association between the presence of antigens and that of late onset epilepsy, while antibodies were associated to the presence of subcutaneous nodules; furthermore specificity and positive predictive values for the antigen capture ELISA was high with samples of epileptic people [47]. It has also been demostrated that this capture ELISA is useful to evaluate symtomaticos patients, who can benefit with immediate treatment [48]. Similar studies have been implememented in the last decade in Asia and Africa.

Epidemiology of Neurocysticercosis in Mexico: From a Public Health Problem to Its Control 265

and cheaper to treat tapeworm carriers than to modify sewage and irrigation in developing

A clear asociation exists between the presence of taeniosis and the severity of NCC, therefore the perception that *T. solium* tapeworms are silent guests causing no harm to humans is erroneous, and tapeworm carriers should be regarded as potential sources of contagion to both themselves and to those living in their close environment [53]. Just how important is the presence of a tapeworm carrier was demonstrated by the case of 4 orthodox Jewish families from New York, in which 4 neurological cases and 7 seropositive people were along two years after. Although these families did not ingest pork meat, the maid who cooked

The information presented above clearly demonstrates a clustered distribution of persons with serological or clinical evidence of cysticercosis, infected pigs and tapeworm carriers, thus allowing to evaluate intervention measures: health education, self-detection of tapeworm carriers, mass treatment against human taeniosis and pigs vaccination. Regarding health education as a community-based intervention measure, a comprehensive study was undertaken in Chalcatzingo, Morelos with approximately 2000 inhabitants. An educational program was developed to identify the local knowledge of both diseases (taeniosis and cysticercosis) and of both parasites (the tapeworm and the cysticercus), in order to promote recognition of the parasites and knowledge of the transmission, and to improve hygienic behavior and sanitary conditions that foster transmission. This was performed by in-depth questionnaires developed by anthropologists. Based on the information obtained, an educational intervention was developed which included explanation of the life cycle, diseases, risk factors and control measures. For this purpose the anthropologists trained local leaders, selected among students, housewives, and teachers as well as the priest, to be in charge of promoting and providing health education, so that education remained in the community after the project ended. The effects of this educational intervention were evaluated by measuring changes in knowledge, attitudes and practices (Table 4) and prevalences of human taeniosis and swine cysticercosis before and after the campaign [56]. The prevalence in pigs at the start of the education intervention was 2.6% and 5.2% by tongue examination and western blot for antibody detection, respectively. Approximately one year after the intervention they were 0% and 1.2%, respectively, and remained so for

Apparently an economic factor facilitated the success, since people learned that by having pigs restrained in certain areas without access to human feces or garbage they would not acquire the disease and thus could be sold at a higher price. In Coapeche, Veracruz, where swine cysticercosis was ascertained by western blot, none of the 53 pigs studied had antibodies or cysticerci. Latrines were present in 91% of houses and pigs were kept in restrained areas, demonstrating that adequate basic sanitary conditions and pig breeding practices are effective and practical to control *T. solium* in rural communities [58]. High standards of meat inspection and proper disposal of infected pig carcasses will also aid in preventing infected pigs from

for these families was from Mexico and she had an intestinal *T solium* [54].

countries with huge territories.

almost 4 years. (Figure 6) [57].

entering the food chain.


**Table 3.** Epidemiologic studies performed with western blot for detection of people with anticysticercus antibodies

## **3. Risk factors and intervention trials**

It has been considered for a long time in Mexico that fruits, such as strawberries, and vegetables, such as lettuce, that are eaten unpeeled and uncooked and that grow at ground level, are the main sources of *Taenia* eggs that cause human NCC. But in a study carried out in the counties of Irapuato in the state of Guanajuato and Zamora, Michoacan, which are important locations for the production of strawberries, no *Taenia* eggs were identified in large amounts of homogenized strawberries collected throughout one year but low numbers of protozoan cysts and one *Ascaris* egg were found [49]. This indicated that, although there was contamination with human feces, strawberries did not carry tapeworm eggs. Also, the absence of *Taenia* eggs in domestic flies was demonstrated in the community of Tianquizolco, where over one thousand flies were caught in homes and assessed for their role in the transmission of *Taenia solium* [50]. On the other hand, multiple studies have demonstrated that the prevalence of tapeworm carriers is higher among household members of NCC patients than in the rest of the population [27,51, 52]. For transmission of NCC the importance of a tapeworm carrier in the household and not of egg ingestion in strawberries or of contaminated flies changes the concept of control, since it is much easier and cheaper to treat tapeworm carriers than to modify sewage and irrigation in developing countries with huge territories.

264 Novel Aspects on Cysticercosis and Neurocysticercosis

Africa.

**Community studied, state** 

cysticercus antibodies

**3. Risk factors and intervention trials** 

Another field study showed and association between the presence of antigens and that of late onset epilepsy, while antibodies were associated to the presence of subcutaneous nodules; furthermore specificity and positive predictive values for the antigen capture ELISA was high with samples of epileptic people [47]. It has also been demostrated that this capture ELISA is useful to evaluate symtomaticos patients, who can benefit with immediate treatment [48]. Similar studies have been implememented in the last decade in Asia and

**Year Number of** 

**samples** 

Xoxocotla, México 1988 13227 4 0.3 11 Angahuan, México 1988 3065 6.5 0.5 5 Churusapa, Perú 1988 279 49 1 7 Maceda, Perú 1988 421 43 1 8 Haparquilla, Perú 1990 365 46 ND 13 Jocote, Guatemala 1991 1161 14 3 17 Quesada, Guatemala 1991 1204 4 1 10 Saylla, Perú 1990-3 501 36 3 24 San Pablo, Ecuador 1992 2723 ND ND 10 Tegucigalpa, Honduras 1998 404 ND 0.6 16 Salama, Honduras 1999 480 ND 2.5 17 Cd. de México, México 1999 1000 ND 0.5 12 **Table 3.** Epidemiologic studies performed with western blot for detection of people with anti-

It has been considered for a long time in Mexico that fruits, such as strawberries, and vegetables, such as lettuce, that are eaten unpeeled and uncooked and that grow at ground level, are the main sources of *Taenia* eggs that cause human NCC. But in a study carried out in the counties of Irapuato in the state of Guanajuato and Zamora, Michoacan, which are important locations for the production of strawberries, no *Taenia* eggs were identified in large amounts of homogenized strawberries collected throughout one year but low numbers of protozoan cysts and one *Ascaris* egg were found [49]. This indicated that, although there was contamination with human feces, strawberries did not carry tapeworm eggs. Also, the absence of *Taenia* eggs in domestic flies was demonstrated in the community of Tianquizolco, where over one thousand flies were caught in homes and assessed for their role in the transmission of *Taenia solium* [50]. On the other hand, multiple studies have demonstrated that the prevalence of tapeworm carriers is higher among household members of NCC patients than in the rest of the population [27,51, 52]. For transmission of NCC the importance of a tapeworm carrier in the household and not of egg ingestion in strawberries or of contaminated flies changes the concept of control, since it is much easier

**% pigs with tongue cysticerci** 

**% people with eggs in feces** 

**% people positive in WB** 

A clear asociation exists between the presence of taeniosis and the severity of NCC, therefore the perception that *T. solium* tapeworms are silent guests causing no harm to humans is erroneous, and tapeworm carriers should be regarded as potential sources of contagion to both themselves and to those living in their close environment [53]. Just how important is the presence of a tapeworm carrier was demonstrated by the case of 4 orthodox Jewish families from New York, in which 4 neurological cases and 7 seropositive people were along two years after. Although these families did not ingest pork meat, the maid who cooked for these families was from Mexico and she had an intestinal *T solium* [54].

The information presented above clearly demonstrates a clustered distribution of persons with serological or clinical evidence of cysticercosis, infected pigs and tapeworm carriers, thus allowing to evaluate intervention measures: health education, self-detection of tapeworm carriers, mass treatment against human taeniosis and pigs vaccination. Regarding health education as a community-based intervention measure, a comprehensive study was undertaken in Chalcatzingo, Morelos with approximately 2000 inhabitants. An educational program was developed to identify the local knowledge of both diseases (taeniosis and cysticercosis) and of both parasites (the tapeworm and the cysticercus), in order to promote recognition of the parasites and knowledge of the transmission, and to improve hygienic behavior and sanitary conditions that foster transmission. This was performed by in-depth questionnaires developed by anthropologists. Based on the information obtained, an educational intervention was developed which included explanation of the life cycle, diseases, risk factors and control measures. For this purpose the anthropologists trained local leaders, selected among students, housewives, and teachers as well as the priest, to be in charge of promoting and providing health education, so that education remained in the community after the project ended. The effects of this educational intervention were evaluated by measuring changes in knowledge, attitudes and practices (Table 4) and prevalences of human taeniosis and swine cysticercosis before and after the campaign [56]. The prevalence in pigs at the start of the education intervention was 2.6% and 5.2% by tongue examination and western blot for antibody detection, respectively. Approximately one year after the intervention they were 0% and 1.2%, respectively, and remained so for almost 4 years. (Figure 6) [57].

Apparently an economic factor facilitated the success, since people learned that by having pigs restrained in certain areas without access to human feces or garbage they would not acquire the disease and thus could be sold at a higher price. In Coapeche, Veracruz, where swine cysticercosis was ascertained by western blot, none of the 53 pigs studied had antibodies or cysticerci. Latrines were present in 91% of houses and pigs were kept in restrained areas, demonstrating that adequate basic sanitary conditions and pig breeding practices are effective and practical to control *T. solium* in rural communities [58]. High standards of meat inspection and proper disposal of infected pig carcasses will also aid in preventing infected pigs from entering the food chain.


Epidemiology of Neurocysticercosis in Mexico: From a Public Health Problem to Its Control 267

and control measures related to infection with *Taenia solium.* Over 120 small glass bottles, each containing a few tapeworm segments fixed in formaldehyde and an instructional guide were distributed among all clinical practitioners (physicians and nurses) working in health centers. The guide contained 10 key points on how to ask questions about tapeworm infections. The small bottles were shown during questionnaire administration to all people that attended the clinic for any medical reason in order to determine if they had seen such parasites in their feces or their family. Information on taeniosis and cysticercosis was also provided to the general population via different media. Seven tapeworm carriers were recorded in the official epidemiology surveillance system the year previous to the study, interestingly, the year after the study, 41 tapeworm carriers (37 *T. saginata*; 4 *T. solium*) were recorded. Thus six times more tapeworm carriers were notified after the study. All four persons with *T. solium* were treated, thereby eliminating the parasite and subsequently preventing new cases of human and swine cysticercosis that might have arisen [59]. This

study demonstrates that self-detection is a feasible tool for control of *T. solium.*

eggs, as well as by association with late onset epilepsy.

The use of mass treatment with praziquantel to eliminate tapeworms from human carriers as a community-based intervention measure was evaluated in two studies. In a small community (559 inhabitants) in La Curva, Sinaloa, over 70% of the population over 5 years of age was treated with a 10 mg/kg dose. One year later, no infections with *Taenia* sp. eggs were found and no pigs with cysticercosis were detected. Seropositivity using ELISA was 11% before treatment and 7% afterwards, in the 30-39 year age group, antibody detection decreased from 30% to 7% suggesting that elimination of tapeworms reduces the possibility of contact with infective eggs. Interestingly, in the geographic section of the community where 3 of the 4 tapeworm carriers were found and treated, seropositivity was reduced from 19% to 2%, indicating that serum antibodies to *Taenia* antigens are short-lived and diminish, as contact with the parasite is lost [34]. In the second study performed in Atotonilco (3007 inhabitants), 87% of the community received a single dose of 5 mg/kg following a recommendation from WHO [60]. The prevalence of taeniosis was reduced by 53% after 6 months and by 56% after 42 months, as measured by CpAg ELISA or egg detection; late onset general seizures decreased by 70%. Anti-cysticercus antibodies in the human population were reduced by 75% after 42 months and antibodies in pigs also showed a significant reduction (55%) after 6 months [61]. In conclusion, the impact of mass chemotherapy against taeniosis to control cysticercosis in the short and long term was successfully demonstrated. Experience with praziquantel however suggested that it should not be given at doses lower than 10mg/kg. This population-based cestocidal treatment eliminated tapeworm carriers but generated symptomatology in a previously asymptomatic neurological case [62]. This observation highlights the importance of weighing targeted treatment, instead of mass drug administration, which can be used since potential tapeworm carriers are identified by direct questioning or by detection of parasite antigens or

Another alternative for the control of cysticercosis is vaccination, especially because pigs are the only intermediate hosts that participate in the maintenance of the parasite in the environment. Based on the high protection obtained with recombinant antigens identified in *T. ovis* and *T. saginata* [63, 64], recombinant vaccines against *T. solium* were elaborated. For

**Table 4.** Main effects of the intervention as per the anthropologic questionnaire

**Figure 6.** Effect of health education provided to the community of Chalcatzingo, Morelos, Mexico. Evaluation of porcine cysticercosis was measured by tongue palpation and serum antibodies detected by Western blot, while that of human taeniosis was measured by coproantigen detection. Assessment was performed before intervention (n = 1,404 for humans and 194 for pigs), 6 months later (n = 792 for humans and 165 for pigs), and 42 months later in 1996 (n = 605 for humans and 334 for pigs). Reproduced from [57].

Self-identification of tapeworm carriers as a community based intervention, alternative to health education, was evaluated in the municipality of Irapuato, Guanajuato. Clinical and animal health care practitioners and schoolteachers were trained in the life cycle, risk factors and control measures related to infection with *Taenia solium.* Over 120 small glass bottles, each containing a few tapeworm segments fixed in formaldehyde and an instructional guide were distributed among all clinical practitioners (physicians and nurses) working in health centers. The guide contained 10 key points on how to ask questions about tapeworm infections. The small bottles were shown during questionnaire administration to all people that attended the clinic for any medical reason in order to determine if they had seen such parasites in their feces or their family. Information on taeniosis and cysticercosis was also provided to the general population via different media. Seven tapeworm carriers were recorded in the official epidemiology surveillance system the year previous to the study, interestingly, the year after the study, 41 tapeworm carriers (37 *T. saginata*; 4 *T. solium*) were recorded. Thus six times more tapeworm carriers were notified after the study. All four persons with *T. solium* were treated, thereby eliminating the parasite and subsequently preventing new cases of human and swine cysticercosis that might have arisen [59]. This study demonstrates that self-detection is a feasible tool for control of *T. solium.*

266 Novel Aspects on Cysticercosis and Neurocysticercosis

**Before intervention**

Chalcatzingo (Health education) **% % % %** 

Atotonilco (Cestocidal treatment) **% % % %** 

Tetelilla (Both interventions) **% % % %** 

**Figure 6.** Effect of health education provided to the community of Chalcatzingo, Morelos, Mexico. Evaluation of porcine cysticercosis was measured by tongue palpation and serum antibodies detected by Western blot, while that of human taeniosis was measured by coproantigen detection. Assessment was performed before intervention (n = 1,404 for humans and 194 for pigs), 6 months later (n = 792 for

Self-identification of tapeworm carriers as a community based intervention, alternative to health education, was evaluated in the municipality of Irapuato, Guanajuato. Clinical and animal health care practitioners and schoolteachers were trained in the life cycle, risk factors

humans and 165 for pigs), and 42 months later in 1996 (n = 605 for humans and 334 for pigs).

**Table 4.** Main effects of the intervention as per the anthropologic questionnaire

**After intervention**

Free roming pigs **29 9 6 4**  Pigs that ingest feces **31 16 14 0**  Outdoors fecalism **49 47 44 21** 

Free roming pigs **18 16 40 3**  Pigs that ingest feces **20 22 15 3**  Outdoors fecalism **50 45 45 39** 

Free roming pigs **48 32 17 14**  Pigs that ingest feces **40 38 30 17**  Outdoors fecalism **78 70 60 52** 

**At 6 months At 42** 

**months**

**Effect detected by the anthropologic questionnaire** 

Reproduced from [57].

The use of mass treatment with praziquantel to eliminate tapeworms from human carriers as a community-based intervention measure was evaluated in two studies. In a small community (559 inhabitants) in La Curva, Sinaloa, over 70% of the population over 5 years of age was treated with a 10 mg/kg dose. One year later, no infections with *Taenia* sp. eggs were found and no pigs with cysticercosis were detected. Seropositivity using ELISA was 11% before treatment and 7% afterwards, in the 30-39 year age group, antibody detection decreased from 30% to 7% suggesting that elimination of tapeworms reduces the possibility of contact with infective eggs. Interestingly, in the geographic section of the community where 3 of the 4 tapeworm carriers were found and treated, seropositivity was reduced from 19% to 2%, indicating that serum antibodies to *Taenia* antigens are short-lived and diminish, as contact with the parasite is lost [34]. In the second study performed in Atotonilco (3007 inhabitants), 87% of the community received a single dose of 5 mg/kg following a recommendation from WHO [60]. The prevalence of taeniosis was reduced by 53% after 6 months and by 56% after 42 months, as measured by CpAg ELISA or egg detection; late onset general seizures decreased by 70%. Anti-cysticercus antibodies in the human population were reduced by 75% after 42 months and antibodies in pigs also showed a significant reduction (55%) after 6 months [61]. In conclusion, the impact of mass chemotherapy against taeniosis to control cysticercosis in the short and long term was successfully demonstrated. Experience with praziquantel however suggested that it should not be given at doses lower than 10mg/kg. This population-based cestocidal treatment eliminated tapeworm carriers but generated symptomatology in a previously asymptomatic neurological case [62]. This observation highlights the importance of weighing targeted treatment, instead of mass drug administration, which can be used since potential tapeworm carriers are identified by direct questioning or by detection of parasite antigens or eggs, as well as by association with late onset epilepsy.

Another alternative for the control of cysticercosis is vaccination, especially because pigs are the only intermediate hosts that participate in the maintenance of the parasite in the environment. Based on the high protection obtained with recombinant antigens identified in *T. ovis* and *T. saginata* [63, 64], recombinant vaccines against *T. solium* were elaborated. For this cDNA from a genomic library from a *T. solium* tapeworm was prepared and with probes from *T. ovis* 18k and 45k antigens, the homologue recombinant DNAs were identified and cloned in *E. coli*. The oncosphere recombinant antigens were purified and used to vaccinate pigs in two independent trials performed in different institutions under controlled conditions. The first one was in 2000 and the second one in 2002. Each pig received 200ug of recombinant antigen TSOL18 or TSOL45 or the carrier, Glutathione S Transferase (GST) as a control group, with 1mg Quil A as adjuvant, intramuscularly each 15 days, in two occasions in the first experiment and thrice in the second one. For challenge, gravid proglottids were separated from a tapeworm recovered from a carrier. Proglottids were minced; eggs were obtained by sieving, washed, counted and used to challenge pigs 3 weeks after the 2nd or 3rd immunization, respectively. Each pig received orally 40,000 eggs in the first experiment, and 9,000 in the second one. Necropsies were performed 3-4 months after challenge infection, carcass musculature was sliced with hand-held knives or scalpels, and the number and viability of cysticerci were determined. Table 5 shows the results of both vaccination trials. Protection was calculated as the percent reduction in the mean number of cysticerci in each group in comparison with the mean number of cysticerci in the control group; as it can be seen, in both experiments TSOL18 give practically full protection. TSOL45 gave a high value of protection in the second experiment but no protection was detected in the first one [65].

Epidemiology of Neurocysticercosis in Mexico: From a Public Health Problem to Its Control 269

Many epidemiological studies have shown a correlation between human cysticercosis, taeniosis and epilepsy and between seropositive people, infected pigs and disposal of feces and have identified community, behavioural and environmental practices that must be

Table 6 summarizes those that the author considers the most relevant. Most importantly, these studies have shown that the main risk factor is the presence of a *Taenia* carrier in the immediate environment. A proposal to declare neurocysticercosis an international

This proposal, if taken in account, could be helpful in the control of cysticercosis, since, if cases of cysticercosis and of taeniosis are reported in all countries, it will provide accurate quantification of the incidence and prevalence of neurocysticercosis at regional level, thus permitting the rational use of resources in eradication campaigns. Although NCC is endemic in several countries of Latin America, Sub-Saharan Africa and Asia [69], due to migration there are many patients with NCC that attend hospitals in several cities of the USA and, even more important is the fact that tapeworm carriers have been identified in USA and even in Muslim countries, therefore now cysticercosis is considered an emerging infectious disease in USA and in some of its states it is now a reportable disease. [19,70,71]. It should be noted that approximately 200 million people cross the Mexico-USA border each

year, multiplying the opportunities of acquiring and transporting adult *T. solium* [72].

If outdoor fecalism is necessary, cover, bury or burn the feces

 Cook sufficiently pork meat to kill possible parasites Freeze pork meat at least 5 days before cooking

 Feed pigs with tortillas, bread and food leftovers Avoid access of pigs to letrines, feces and garbage

Do not sell or buy pork meat with cysticerci

After defecating check feces in search of a tapeworm

If you have a tapeworm ask for treatment

 Wash hands before eating and cooking Do not eat contaminated pork meat

Wash hands after defecating

Avoid having outdoor fecalism

Wash properly fruit and vegetables

Do not use pigpens as toilets

modified to prevent continued transmission of cysticercosis and taeniosis.

reportable disease has been published [69].

**Personal measures**

**Hygienic measures** 

**Pig breeding measures**  Avoid free roaming pigs Keep pigs in corrals

**Table 6.** Preventive measures against cysticercosis

**4. Conclusion** 


**Table 5.** Number of cysticerci found in individual pigs

The humoral immune response of all pigs was evaluated against the cysticercal enriched glycoprotein antigen used for the diagnosis of human cysticercosis [42] specific diagnostic bands were obtained in all control pigs but not in immunized pigs that did not developed cysticerci. The immune response was also evaluated against the vaccinating antigen by ELISA; specific, complement-fixing antibodies against the recombinant antigens increased after challenge and thereafter decreased; Furthermore, these and oncosphere antigens from other taeniid cestodes, contain a protein sequence motif suggesting a tertiary structure similar to the fibronectin type III domain [66]. Vaccination results show that pigs became protected from acquiring cysticercosis in experimental conditions, therefore they were evaluated in field trials in Peru [67] and Cameroon [68]; similar high levels of protection were obtained.

## **4. Conclusion**

268 Novel Aspects on Cysticercosis and Neurocysticercosis

detected in the first one [65].

Number of cysticerci in experiment 1 (infected with 40,000 eggs per pig)

Number of cysticerci in experiment 2 (infected with 9,000 eggs per pig)

GST or PBS 6, 10, 11, 13, 17, 26, 28, 40, 59, 64, 100,127

**Table 5.** Number of cysticerci found in individual pigs

GST 167, 206, 234, 262, 415 257

in Peru [67] and Cameroon [68]; similar high levels of protection were obtained.

TSOL18 0, 0, 0, 0, 0 0 100

TSOL18 0, 0, 0, 0, 1 0.2 99.5

The humoral immune response of all pigs was evaluated against the cysticercal enriched glycoprotein antigen used for the diagnosis of human cysticercosis [42] specific diagnostic bands were obtained in all control pigs but not in immunized pigs that did not developed cysticerci. The immune response was also evaluated against the vaccinating antigen by ELISA; specific, complement-fixing antibodies against the recombinant antigens increased after challenge and thereafter decreased; Furthermore, these and oncosphere antigens from other taeniid cestodes, contain a protein sequence motif suggesting a tertiary structure similar to the fibronectin type III domain [66]. Vaccination results show that pigs became protected from acquiring cysticercosis in experimental conditions, therefore they were evaluated in field trials

X Protection

X Protection

42

(%)

(%)

this cDNA from a genomic library from a *T. solium* tapeworm was prepared and with probes from *T. ovis* 18k and 45k antigens, the homologue recombinant DNAs were identified and cloned in *E. coli*. The oncosphere recombinant antigens were purified and used to vaccinate pigs in two independent trials performed in different institutions under controlled conditions. The first one was in 2000 and the second one in 2002. Each pig received 200ug of recombinant antigen TSOL18 or TSOL45 or the carrier, Glutathione S Transferase (GST) as a control group, with 1mg Quil A as adjuvant, intramuscularly each 15 days, in two occasions in the first experiment and thrice in the second one. For challenge, gravid proglottids were separated from a tapeworm recovered from a carrier. Proglottids were minced; eggs were obtained by sieving, washed, counted and used to challenge pigs 3 weeks after the 2nd or 3rd immunization, respectively. Each pig received orally 40,000 eggs in the first experiment, and 9,000 in the second one. Necropsies were performed 3-4 months after challenge infection, carcass musculature was sliced with hand-held knives or scalpels, and the number and viability of cysticerci were determined. Table 5 shows the results of both vaccination trials. Protection was calculated as the percent reduction in the mean number of cysticerci in each group in comparison with the mean number of cysticerci in the control group; as it can be seen, in both experiments TSOL18 give practically full protection. TSOL45 gave a high value of protection in the second experiment but no protection was

Many epidemiological studies have shown a correlation between human cysticercosis, taeniosis and epilepsy and between seropositive people, infected pigs and disposal of feces and have identified community, behavioural and environmental practices that must be modified to prevent continued transmission of cysticercosis and taeniosis.

Table 6 summarizes those that the author considers the most relevant. Most importantly, these studies have shown that the main risk factor is the presence of a *Taenia* carrier in the immediate environment. A proposal to declare neurocysticercosis an international reportable disease has been published [69].

This proposal, if taken in account, could be helpful in the control of cysticercosis, since, if cases of cysticercosis and of taeniosis are reported in all countries, it will provide accurate quantification of the incidence and prevalence of neurocysticercosis at regional level, thus permitting the rational use of resources in eradication campaigns. Although NCC is endemic in several countries of Latin America, Sub-Saharan Africa and Asia [69], due to migration there are many patients with NCC that attend hospitals in several cities of the USA and, even more important is the fact that tapeworm carriers have been identified in USA and even in Muslim countries, therefore now cysticercosis is considered an emerging infectious disease in USA and in some of its states it is now a reportable disease. [19,70,71]. It should be noted that approximately 200 million people cross the Mexico-USA border each year, multiplying the opportunities of acquiring and transporting adult *T. solium* [72].

#### **Personal measures**


#### **Hygienic measures**


#### **Pig breeding measures**


#### **Table 6.** Preventive measures against cysticercosis

The efforts and advancements described above, and others, drove Mexico onto a new stage regarding NCC: it is not anymore a public health problem. This parasitic disease was recognized as a public health problem when necropsy cases, published between 1947 and 1970, reported high frequencies of NCC. A recent publication [57] indicates that this disease has been controlled; the idea is supported by the dramatic decrease in the frequency of human NCC and human taeniosis obtained from the National Information System for Epidemiological Surveillance of the Ministry of Health in Mexico. The decrease was probably due to three reasons: 1) the abundant literature published by the Mexican scientific and medical communities working on cysticercosis; 2) the establishment of a National Program for the Control of *Taenia solium* since 1994; 3) the living conditions in Mexico have improved greatly in social, economy and health sectors.

Epidemiology of Neurocysticercosis in Mexico: From a Public Health Problem to Its Control 271

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756.

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## **Author details**

Ana Flisser *Department of Microbiology and Parasitology, Faculty of Medicine, National Autonomous University of Mexico (UNAM), Mexico DF* 

## **Acknowledgement**

This chapter is dedicated to all the students and collaborators that I have had along 40 years in cysticercosis research. Gustavo Barradas, Unidad de Informatica y Telecomunicaciones, Facultad de Medicina, UNAM, provided technical support in the preparation of the manuscript.

## **5. References**


[7] Yoshino K. Studies on the post-embryonal development of *Taenia solium.* Part III. On the development of cysticercus cellulosae within the definitive intermediate host*.* Journal of the Medical Association of Formosa 1933;32:166-169.

270 Novel Aspects on Cysticercosis and Neurocysticercosis

**Author details** 

**Acknowledgement** 

Ana Flisser

manuscript.

**5. References** 

Hygiene 2006;74:598-599.

1933;32:139-141

improved greatly in social, economy and health sectors.

*Department of Microbiology and Parasitology, Faculty of Medicine, National Autonomous University of Mexico (UNAM), Mexico DF* 

an anonymous translator (accessed 8 September 2012)

Journal of the Medical Association of Formosa 1933;32:155-158

The efforts and advancements described above, and others, drove Mexico onto a new stage regarding NCC: it is not anymore a public health problem. This parasitic disease was recognized as a public health problem when necropsy cases, published between 1947 and 1970, reported high frequencies of NCC. A recent publication [57] indicates that this disease has been controlled; the idea is supported by the dramatic decrease in the frequency of human NCC and human taeniosis obtained from the National Information System for Epidemiological Surveillance of the Ministry of Health in Mexico. The decrease was probably due to three reasons: 1) the abundant literature published by the Mexican scientific and medical communities working on cysticercosis; 2) the establishment of a National Program for the Control of *Taenia solium* since 1994; 3) the living conditions in Mexico have

This chapter is dedicated to all the students and collaborators that I have had along 40 years in cysticercosis research. Gustavo Barradas, Unidad de Informatica y Telecomunicaciones, Facultad de Medicina, UNAM, provided technical support in the preparation of the

[2] Bruschi F, Masetti M, Locci MT, Ciranni R, Fornaciari G. Cysticercosis in an Egyptian Mummy of the Late Ptolemaic Period. American Journal of Tropical Medicine and

[3] Athenian Society. Aristophanes. The Knights. http://ebooks.adelaide.edu.au/a/ aristophanes/knights/ The University of Adelaide Library, University of Adelaide, South Australia. The text appears to be derived from the edition published in 1912 by

[6] Yoshino K. Studies on the post-embryonal development of *Taenia solium.* Part II. On the migratory course of the oncosphere of *Taenia solium* within the intermediate host.

[4] Grove DL. A history oh human helminthology. CABI Intl Oxon UK1990. 355-383. [5] Yoshino K. Studies on the post-embryonal development of *Taenia solium.* Part I. On the hatching of the eggs of *Taenia solium.* Journal of the Medical Association of Formosa

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[38] Garcia HH, Araoz R, Gilman RH, Valdez J, González AE, Gavidia C, Bravo ML, Tsang VCW, the Cysticercosis Working Group in Perú. Increased Prevalence of Cysticercosis and Taeniasis Among Professional Fried Pork Vendors and the General Population of a Village in the Perúvian Highlands. American Journal of Tropical Medicine and Hygiene

[39] Sánchez AL, Medina MT, Ljungstrom I. Prevalence of taeniasis and cysticercosis in a

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	- [72] Flisser A, Viniegra AE, Aguilar-Vega L, Garza-Rodriguez A, Maravilla P, Avila G. Portrait of human tapeworms. Journal of Parasitology 2004;90:914–916.

**Chapter 10** 

© 2013 Carpio, licensee InTech. This is an open access chapter distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use,

distribution, and reproduction in any medium, provided the original work is properly cited.

© 2013 Carpio, licensee InTech. This is a paper distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

why the clinical spectrum of symptomatic NCC is currently poorly understood [9].

A myriad of papers have reported a wide range of diverse symptoms and signs related to NC, such as manifestations of brainstem dysfunction, cerebellar ataxia, sensory deficits,

**2. Clinical manifestations of neurocysticercosis** 

**Clinical Diagnoses of Neurocysticercosis** 

Neurocysticercosis (NCC), the most common parasitic brain disease worldwide, is endemic in countries with poor sanitation, and is increasingly being reported in developed countries due to globalization and immigration. As of many decades ago innumerable papers have been published regarding the clinical diagnosis of NC. It has been typical to affirm that clinical manifestations are extremely heterogeneous because they depend on the number, location, size, viability, or evolutionary phase of the parasite, as well as on the immunological response of the patient [1,2]. One of the most intriguing aspects of NCC is that presumably a high percentage of the individuals harboring NCC remain asymptomatic [3]; however, among the symptomatic group, the only clinical manifestation in most patients with parenchymal NCC is seizures, and their neurological status is usually normal [2]. In some patients NCC develops clinical manifestations many years after the parasite lodges in the central nervous system [4], as either brain inflammation around the parasite or mass effect. It has also been stated that almost any neurological symptomatology may be found, ranging from mild headache or treatable acute seizures to very severe neurological manifestations, such as intracranial hypertension (ICH), dementia, or even death [5-8]. These assumptions are probably true, since empirical observation has shown that many of these factors are related to a specific clinical manifestation; however, to date there is no definitive study, using appropriate methodology, designed to address the precise role of each of these factors, or the interaction among them, on the development of symptoms or signs due to NC. This is

Additional information is available at the end of the chapter

Arturo Carpio

**1. Introduction** 

http://dx.doi.org/10.5772/52388

## **Clinical Diagnoses of Neurocysticercosis**

## Arturo Carpio

276 Novel Aspects on Cysticercosis and Neurocysticercosis

[72] Flisser A, Viniegra AE, Aguilar-Vega L, Garza-Rodriguez A, Maravilla P, Avila G.

Portrait of human tapeworms. Journal of Parasitology 2004;90:914–916.

Additional information is available at the end of the chapter

http://dx.doi.org/10.5772/52388

## **1. Introduction**

Neurocysticercosis (NCC), the most common parasitic brain disease worldwide, is endemic in countries with poor sanitation, and is increasingly being reported in developed countries due to globalization and immigration. As of many decades ago innumerable papers have been published regarding the clinical diagnosis of NC. It has been typical to affirm that clinical manifestations are extremely heterogeneous because they depend on the number, location, size, viability, or evolutionary phase of the parasite, as well as on the immunological response of the patient [1,2]. One of the most intriguing aspects of NCC is that presumably a high percentage of the individuals harboring NCC remain asymptomatic [3]; however, among the symptomatic group, the only clinical manifestation in most patients with parenchymal NCC is seizures, and their neurological status is usually normal [2]. In some patients NCC develops clinical manifestations many years after the parasite lodges in the central nervous system [4], as either brain inflammation around the parasite or mass effect. It has also been stated that almost any neurological symptomatology may be found, ranging from mild headache or treatable acute seizures to very severe neurological manifestations, such as intracranial hypertension (ICH), dementia, or even death [5-8]. These assumptions are probably true, since empirical observation has shown that many of these factors are related to a specific clinical manifestation; however, to date there is no definitive study, using appropriate methodology, designed to address the precise role of each of these factors, or the interaction among them, on the development of symptoms or signs due to NC. This is why the clinical spectrum of symptomatic NCC is currently poorly understood [9].

## **2. Clinical manifestations of neurocysticercosis**

A myriad of papers have reported a wide range of diverse symptoms and signs related to NC, such as manifestations of brainstem dysfunction, cerebellar ataxia, sensory deficits,

involuntary movements, stroke-like symptoms, extrapyramidal signs, dementia, Bruns syndrome, Kluver-Bucy syndrome, cortical blindness, etc [2,10]; however, in some cases it is not possible to establish a clear cause-effect relationship between these pathologies and NC, and a fortuitous relationship among them may occur. Most of this information comes from retrospective data based on uncontrolled studies, historical case series, and anecdotal reports collected mainly from hospital neurological settings. A systematic review was conducted to estimate the clinical manifestation frequencies of symptomatic NCC [9]. The authors reviewed 1569 papers and only 21, based on minimal quality standards, were included in the final analysis. Most of the studies report clinical manifestations of NCC based on hospital medical charts, which don't present reliable information; the majority of them do not provided definitions of manifestations and, if they do, they vary from study to study. Among NCC patients seen in neurology clinics, about 79% had seizures/epilepsy, 38% severe headaches, 16% focal deficits, and 12% signs of increased intracranial pressure. Several other symptoms were also reported in less than 10% of the patients. The authors concluded that these estimates are only applicable to patients who are assisted in neurology clinics and likely over estimate the frequency of manifestations among all NCC cases [9]. Table 1 shows some representative studies which confirm the above mentioned clinical manifestations.

Clinical Diagnoses of Neurocysticercosis 279

historical cases from hospital records

historical cases from hospital records

historical cases from hospital records

questionnaire or hospital records

historical cases from hospital records

prospective cohort study, definitions

of clinical manifestations

direct

Age NCC diagnostic criteria design

CT scans: small, multiple, calcifications or cystic, contrast enhanced or not, lesions within the parenchyma

diagnostic criteria \*

CT scan (details not

All ages Del Brutto et al

provided)

All ages CT scan or MRI (details not provided)

> or multiple parenchymal,

CT scan or MRI single

intraventricular, spinal,

or subarachnoid lesions that were contrast-enhancing, cystic, or calcified

All ages CT scan or MRI: One or more active or degenerative parenchymal cysts associated or not with an extraparenchymal

location

Country (reference)

Brazil, (Forlenza OV, 1997)

Brazil (Loureiro MG et al, 1997)

Mexico (Ruiz M et al, 1997)

Mexico (Saenz B, et al, 2006)

USA (Rosenfeld EA et al, 1996)

Ecuador (Carpio A, et al. 2008)

\* Reference 31

Clinical manifestations

15%

Seizures 60 5%. depression 52% intracranial hypertension

Seizures 91%, headaches 23%, motor deficit 7%

seizures, intracranial hypertension and

learning disabilities

Seizures 80% adults, 56% children headache 35% adults, 21%, children

Seizures 87%, headaches 32%, altered mental status 13%

Seizures 62%, headaches 70%, depression 17% 178

**Table 1.** Clinical manifestations of neurocysticercosis

No. Pts.

> 18 to 59 years old

< 17 years old

1 to 15 years old

38

44

122

206

47

Clinical manifestations of NCC are determined mainly by the location of the parasite within the CNS, the evolutive phase of the parasite, and the immunological response of the patient, expressed as severity of disease activity. The clinical manifestations of parenchymal NCC are quite different from those of extraparenchymal NCC [11,12]. When the parasites are localized in the parenchyma, the main clinical manifestations are epileptic seizures or focal neurological deficits; if the parasites are localized inside the ventricles or the subarachnoid location there are signs and symptoms of cranial hypertension syndrome, cranial nerves abnormalities and hydrocephalus [12]. Regarding the parasite evolutive phase, the transitional or degenerative phase develops clinical manifestations due to the inflammatory reaction of the brain [13]. NCC predominantly affects adults in their third and fourth decade of life, and is relatively uncommon in children and the elderly [1,2] Reports of NCC are very rarely in children younger than 2 years of age because of the prolonged incubation period of *T solium.* Most often, the disease is recognized in children older than 7, due to this incubation period.

There is also clinical heterogeneity across geographical areas; most cases from the Indian subcontinent present single degenerative lesions, whereas those from Latin America present few viable cysts [14]. These differences are probably due to complex interactions between the host, parasite, and environmental factors [3]. Genetic differences in T. solium cysticerci have been reported from different countries [15,16] and may contribute towards the clinical variations among countries. A genetic susceptibility to NCC has been suggested by a reported positive association of HLA-DRBII 13 with single, contrast enhancing CT lesions [17]. However, familial aggregation of seizures in first degree relatives of NCC patients with seizures was not found [18].


\* Reference 31

278 Novel Aspects on Cysticercosis and Neurocysticercosis

manifestations.

incubation period.

seizures was not found [18].

involuntary movements, stroke-like symptoms, extrapyramidal signs, dementia, Bruns syndrome, Kluver-Bucy syndrome, cortical blindness, etc [2,10]; however, in some cases it is not possible to establish a clear cause-effect relationship between these pathologies and NC, and a fortuitous relationship among them may occur. Most of this information comes from retrospective data based on uncontrolled studies, historical case series, and anecdotal reports collected mainly from hospital neurological settings. A systematic review was conducted to estimate the clinical manifestation frequencies of symptomatic NCC [9]. The authors reviewed 1569 papers and only 21, based on minimal quality standards, were included in the final analysis. Most of the studies report clinical manifestations of NCC based on hospital medical charts, which don't present reliable information; the majority of them do not provided definitions of manifestations and, if they do, they vary from study to study. Among NCC patients seen in neurology clinics, about 79% had seizures/epilepsy, 38% severe headaches, 16% focal deficits, and 12% signs of increased intracranial pressure. Several other symptoms were also reported in less than 10% of the patients. The authors concluded that these estimates are only applicable to patients who are assisted in neurology clinics and likely over estimate the frequency of manifestations among all NCC cases [9]. Table 1 shows some representative studies which confirm the above mentioned clinical

Clinical manifestations of NCC are determined mainly by the location of the parasite within the CNS, the evolutive phase of the parasite, and the immunological response of the patient, expressed as severity of disease activity. The clinical manifestations of parenchymal NCC are quite different from those of extraparenchymal NCC [11,12]. When the parasites are localized in the parenchyma, the main clinical manifestations are epileptic seizures or focal neurological deficits; if the parasites are localized inside the ventricles or the subarachnoid location there are signs and symptoms of cranial hypertension syndrome, cranial nerves abnormalities and hydrocephalus [12]. Regarding the parasite evolutive phase, the transitional or degenerative phase develops clinical manifestations due to the inflammatory reaction of the brain [13]. NCC predominantly affects adults in their third and fourth decade of life, and is relatively uncommon in children and the elderly [1,2] Reports of NCC are very rarely in children younger than 2 years of age because of the prolonged incubation period of *T solium.* Most often, the disease is recognized in children older than 7, due to this

There is also clinical heterogeneity across geographical areas; most cases from the Indian subcontinent present single degenerative lesions, whereas those from Latin America present few viable cysts [14]. These differences are probably due to complex interactions between the host, parasite, and environmental factors [3]. Genetic differences in T. solium cysticerci have been reported from different countries [15,16] and may contribute towards the clinical variations among countries. A genetic susceptibility to NCC has been suggested by a reported positive association of HLA-DRBII 13 with single, contrast enhancing CT lesions [17]. However, familial aggregation of seizures in first degree relatives of NCC patients with

**Table 1.** Clinical manifestations of neurocysticercosis

## **3. Parenchymal neurocysticercosis**

As previously stated, the most common clinical manifestation of parenchymal NCC is epileptic seizure, which occurs in 60-90% of cases. A clinical study including 336 patients [12], reported that seizures were the most common manifestation in the parenchymal location [88%), in comparison with the extraparenchymal location [20%); similarly, focal deficits or motor abnormalities were commonest in the parenchymal location [24% vs. 14%). Focal neurological deficits, when present, are usually transient, over a period of a few days, weeks, or months, with periods of remission and relapse, most likely due to different evolutionary stages of the parasite.

Clinical Diagnoses of Neurocysticercosis 281

extraparenchymal location [88%), in comparison with the parenchymal location (10%). [12] When cysticerci are located inside the ventricular system, life-threatening acute intracranial hypertension as a result of hydrocephalus may occur; as a consequence, severe headache, dizziness and consciousness alteration are the predominant clinical manifestations [5,27].

Cysts in the subarachnoid space may lodge in the Sylvian fissure or basal cisterns and grow to a big size (racemose form), causing intracranial hypertension. This is associated with an intense inflammatory reaction, and fibrosis and progressive thickening of the leptomeninges at the base of the brain [13]. There is an obstruction of the CSF circulation, resulting in hydrocephalus and progressive intracranial hypertension. Inflammation of meninges, cranial nerve involvement, chiasmatic syndrome, and cerebral infarcts secondary to vasculitis may develop [5,6]. When hydrocephalus due to cysticercotic meningitis is present, the mortality rate is high (50%), and most patients die within 2 years after CSF shunting [6,8]. This is why ventricular and basal cisternal locations are considered to be malignant forms of NCC [28]. In cases with NCC meningitis, cerebrospinal fluid (CSF) may show mild elevation of protein, hypoglycorrhachia and lymphocytic pleocytosis, which is similar to

Spinal cord cysticercosis is rare. Patients experience nonspecific clinical manifestations, such us nerve root pain or spinal cord compression syndromes, according to the level of the lesion [29]. Severe forms of NCC may exceptionally occur, including cysticercotic encephalitis, which result in permanent neurologic sequel, such as amaurosis.

Two main techniques, the Enzyme-linked Immunosorbent Assay (Ab-ELISA) and Enzyme-Linked Immunoelectrotransfer Blot (EITB) Assay, are used for immunological diagnosis of NCC. ELISA test for antibodies or antigen detection have showed higher sensitivities and specificities in CSF than in sera [31]. EITB sensibility seems to be not significantly different in sera and CSF while specificity is higher in CSF [32]. Sensibility of either tests falls in cases of single cysts in parenchyma or when they are calcified [33]. In patients with reliable diagnosis of NCC by imaging studies, immunological test is not required, since a negative test will not discard a NCC. When imaging of extraparnechymal cysts is doubtful,

Computed tomography and magnetic resonance imaging have been useful in the study of the parasite evolution within the brain parenchyma [2]. Once the oncosphere has passed into the parenchyma, it grows and evolves through vesicular, colloidal, granular-nodular, and calcified phases [13]. In the vesicular phase the larva lives inside a translucent liquidfilled cystic structure surrounded by a thin membrane. The CT scan depicts circumscribed, rounded, hypodense areas, varying in size and number, without enhancement by contrast media [34]. With the MRI, the vesicular larva appears with a CSF-like intensity signal on all sequences. Both MRI and CT may show a high intensity, 2-3 mm. mural nodule, depicting the scolex, in the interior of some vesicular cysts (Figure 1). It is better seen on fluid

other chronic basal meningitis, such as tuberculosis and mycosis.[2]

**5. Immunological and imaging diagnosis** 

determination of antigens by ELISA in CSF may be helpful [31].

attenuated inversion recovery (FLAIR) sequence [35].

Hydrocephalus and intraventricular NCC is extremely rare in children [23,30]

Clinical manifestations are clearly different when comparing by age [19]. Most cases of childhood NCC present mild to moderate symptomatology and single lesions [20-22]. A study [23] specifically carried out to compare the clinical manifestations between pediatric and adult NCC patients reported that seizures were more frequent in children [80.4% versus 56.1%), intracranial hypertension and headaches were more frequent in adults [27.2% versus 15.2% and 35.1% versus 21.7%, respectively), and focal deficits were 17% in adults and 12% in children. Although these age-related differences seem clear, a single effect of age is difficult to demonstrate, since various confounding factors are probably involved [19].

Most paediatric cases show a single transitional cyst, also named solitary cysticercus granuloma [2,24]. The single transitional cyst or single enhancing lesion on CT (SECTL) or hyperintense lesion on magnetic resonance imaging (MRI), is a common finding in patients with newly identified seizures in developing countries. These patients, mainly children and young adults, have some benign and transitory clinical manifestations, predominantly partial or partial secondary generalized seizures, and occasionally Todd's paresis or focal neurological deficits. SECTL tend to resolve spontaneously, without anticysticercal drugs or surgery, since the parasite is already in the degenerative phase and will eventually disappear or become calcified.

Symptomatology of altered mental state and psychiatric manifestations remains poorly described in the literature [9]. In two studies [25,26] which provided definitions of clinical manifestations, depression was reported about 53% and 15%, respectively. Differences on gender have also been identified. Inflammation surrounding parenchymal cysticerci is more intense in women [19]; and multiple degenerating parasites localized in the parenchyma are more frequently reported in young women. Regardless of the localization of the parasite, the inflammatory response, as expressed by cerebrospinal fluid (CSF) cellularity is also more intense in women [3]. A recent prospective study [19] confirms that there are significant gender and age differences in the local immune response, even after adjusting for differences in healthcare access.

## **4. Extraparenchymal neurocysticercosis**

The extraparenchymal location (around 15–30% of cases) develops different clinical manifestations. Headache and signs of intracranial pressure are more frequent in the extraparenchymal location [88%), in comparison with the parenchymal location (10%). [12] When cysticerci are located inside the ventricular system, life-threatening acute intracranial hypertension as a result of hydrocephalus may occur; as a consequence, severe headache, dizziness and consciousness alteration are the predominant clinical manifestations [5,27].

Cysts in the subarachnoid space may lodge in the Sylvian fissure or basal cisterns and grow to a big size (racemose form), causing intracranial hypertension. This is associated with an intense inflammatory reaction, and fibrosis and progressive thickening of the leptomeninges at the base of the brain [13]. There is an obstruction of the CSF circulation, resulting in hydrocephalus and progressive intracranial hypertension. Inflammation of meninges, cranial nerve involvement, chiasmatic syndrome, and cerebral infarcts secondary to vasculitis may develop [5,6]. When hydrocephalus due to cysticercotic meningitis is present, the mortality rate is high (50%), and most patients die within 2 years after CSF shunting [6,8]. This is why ventricular and basal cisternal locations are considered to be malignant forms of NCC [28]. In cases with NCC meningitis, cerebrospinal fluid (CSF) may show mild elevation of protein, hypoglycorrhachia and lymphocytic pleocytosis, which is similar to other chronic basal meningitis, such as tuberculosis and mycosis.[2]

Spinal cord cysticercosis is rare. Patients experience nonspecific clinical manifestations, such us nerve root pain or spinal cord compression syndromes, according to the level of the lesion [29]. Severe forms of NCC may exceptionally occur, including cysticercotic encephalitis, which result in permanent neurologic sequel, such as amaurosis. Hydrocephalus and intraventricular NCC is extremely rare in children [23,30]

## **5. Immunological and imaging diagnosis**

280 Novel Aspects on Cysticercosis and Neurocysticercosis

evolutionary stages of the parasite.

disappear or become calcified.

differences in healthcare access.

**4. Extraparenchymal neurocysticercosis** 

**3. Parenchymal neurocysticercosis** 

As previously stated, the most common clinical manifestation of parenchymal NCC is epileptic seizure, which occurs in 60-90% of cases. A clinical study including 336 patients [12], reported that seizures were the most common manifestation in the parenchymal location [88%), in comparison with the extraparenchymal location [20%); similarly, focal deficits or motor abnormalities were commonest in the parenchymal location [24% vs. 14%). Focal neurological deficits, when present, are usually transient, over a period of a few days, weeks, or months, with periods of remission and relapse, most likely due to different

Clinical manifestations are clearly different when comparing by age [19]. Most cases of childhood NCC present mild to moderate symptomatology and single lesions [20-22]. A study [23] specifically carried out to compare the clinical manifestations between pediatric and adult NCC patients reported that seizures were more frequent in children [80.4% versus 56.1%), intracranial hypertension and headaches were more frequent in adults [27.2% versus 15.2% and 35.1% versus 21.7%, respectively), and focal deficits were 17% in adults and 12% in children. Although these age-related differences seem clear, a single effect of age is difficult to demonstrate, since various confounding factors are probably involved [19].

Most paediatric cases show a single transitional cyst, also named solitary cysticercus granuloma [2,24]. The single transitional cyst or single enhancing lesion on CT (SECTL) or hyperintense lesion on magnetic resonance imaging (MRI), is a common finding in patients with newly identified seizures in developing countries. These patients, mainly children and young adults, have some benign and transitory clinical manifestations, predominantly partial or partial secondary generalized seizures, and occasionally Todd's paresis or focal neurological deficits. SECTL tend to resolve spontaneously, without anticysticercal drugs or surgery, since the parasite is already in the degenerative phase and will eventually

Symptomatology of altered mental state and psychiatric manifestations remains poorly described in the literature [9]. In two studies [25,26] which provided definitions of clinical manifestations, depression was reported about 53% and 15%, respectively. Differences on gender have also been identified. Inflammation surrounding parenchymal cysticerci is more intense in women [19]; and multiple degenerating parasites localized in the parenchyma are more frequently reported in young women. Regardless of the localization of the parasite, the inflammatory response, as expressed by cerebrospinal fluid (CSF) cellularity is also more intense in women [3]. A recent prospective study [19] confirms that there are significant gender and age differences in the local immune response, even after adjusting for

The extraparenchymal location (around 15–30% of cases) develops different clinical manifestations. Headache and signs of intracranial pressure are more frequent in the Two main techniques, the Enzyme-linked Immunosorbent Assay (Ab-ELISA) and Enzyme-Linked Immunoelectrotransfer Blot (EITB) Assay, are used for immunological diagnosis of NCC. ELISA test for antibodies or antigen detection have showed higher sensitivities and specificities in CSF than in sera [31]. EITB sensibility seems to be not significantly different in sera and CSF while specificity is higher in CSF [32]. Sensibility of either tests falls in cases of single cysts in parenchyma or when they are calcified [33]. In patients with reliable diagnosis of NCC by imaging studies, immunological test is not required, since a negative test will not discard a NCC. When imaging of extraparnechymal cysts is doubtful, determination of antigens by ELISA in CSF may be helpful [31].

Computed tomography and magnetic resonance imaging have been useful in the study of the parasite evolution within the brain parenchyma [2]. Once the oncosphere has passed into the parenchyma, it grows and evolves through vesicular, colloidal, granular-nodular, and calcified phases [13]. In the vesicular phase the larva lives inside a translucent liquidfilled cystic structure surrounded by a thin membrane. The CT scan depicts circumscribed, rounded, hypodense areas, varying in size and number, without enhancement by contrast media [34]. With the MRI, the vesicular larva appears with a CSF-like intensity signal on all sequences. Both MRI and CT may show a high intensity, 2-3 mm. mural nodule, depicting the scolex, in the interior of some vesicular cysts (Figure 1). It is better seen on fluid attenuated inversion recovery (FLAIR) sequence [35].

Clinical Diagnoses of Neurocysticercosis 283

A: Cyst in the fourth ventricle B: Racemose cysts in the basal subarachanoid space C: cysts in the prepontine cisternal

**D**

Extraparenchymal NCC is more difficult to detect by imaging because the attenuation and signal intensity of the cyst's content is similar to that of CSF, the cystic wall is usually very thin and the cysts lack frequently of a scolex and generally not enhanced after contrast administration. In these cases, new MRI techniques such as fluid attenuated inversion recovery (FLAIR) and fast imaging employing steady-state acquisition (FIESTA) sequences, that seems to permit a better diagnosis (34-36) (Figure 2) . A common neuroimaging finding is hydrocephalus related to inflammatory occlusion of Luschka and Magendie foramina and

space (MRI FIESTA sequence) D: Racemose cysts in the degenerative stage

**A B**

**Figure 2.** Imaging of extraparenchymal neurocysticercosis

**C**

A: vesicular cysts B: colloidal cysts C: nodular cysts D: calcified cysts

**Figure 1.** Imaging of parenchymal neurocysticercosis

As the cyst degenerates, the CT scan shows an annular or nodular enhancement surrounded by perilesional edema. In this stage, the fluid content gives slightly higher signal than CSF and is sometimes isodense with the parenchyma on MRI-T1 and/or proton densityweighted, and high signal on T2 images. The capsule shows higher signal than the adjacent brain with thick ring enhancement on T1 images, while on T2 images there is a low ring signal surrounded by high signal lesion, due mostly to edema. Finally, when the cyst dies it may disappear or become an inactive calcified nodule of homogenous high density on CT, or low intensity on proton-weighted MRI [2] (Figure 1).

A: Cyst in the fourth ventricle B: Racemose cysts in the basal subarachanoid space C: cysts in the prepontine cisternal space (MRI FIESTA sequence) D: Racemose cysts in the degenerative stage

**Figure 2.** Imaging of extraparenchymal neurocysticercosis

282 Novel Aspects on Cysticercosis and Neurocysticercosis

**A**

A: vesicular cysts B: colloidal cysts C: nodular cysts D: calcified cysts **Figure 1.** Imaging of parenchymal neurocysticercosis

**C D**

or low intensity on proton-weighted MRI [2] (Figure 1).

As the cyst degenerates, the CT scan shows an annular or nodular enhancement surrounded by perilesional edema. In this stage, the fluid content gives slightly higher signal than CSF and is sometimes isodense with the parenchyma on MRI-T1 and/or proton densityweighted, and high signal on T2 images. The capsule shows higher signal than the adjacent brain with thick ring enhancement on T1 images, while on T2 images there is a low ring signal surrounded by high signal lesion, due mostly to edema. Finally, when the cyst dies it may disappear or become an inactive calcified nodule of homogenous high density on CT,

**B**

Extraparenchymal NCC is more difficult to detect by imaging because the attenuation and signal intensity of the cyst's content is similar to that of CSF, the cystic wall is usually very thin and the cysts lack frequently of a scolex and generally not enhanced after contrast administration. In these cases, new MRI techniques such as fluid attenuated inversion recovery (FLAIR) and fast imaging employing steady-state acquisition (FIESTA) sequences, that seems to permit a better diagnosis (34-36) (Figure 2) . A common neuroimaging finding is hydrocephalus related to inflammatory occlusion of Luschka and Magendie foramina and basal fibrous arachnoiditis [36]. Cysts located within CSF cisterns usually have a multilobulated appearance (racemose form), displacing neighboring structures as a mass occupying lesion (Figure 2).

Clinical Diagnoses of Neurocysticercosis 285

exists that these criteria should be revised to incorporate current scientific knowledge, in

So far, diagnosis of NCC is mainly done by neuroimaging. New imaging techniques have improved detection of the scolex inside the cysts, which can be considered pathognomonic of neurocysticercosis There is no a typical clinical manifestation of NCC. Prospective cohort studies addressed to analyzing NCC clinical manifestations, including definitions and appropriate methodology, which make them comparable, are extremely scarce in the medical literature. Location of the parasite in the CNS, age, sex, and immunological response of the patient, all seem to play an important role in occurrence of symptoms and signs; however, the relative contribution of these factors, alone or in combination, is still

In the parenchymal location, seizures are the most frequent clinical manifestation, followed by headache, motor focal deficits, and psychiatric and cognitive symptomatology. These clinical manifestations may have periods of remission and exacerbation, according to the evolutionary phase of the parasite inside the parenchyma. Diagnosis of extraparenchymal NCC is even more difficult, considering that unspecific symptoms and signs of intracranial hypertension and meningitis may occur, with or without signs of CSF inflammation. The clinical manifestations due to parenchymal location are usually benign and are sometimes transitory in time; on the contrary, clinical presentation of the extraparenchymal location is

Properly-designed studies with similar methodology are needed to learn more about the natural history of the disease and the true distribution of clinical manifestations. There is

[1] Takayanagui OM, Jardim E. Clinical aspects of neurocysticercosis: analysis of 500 cases.

[2] Carpio A. Neurocysticercosis: an update. Lancet Infectious Diseases. 2002;2:751-762. [3] Fleury A, Escobar A, Fragoso G, Sciutto E, Larralde C. Clinical heterogeneity of human neurocysticercosis results from complex interactions among parasite, host and

environmental factors. Trans R Soc Trop Med Hyg. 2010;104:243-250

also an urgent need to develop new consensus diagnosis criteria for NC.

order to achieve a new consensus on the diagnosis of NCC.

life-threatening and may develop permanent sequels.

*School of Medicine, University of Cuenca, Ecuador* 

Arq Neuropsiquiatr 1983;41:50–63.

*G.H. Sergievsky Center, Columbia University, New York, USA* 

**7. Conclusion** 

unknown.

**Author details** 

Arturo Carpio

**8. References** 

## **6. Difficulties with clinical diagnosis of neurocysticercosis**

Del Brutto et al. proposed a diagnostic criterion for NCC in 2001 [37]. In spite of the fact that this proposal has not been validated so far, it has been an important contribution to current NCC knowledge. This proposal includes four categories of criteria—absolute, major, minor, and epidemiologic. A combination of these criteria results in degrees of diagnostic certainty: definitive and probable. Diagnosis of definitive NCC is made by the presence of one absolute criterion or of two major plus one minor or one epidemiologic criterion [37].

These diagnostic criteria may be useful to identify patients with mainly parenchymal forms of NC, but it is not for patients with extraparenchymal forms of NCC [31]. Among minor criteria are considered "manifestations suggestive of neurocysticercosis" which include seizures, focal neurologic deficits, increased intracranial pressure, and intellectual deterioration, which are predominantly related to parenchymal location of lesions, but not necessarily to extraparenchymal clinical manifestations such us those associated with intraventricular cysts or NCC meningeal inflammation. Among absolute criteria are considered "Cystic lesions showing the scolex on CT or MRI" or "spontaneous resolution of small single enhancing lesions" It is extremely rare to see scolex on CT or MRI inside the ventricles or the subarachnoid location, and it is also very difficult to demonstrate spontaneous resolution of small single enhancing lesions in the extraparenchymal location.

This diagnostic criteria proposal may be a source of bias even in the event of diagnosing parenchymal NC. For example, a patient who lives in any country where cysticercosis is endemic (epidemiologic criterion), with seizure or headache (1 minor criterion), a CT showing a calcification (one major criterion), and a positive serum enzyme-linked immunoelectrotransfer blot assay –EITB- (one major criterion), according to this proposal, should be categorized as "definitive NC". However, it could be erroneous to diagnose this patient as having NC, since headache is extremely unspecific, a positive EITB means just exposure to the parasite, and a calcification could correspond to many other pathologies, but not necessarily NC. The selection of these patients in some reports, especially those community-based, is probably over-diagnosing NC.

There are additional problems related to this proposal that could lead to a misleading diagnosis. According to these diagnosis criteria, two major criteria fulfill a definite NCC diagnosis criterion. In this context, a patient with a positive immunologic test for the detection of anticysticercal antibodies (major criterion) plus a plain X-ray films showing "cigarshaped" calcifications in thigh and calf muscles (major criterion) could be diagnosed as NC. It is very hard to accept that such a patient may unquestionably have cysticerci in the brain. Some authors have claimed that the generalized use of Del Brutto et al [38 ] criteria have created some distortion in the present perception of NCC [31] A broad consensus now exists that these criteria should be revised to incorporate current scientific knowledge, in order to achieve a new consensus on the diagnosis of NCC.

## **7. Conclusion**

284 Novel Aspects on Cysticercosis and Neurocysticercosis

occupying lesion (Figure 2).

basal fibrous arachnoiditis [36]. Cysts located within CSF cisterns usually have a multilobulated appearance (racemose form), displacing neighboring structures as a mass

Del Brutto et al. proposed a diagnostic criterion for NCC in 2001 [37]. In spite of the fact that this proposal has not been validated so far, it has been an important contribution to current NCC knowledge. This proposal includes four categories of criteria—absolute, major, minor, and epidemiologic. A combination of these criteria results in degrees of diagnostic certainty: definitive and probable. Diagnosis of definitive NCC is made by the presence of one

These diagnostic criteria may be useful to identify patients with mainly parenchymal forms of NC, but it is not for patients with extraparenchymal forms of NCC [31]. Among minor criteria are considered "manifestations suggestive of neurocysticercosis" which include seizures, focal neurologic deficits, increased intracranial pressure, and intellectual deterioration, which are predominantly related to parenchymal location of lesions, but not necessarily to extraparenchymal clinical manifestations such us those associated with intraventricular cysts or NCC meningeal inflammation. Among absolute criteria are considered "Cystic lesions showing the scolex on CT or MRI" or "spontaneous resolution of small single enhancing lesions" It is extremely rare to see scolex on CT or MRI inside the ventricles or the subarachnoid location, and it is also very difficult to demonstrate spontaneous resolution of small single enhancing lesions in the extraparenchymal location.

This diagnostic criteria proposal may be a source of bias even in the event of diagnosing parenchymal NC. For example, a patient who lives in any country where cysticercosis is endemic (epidemiologic criterion), with seizure or headache (1 minor criterion), a CT showing a calcification (one major criterion), and a positive serum enzyme-linked immunoelectrotransfer blot assay –EITB- (one major criterion), according to this proposal, should be categorized as "definitive NC". However, it could be erroneous to diagnose this patient as having NC, since headache is extremely unspecific, a positive EITB means just exposure to the parasite, and a calcification could correspond to many other pathologies, but not necessarily NC. The selection of these patients in some reports, especially those

There are additional problems related to this proposal that could lead to a misleading diagnosis. According to these diagnosis criteria, two major criteria fulfill a definite NCC diagnosis criterion. In this context, a patient with a positive immunologic test for the detection of anticysticercal antibodies (major criterion) plus a plain X-ray films showing "cigarshaped" calcifications in thigh and calf muscles (major criterion) could be diagnosed as NC. It is very hard to accept that such a patient may unquestionably have cysticerci in the brain. Some authors have claimed that the generalized use of Del Brutto et al [38 ] criteria have created some distortion in the present perception of NCC [31] A broad consensus now

community-based, is probably over-diagnosing NC.

absolute criterion or of two major plus one minor or one epidemiologic criterion [37].

**6. Difficulties with clinical diagnosis of neurocysticercosis** 

So far, diagnosis of NCC is mainly done by neuroimaging. New imaging techniques have improved detection of the scolex inside the cysts, which can be considered pathognomonic of neurocysticercosis There is no a typical clinical manifestation of NCC. Prospective cohort studies addressed to analyzing NCC clinical manifestations, including definitions and appropriate methodology, which make them comparable, are extremely scarce in the medical literature. Location of the parasite in the CNS, age, sex, and immunological response of the patient, all seem to play an important role in occurrence of symptoms and signs; however, the relative contribution of these factors, alone or in combination, is still unknown.

In the parenchymal location, seizures are the most frequent clinical manifestation, followed by headache, motor focal deficits, and psychiatric and cognitive symptomatology. These clinical manifestations may have periods of remission and exacerbation, according to the evolutionary phase of the parasite inside the parenchyma. Diagnosis of extraparenchymal NCC is even more difficult, considering that unspecific symptoms and signs of intracranial hypertension and meningitis may occur, with or without signs of CSF inflammation. The clinical manifestations due to parenchymal location are usually benign and are sometimes transitory in time; on the contrary, clinical presentation of the extraparenchymal location is life-threatening and may develop permanent sequels.

Properly-designed studies with similar methodology are needed to learn more about the natural history of the disease and the true distribution of clinical manifestations. There is also an urgent need to develop new consensus diagnosis criteria for NC.

## **Author details**

Arturo Carpio *School of Medicine, University of Cuenca, Ecuador G.H. Sergievsky Center, Columbia University, New York, USA* 

## **8. References**


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Clin Infect Dis 1996;23:262–268.

[4] Dixon HBF, Lipscomb FM. Cysticercosis:an analysis and follow-up of 450 cases. Med Res Council spec.rep., no.299. London: Her Majesty's Stationery Office, 1961;1-58. [5] Agapejev S, Pouza AF, Bazan R, Faleiros AT. Clinical and evolutive aspects of

[6] Cárdenas G, Jung H, Ríos C, Fleury A, Soto-Hernández JL. Severe cysticercal meningitis: clinical and imaging characteristics. Am J Trop Med Hyg. 2010;82(1):121-5. [7] Rodrigues CL, de Andrade DC, Livramento JA, Machado LR, Abraham R, et al. Spectrum of cognitive impairment in neurocysticercosis: differences according to

[8] Sotelo J, Marin C. Hydrocephalus secondary to cysticercotic arachnoiditis. A long-term

[9] Carabin H, Ndimubanzi PC, Budke CM, Nguyen H, Qian Y, et al. Clinical manifestations associated with neurocysticercosis: a systematic review. PLoS Negl Trop

[10] Patel R, Jha S, Yadav RK. Pleomorphism of the clinical manifestations of

[11] Monteiro L, Almeida-Pinto J, Stocker A, Sampaio-Silva M. Active neurocysticercosis, parenchymal and extraparenchymal: a study of 38 patients. J Neurol 1993;241:15–21. [12] Carpio A, Placencia M, Santillan F, Escobar A. A proposal for a classification of

[13] Escobar A. The pathology of neurocysticercosis In, Palacios E, Rodriguez-Carbajal J, Taveras JM, eds. Cysticercosis of Central Nervous System. Springfield: Charles C

[14] Singh G. Neurocysticercosis in South-Central America and the Indian subcontinent. A

[15] Maravilla, P., Gonzalez-Guzman, R., Zuniga, G., Peniche, A., Dominguez-Alpizar, J.L. et al. Genetic polymorphism in Taenia solium cysticerci recovered from experimental

[16] Vega, R., Pinero, D., Ramanankandrasana, B., Dumas, M., Bouteille, B., et al. Population genetic structure of Taenia solium from Madagascar and Mexico: implications for clinical profile diversity and immunological technology. Int J Parasitol 2003;33:1479-

[17] Jain, S., Padma, M.V., Kanga, U., Mehra, N.K. and Maheshwari, N.C. Family studies and human leukocyte antigen class II typing in Indian probands with seizures in association with single small enhancing computed tomography lesions. Epilepsia

[18] Kelvin, E.A., Carpio, A., Hesdorffer, D.C., Bagiella, E., Leslie. et al. Investigation of familial aggregation of seizures in neurocysticercosis patients. Epilepsy Res 2009;84:67-

[19] Kelvin EA, Carpio A, Bagiella E, Leslie D, Leon P, Andrews H, et al. The association of host age and gender with inflammation around neurocysticercosis cysts. Ann Trop Med

[20] Rosenfeld EA, Byrd SE, Shulman ST. Neurocysticercosis among children in Chicago.

hydrocephalus in neurocysticercosis. Arq Neuropsiquiatr. 2007;65:674-80.

disease phase. Neurology. 2012 Mar 20;78(12):861-866.

neurocysticercosis. Can J Neuro Sci 1994;21:43-47.

infections in pigs. Infect Genet Evol 2008;8:213-216.

follow-up review of 92 cases. J Neurosurg 1987;66:686–689.

neurocysticercosis. Trans R Soc Trop Med Hyg. 2006;100:134-141.

comparative evaluation. Arq Neuropsiquiatr 1997;55:349–356.

	- [37] Del Brutto OH, Rajshekhar V, White Jr AC, Tsang VCW, Nash TE, Takayanagui OM, et al. Proposed diagnostic criteria for neurocysticercosis. Neurology 2001;57:177–183.

**Chapter 11** 

© 2013 Carpio, licensee InTech. This is an open access chapter distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use,

distribution, and reproduction in any medium, provided the original work is properly cited.

© 2013 Carpio, licensee InTech. This is a paper distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Neurocysticercosis (NCC) is a biological marker of poverty and underdevelopment [1]. The frequency of this brain parasitic disease is probably high worldwide, as well as epilepsy; however, little is known about the natural history of the infection in humans. Both, NCC and epilepsy, are an increasing burden on the welfare and economy of developing countries [2]. This is why knowledge of these diseases should be improved, in order to prevent them. Acute symptomatic seizures are the most common clinical manifestation of NCC in those patients in whom a cysticercus is located in brain parenchyma [1-3]. Epilepsy attributable to NCC usually has a good prognosis in terms of seizure control and seizure remission [1,4]. Although some authors have suggested that anticysticercal treatment is associated with reduced seizure recurrence, there is no evidence that cysticidal treatment does more good than harm in addition to conventional antiepileptic treatment [2,5 ] According to new information, the traditional view of the relationship between NCC and epilepsy [2,3] can be

questioned. Nowadays, NCC is considered a comorbid component of epilepsy [6]

In developed countries, the age-adjusted incidence of epilepsy ranges from 24 to 53/100,000 [7]. In developing countries (DC) the incidence of epilepsy on the basis of two studies carried out in South America in the last decade, ranged from 114 to 190/100,000 [8]; considerably higher than that reported in industrialized countries. New studies carried out in the last few years confirm high incidence: A hospital-based study of children in Tunisia reported the incidence of first unprovoked seizures to be 102/100,000 [9]. A populationbased study carried out among children in Kenya, reported the incidence of epilepsy (two or more two unprovoked seizures) to be 187/100,000 [10]. In a population-based study among all ages in Honduras, Central America [11], the incidence of patients with non-febrile seizures was 92.7/100,000. Because of the broader case-inclusion criteria and uncertainty

**2. Epidemiology of neurocysticercosis and epilepsy** 

**Neurocysticercosis and Epilepsy** 

Additional information is available at the end of the chapter

Arturo Carpio

**1. Introduction** 

http://dx.doi.org/10.5772/52389

[38] Machado LR. The diagnosis of neurocysticercosis: a closed question? Arq Neuropsiquiatr. 2010;68(1):1-2.

## **Chapter 11**

## **Neurocysticercosis and Epilepsy**

## Arturo Carpio

288 Novel Aspects on Cysticercosis and Neurocysticercosis

Neuropsiquiatr. 2010;68(1):1-2.

[37] Del Brutto OH, Rajshekhar V, White Jr AC, Tsang VCW, Nash TE, Takayanagui OM, et al. Proposed diagnostic criteria for neurocysticercosis. Neurology 2001;57:177–183. [38] Machado LR. The diagnosis of neurocysticercosis: a closed question? Arq

Additional information is available at the end of the chapter

http://dx.doi.org/10.5772/52389

## **1. Introduction**

Neurocysticercosis (NCC) is a biological marker of poverty and underdevelopment [1]. The frequency of this brain parasitic disease is probably high worldwide, as well as epilepsy; however, little is known about the natural history of the infection in humans. Both, NCC and epilepsy, are an increasing burden on the welfare and economy of developing countries [2]. This is why knowledge of these diseases should be improved, in order to prevent them.

Acute symptomatic seizures are the most common clinical manifestation of NCC in those patients in whom a cysticercus is located in brain parenchyma [1-3]. Epilepsy attributable to NCC usually has a good prognosis in terms of seizure control and seizure remission [1,4]. Although some authors have suggested that anticysticercal treatment is associated with reduced seizure recurrence, there is no evidence that cysticidal treatment does more good than harm in addition to conventional antiepileptic treatment [2,5 ] According to new information, the traditional view of the relationship between NCC and epilepsy [2,3] can be questioned. Nowadays, NCC is considered a comorbid component of epilepsy [6]

## **2. Epidemiology of neurocysticercosis and epilepsy**

In developed countries, the age-adjusted incidence of epilepsy ranges from 24 to 53/100,000 [7]. In developing countries (DC) the incidence of epilepsy on the basis of two studies carried out in South America in the last decade, ranged from 114 to 190/100,000 [8]; considerably higher than that reported in industrialized countries. New studies carried out in the last few years confirm high incidence: A hospital-based study of children in Tunisia reported the incidence of first unprovoked seizures to be 102/100,000 [9]. A populationbased study carried out among children in Kenya, reported the incidence of epilepsy (two or more two unprovoked seizures) to be 187/100,000 [10]. In a population-based study among all ages in Honduras, Central America [11], the incidence of patients with non-febrile seizures was 92.7/100,000. Because of the broader case-inclusion criteria and uncertainty

© 2013 Carpio, licensee InTech. This is an open access chapter distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. © 2013 Carpio, licensee InTech. This is a paper distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

regarding age-specific distribution in these studies, there is no way to compare results from these incidence studies. Nonetheless, the incidence of epilepsy is probably higher in DC than in industrialized countries [7].

Neurocysticercosis and Epilepsy 291

**3. Definitions of epileptic seizures, epilepsy and neurocysticercosis** 

effective health care for people with epilepsy and NCC [2].

scolex, in the interior of some parenchymal vesicular cysts.

on proton-weighted MRI [1].

subarachonoid and intraventricular forms.

In order to understand the relationship between epilepsy and NCC, it is crucial to promote consistency in definitions to enhance research studies, facilitate comparison between populations, provide data useful for detection, treatment, and prevention, and to promote

The natural history of the cysticerci in the CNS is not entirely understood. Computed tomography (CT) and magnetic resonance imaging (MRI) have been useful in the study of the evolution of the cysticercus within the brain parenchyma [20]. Once the oncosphere has passed into the parenchyma, it grows and evolves through vesicular, colloidal, granularnodular, and calcified phases [21]. In the vesicular phase the host tends to show immune tolerance, and in most cases there is no surrounding parenchymal reaction; the larva lives inside a translucent liquid-filled cystic structure surrounded by a thin membrane, where it can remain viable from a few months to several years [22] The CT scan depicts circumscribed, rounded, hypodense areas, varying in size and number, without enhancement by contrast media [20].With the MRI, the vesicular larva appears with a CSFlike intensity signal on all sequences, with no surrounding high signal on T2-weighted images. Both MRI and CT may show a high intensity, 2-3 mm. mural nodule, depicting the

As the cyst degenerates, it goes through a transitional stage: the contrast enhanced CT scan shows an annular (colloidal phase) or nodular [nodular phase) enhancement surrounded by irregular perilesional edema [1,20,22]. In this stage, the fluid content gives slightly higher signal than CSF and is sometimes isodense with the parenchyma on MRI-T1 and/or proton density-weighted, and high signal on T2 images. The capsule shows higher signal than the adjacent brain with thick ring enhancement on T1 images, while on T2 images there is a low ring signal surrounded by high signal lesion, due mostly to edema. Although these pathological changes are responsible for clinical manifestations, usually seizures and headache, they may not cause symptoms at all. Finally, when the cyst dies it may disappear or become an inactive calcified nodule of homogenous high density on CT, or low intensity

Based on the abovementioned concepts a classification based on the viability and location of the parasite in the CNS of the host has been proposed: active, when the parasite is alive; transitional, if it is in the degenerative phase; inactive, if there is evidence of its death [20]. Each viability category was subdivided into parenchymal and extraparenchymal forms. The viability criterion is very important, as it allows us to analyze the natural history of the parasite, and, according to the parasite's evolutionary stage, the production of physiopathological changes in the host's CNS. On the basis of this classification, it is possible to relate clinical manifestations and therapeutic procedures to each category of the proposed classification. For example, seizures are the main symptom in the transitional parenchymal form due to the brain inflammatory reaction, whereas cranial nerve abnormalities and intracranial hypertension syndromes are more frequent in the

Reported prevalence of active epilepsy, based on methodology recommended by the International League Against Epilepsy (ILAE) Commission of Epidemiology and Prognosis [12] in DC, ranges from 3.9 to 15.7 per 1,000 person-years [13] These differences, however, are due to different approaches used by the studies and differences regarding the definitions of active epilepsy: Limiting assessment to those studies that provide information for ageadjusted rates, the average age adjusted prevalence of active epilepsy is 8.5/1,000, which is similar to the developed countries. This prevalence is low given the high incidence of epilepsy in DC and may be due to the fact that mortality of people with epilepsy is higher in comparison with that of developed countries. While studies from developed countries show a consistent pattern suggesting that the onset of epilepsy occurs at both extremes of life [7]; on the other hand, incidence in DC is highest among young and middle-aged adults [13], perhaps as a manifestation of diseases prevalent in these age-groups. This includes conditions such as brain injury and infectious and parasitic diseases endemic in these countries.

Taenia solium infection is widely endemic in poor countries, both in highlands or tropical areas, in Central and South America, and non-Muslim populations of Asia and Africa [1]. Cysticercosis is considered an emerging infectious disease in some developed countries; persons who have never left the United States as well as visitors to disease-endemic regions are at risk [2]. Data on the frequency of NCC from endemic countries have been compiled from clinic-based patients or autopsied cadavers. NCC was found, for example, in 2.8%- 3.6% of all autopsies in some hospitals [14]. However, such statistics can be misleading because differences in availability of medical services and lack of comprehensive and consistent reporting in most countries still confound attempts to compare incidence and prevalence among countries [15]. Most reports fail to provide even minimal information regarding diagnostic criteria and definitions; consequently, these data are definitely biased and hardly representative of the general population.

Immunoserologic assays, such as enzyme-linked immunoelectrotransfer blot assay (EITB) or enzyme-linked immunosorbent assay (ELISA), detect antibodies against T. solium or cysticercus [1] Epidemiological surveys for human cysticercosis, using EITB as a marker of infection, report a seroprevalence from 8% to 12% in some regions of Latin American [16,17]. These assays are useful for identification of individuals who have had systemic contact with the parasite at some time. Seropositivity, however, does not necessarily mean an active systemic infection or central nervous system (CNS) involvement at any time [1,18]. Most seropositive individuals in these populations were asymptomatic. There are no prospective cohort studies providing information on the proportion of seropositive individuals that will develop seizures or other neurological symptoms. Some studies [17], but not all [19] have reported an association between seizures and seropositivity. Although a higher proportion of patients with epilepsy have been shown to be EITB positive when compared to those without epilepsy, the proportion of seropositivity in epileptic patients is similar to that reported in the general population in these same areas [17].

## **3. Definitions of epileptic seizures, epilepsy and neurocysticercosis**

290 Novel Aspects on Cysticercosis and Neurocysticercosis

than in industrialized countries [7].

regarding age-specific distribution in these studies, there is no way to compare results from these incidence studies. Nonetheless, the incidence of epilepsy is probably higher in DC

Reported prevalence of active epilepsy, based on methodology recommended by the International League Against Epilepsy (ILAE) Commission of Epidemiology and Prognosis [12] in DC, ranges from 3.9 to 15.7 per 1,000 person-years [13] These differences, however, are due to different approaches used by the studies and differences regarding the definitions of active epilepsy: Limiting assessment to those studies that provide information for ageadjusted rates, the average age adjusted prevalence of active epilepsy is 8.5/1,000, which is similar to the developed countries. This prevalence is low given the high incidence of epilepsy in DC and may be due to the fact that mortality of people with epilepsy is higher in comparison with that of developed countries. While studies from developed countries show a consistent pattern suggesting that the onset of epilepsy occurs at both extremes of life [7]; on the other hand, incidence in DC is highest among young and middle-aged adults [13], perhaps as a manifestation of diseases prevalent in these age-groups. This includes conditions such as

Taenia solium infection is widely endemic in poor countries, both in highlands or tropical areas, in Central and South America, and non-Muslim populations of Asia and Africa [1]. Cysticercosis is considered an emerging infectious disease in some developed countries; persons who have never left the United States as well as visitors to disease-endemic regions are at risk [2]. Data on the frequency of NCC from endemic countries have been compiled from clinic-based patients or autopsied cadavers. NCC was found, for example, in 2.8%- 3.6% of all autopsies in some hospitals [14]. However, such statistics can be misleading because differences in availability of medical services and lack of comprehensive and consistent reporting in most countries still confound attempts to compare incidence and prevalence among countries [15]. Most reports fail to provide even minimal information regarding diagnostic criteria and definitions; consequently, these data are definitely biased

Immunoserologic assays, such as enzyme-linked immunoelectrotransfer blot assay (EITB) or enzyme-linked immunosorbent assay (ELISA), detect antibodies against T. solium or cysticercus [1] Epidemiological surveys for human cysticercosis, using EITB as a marker of infection, report a seroprevalence from 8% to 12% in some regions of Latin American [16,17]. These assays are useful for identification of individuals who have had systemic contact with the parasite at some time. Seropositivity, however, does not necessarily mean an active systemic infection or central nervous system (CNS) involvement at any time [1,18]. Most seropositive individuals in these populations were asymptomatic. There are no prospective cohort studies providing information on the proportion of seropositive individuals that will develop seizures or other neurological symptoms. Some studies [17], but not all [19] have reported an association between seizures and seropositivity. Although a higher proportion of patients with epilepsy have been shown to be EITB positive when compared to those without epilepsy, the proportion of seropositivity in epileptic patients is similar to that

brain injury and infectious and parasitic diseases endemic in these countries.

and hardly representative of the general population.

reported in the general population in these same areas [17].

In order to understand the relationship between epilepsy and NCC, it is crucial to promote consistency in definitions to enhance research studies, facilitate comparison between populations, provide data useful for detection, treatment, and prevention, and to promote effective health care for people with epilepsy and NCC [2].

The natural history of the cysticerci in the CNS is not entirely understood. Computed tomography (CT) and magnetic resonance imaging (MRI) have been useful in the study of the evolution of the cysticercus within the brain parenchyma [20]. Once the oncosphere has passed into the parenchyma, it grows and evolves through vesicular, colloidal, granularnodular, and calcified phases [21]. In the vesicular phase the host tends to show immune tolerance, and in most cases there is no surrounding parenchymal reaction; the larva lives inside a translucent liquid-filled cystic structure surrounded by a thin membrane, where it can remain viable from a few months to several years [22] The CT scan depicts circumscribed, rounded, hypodense areas, varying in size and number, without enhancement by contrast media [20].With the MRI, the vesicular larva appears with a CSFlike intensity signal on all sequences, with no surrounding high signal on T2-weighted images. Both MRI and CT may show a high intensity, 2-3 mm. mural nodule, depicting the scolex, in the interior of some parenchymal vesicular cysts.

As the cyst degenerates, it goes through a transitional stage: the contrast enhanced CT scan shows an annular (colloidal phase) or nodular [nodular phase) enhancement surrounded by irregular perilesional edema [1,20,22]. In this stage, the fluid content gives slightly higher signal than CSF and is sometimes isodense with the parenchyma on MRI-T1 and/or proton density-weighted, and high signal on T2 images. The capsule shows higher signal than the adjacent brain with thick ring enhancement on T1 images, while on T2 images there is a low ring signal surrounded by high signal lesion, due mostly to edema. Although these pathological changes are responsible for clinical manifestations, usually seizures and headache, they may not cause symptoms at all. Finally, when the cyst dies it may disappear or become an inactive calcified nodule of homogenous high density on CT, or low intensity on proton-weighted MRI [1].

Based on the abovementioned concepts a classification based on the viability and location of the parasite in the CNS of the host has been proposed: active, when the parasite is alive; transitional, if it is in the degenerative phase; inactive, if there is evidence of its death [20]. Each viability category was subdivided into parenchymal and extraparenchymal forms. The viability criterion is very important, as it allows us to analyze the natural history of the parasite, and, according to the parasite's evolutionary stage, the production of physiopathological changes in the host's CNS. On the basis of this classification, it is possible to relate clinical manifestations and therapeutic procedures to each category of the proposed classification. For example, seizures are the main symptom in the transitional parenchymal form due to the brain inflammatory reaction, whereas cranial nerve abnormalities and intracranial hypertension syndromes are more frequent in the subarachonoid and intraventricular forms.

According to the definitions suggested by the International League Against Epilepsy's Epidemiology Commissions [6,12] , epilepsy is defined as two or more unprovoked seizures occurring at least 24 hours apart. Unprovoked seizures may occur subsequent to a welldemonstrated antecedent condition, known to substantially increase the risk of epileptic seizures. New terminology and concepts proposed by the ILAE divide the causes of epilepsy into three broad categories: genetic, structural/metabolic, and unknown [23]. "The cause is considered structural/metabolic when a structural lesion (either static or progressive) or metabolic condition (e.g., inborn errors of metabolism) is present and is known to be associated with an increased risk of epilepsy." The cause is attributed to the condition that is most directly linked and proximate to the development of epilepsy, for example NCC, in the calcified phase.

Neurocysticercosis and Epilepsy 293

differentiate between provoked or acute symptomatic seizures and recurrent unprovoked seizures (epilepsy). This differentiation is very important, due to its implications concerning treatment and prognosis, as will be discussed below. Presumably, the inclusion of people with only acute symptomatic seizures as cases of epilepsy is one of the reasons for the high

In spite of the clinical heterogeneity and variability in the clinical forms of expression and evolution of NCC, all reports of medical literatures agree that seizures are the most common symptom of NC, occurring in 70-90% of patients [18,26]. However, seizure is more frequent when the parasite is located in the brain parenchyma of the patient, in comparison with the intraventricular or subarachnoid locations [20]. There is no uniformity in the reported distribution of seizure types in patients with NC. Some authors report a higher proportion of partial seizures, others conclude that generalized seizures are more frequent [1, 5, 27]. It seems that either generalized seizures or partial seizures with secondarily generalization are

Seizures may occur at any evolutionary stage of the parasite. A recent prospective cohort study [28], whose aim was to describe seizure as a presenting symptom in individuals with recently diagnosed neurocysticercosis showed that most of the seizures in patients in the youngest age category (3–24 years old) appear to be provoked or acute symptomatic seizures due to transitional cysts in the brain parenchyma rather than new onset idiopathic ones. Thus, it appears that children are more likely to have NCC-related seizures than adults. The authors found transitional cysts to be associated with a significantly higher probability of seizure in the chi-square analysis; however, in the regression models that adjusted for patient age and gender as well as the number and location of the cysts, no specific cyst phase was found to be significantly associated with having seizures. Patients with cysts in the parietal lobe and with cysts in the frontal lobe were also more likely to

It has been suggested that both age and gender influence the strength of the host's immune response. In a recent study [29] the odds of having transitional cysts were found to be 1.5 fold higher for the female patients than for the male; additionally, the number of transitional cysts was found to be 1.8-fold higher in the female patients than in the male, and this gender effect was not only statistically significant but also constant over time. It therefore appears that there are significant gender and age differences in the local immune response to NCC,

Some studies have also found familial aggregation of NCC infection [17, 30], as well as regional differences in the clinical manifestations of human NCC may indicate a role of host genetics [31]. However, in a recent study that investigated whether there is familial aggregation of seizures in first-degree relatives of NCC patients with seizure versus NCC patients without seizure as presenting symptom in a group of patients in Ecuador [32], there

**4. Seizures as a main clinical manifestation of neurocysticercosis** 

most commonly reported, while complex partial seizures are less frequent [1]

proportion of epilepsy reported in some studies [13].

even after adjusting for differences in healthcare access.

was no trend toward familial aggregation of seizures in NCC patients.

present seizures.

An epileptic seizure is defined as ''a transient occurrence of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain'' [24]. Epileptic seizures, even if recurrent, are not always synonymous with epilepsy per se [6]. Provoked or acute symptomatic seizures are seizures which occur ''in close temporal association with an acute systemic, metabolic, or toxic insult or in association with an acute central nervous system (CNS) insult (infection, stroke, cranial trauma, intracerebral hemorrhage, or acute alcohol intoxication or withdrawal),'' [12,25]. Such seizures are often isolated epileptic events associated with acute conditions, but may recur if the acute condition recurs, as happens in the transitional or degenerative form of NCC, due to the underlying inflammatory process

The interval between the insult and the seizure—which is used to separate acute symptomatic from unprovoked seizures—may vary according to the underlying clinical condition [23,25] . In the NCC case, this interval lasts while the inflammation signs due to the transitional or degenerative cysts persist (six months on average). Nonetheless, if seizures occur in the presence of parasites in viable or calcified phase, they are unprovoked and, if recurrent, should be considered epilepsy [1-3].

Seizures associated with NCC may be categorized as either acute symptomatic or as remote symptomatic seizures. Individuals with cysticerci in the transitional form or degenerative phase develop acute symptomatic seizures due to the acute inflammatory response of the brain; on the other hand, a patient with seizures who has active, viable cysts and/or inactive, non-inflammed calcified parasites may be categorized as having unprovoked seizures [3]. NCC has an unpredictable clinical course, which makes it difficult to categorize all cases into the proposed classification of the ILAE Commission. For instance, a patient with chronic recurrent seizures, whose imaging studies show several non-inflamed parenchymal calcifications, should be categorized as having remote symptomatic unprovoked seizures. The same patient, some years later, can experience a recurrence of parenchymal transitional cysts. This case should be considered to have multiple episodes of NCC now resulting in acute symptomatic seizures.

The recent ILAE report defines comorbidity as the co-occurrence of two or more separate medical conditions in the same individual and includes NCC as a comorbid condition associated with epilepsy [6]. Ultimately, in patients with NC, what matters most is to differentiate between provoked or acute symptomatic seizures and recurrent unprovoked seizures (epilepsy). This differentiation is very important, due to its implications concerning treatment and prognosis, as will be discussed below. Presumably, the inclusion of people with only acute symptomatic seizures as cases of epilepsy is one of the reasons for the high proportion of epilepsy reported in some studies [13].

## **4. Seizures as a main clinical manifestation of neurocysticercosis**

292 Novel Aspects on Cysticercosis and Neurocysticercosis

example NCC, in the calcified phase.

and, if recurrent, should be considered epilepsy [1-3].

acute symptomatic seizures.

According to the definitions suggested by the International League Against Epilepsy's Epidemiology Commissions [6,12] , epilepsy is defined as two or more unprovoked seizures occurring at least 24 hours apart. Unprovoked seizures may occur subsequent to a welldemonstrated antecedent condition, known to substantially increase the risk of epileptic seizures. New terminology and concepts proposed by the ILAE divide the causes of epilepsy into three broad categories: genetic, structural/metabolic, and unknown [23]. "The cause is considered structural/metabolic when a structural lesion (either static or progressive) or metabolic condition (e.g., inborn errors of metabolism) is present and is known to be associated with an increased risk of epilepsy." The cause is attributed to the condition that is most directly linked and proximate to the development of epilepsy, for

An epileptic seizure is defined as ''a transient occurrence of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain'' [24]. Epileptic seizures, even if recurrent, are not always synonymous with epilepsy per se [6]. Provoked or acute symptomatic seizures are seizures which occur ''in close temporal association with an acute systemic, metabolic, or toxic insult or in association with an acute central nervous system (CNS) insult (infection, stroke, cranial trauma, intracerebral hemorrhage, or acute alcohol intoxication or withdrawal),'' [12,25]. Such seizures are often isolated epileptic events associated with acute conditions, but may recur if the acute condition recurs, as happens in the transitional or degenerative form of NCC, due to the underlying inflammatory process

The interval between the insult and the seizure—which is used to separate acute symptomatic from unprovoked seizures—may vary according to the underlying clinical condition [23,25] . In the NCC case, this interval lasts while the inflammation signs due to the transitional or degenerative cysts persist (six months on average). Nonetheless, if seizures occur in the presence of parasites in viable or calcified phase, they are unprovoked

Seizures associated with NCC may be categorized as either acute symptomatic or as remote symptomatic seizures. Individuals with cysticerci in the transitional form or degenerative phase develop acute symptomatic seizures due to the acute inflammatory response of the brain; on the other hand, a patient with seizures who has active, viable cysts and/or inactive, non-inflammed calcified parasites may be categorized as having unprovoked seizures [3]. NCC has an unpredictable clinical course, which makes it difficult to categorize all cases into the proposed classification of the ILAE Commission. For instance, a patient with chronic recurrent seizures, whose imaging studies show several non-inflamed parenchymal calcifications, should be categorized as having remote symptomatic unprovoked seizures. The same patient, some years later, can experience a recurrence of parenchymal transitional cysts. This case should be considered to have multiple episodes of NCC now resulting in

The recent ILAE report defines comorbidity as the co-occurrence of two or more separate medical conditions in the same individual and includes NCC as a comorbid condition associated with epilepsy [6]. Ultimately, in patients with NC, what matters most is to In spite of the clinical heterogeneity and variability in the clinical forms of expression and evolution of NCC, all reports of medical literatures agree that seizures are the most common symptom of NC, occurring in 70-90% of patients [18,26]. However, seizure is more frequent when the parasite is located in the brain parenchyma of the patient, in comparison with the intraventricular or subarachnoid locations [20]. There is no uniformity in the reported distribution of seizure types in patients with NC. Some authors report a higher proportion of partial seizures, others conclude that generalized seizures are more frequent [1, 5, 27]. It seems that either generalized seizures or partial seizures with secondarily generalization are most commonly reported, while complex partial seizures are less frequent [1]

Seizures may occur at any evolutionary stage of the parasite. A recent prospective cohort study [28], whose aim was to describe seizure as a presenting symptom in individuals with recently diagnosed neurocysticercosis showed that most of the seizures in patients in the youngest age category (3–24 years old) appear to be provoked or acute symptomatic seizures due to transitional cysts in the brain parenchyma rather than new onset idiopathic ones. Thus, it appears that children are more likely to have NCC-related seizures than adults. The authors found transitional cysts to be associated with a significantly higher probability of seizure in the chi-square analysis; however, in the regression models that adjusted for patient age and gender as well as the number and location of the cysts, no specific cyst phase was found to be significantly associated with having seizures. Patients with cysts in the parietal lobe and with cysts in the frontal lobe were also more likely to present seizures.

It has been suggested that both age and gender influence the strength of the host's immune response. In a recent study [29] the odds of having transitional cysts were found to be 1.5 fold higher for the female patients than for the male; additionally, the number of transitional cysts was found to be 1.8-fold higher in the female patients than in the male, and this gender effect was not only statistically significant but also constant over time. It therefore appears that there are significant gender and age differences in the local immune response to NCC, even after adjusting for differences in healthcare access.

Some studies have also found familial aggregation of NCC infection [17, 30], as well as regional differences in the clinical manifestations of human NCC may indicate a role of host genetics [31]. However, in a recent study that investigated whether there is familial aggregation of seizures in first-degree relatives of NCC patients with seizure versus NCC patients without seizure as presenting symptom in a group of patients in Ecuador [32], there was no trend toward familial aggregation of seizures in NCC patients.

Neurologists from developing countries frequently see patients in whom the first seizure occurred many years before consultation; and when the second seizure occurs at the time of consultation, the imaging study shows one or more calcifications and one cyst in the transitional form with perilesional edema. We can assume that when the first seizures occurred, the patient had cysts in a transitional form, which eventually became calcified, and currently the patient has new acute seizures [1]. According to the ILAE Commission we should categorize these patients as having isolated epileptic events associated with a recurrent acute condition (transitional form) [3].

Neurocysticercosis and Epilepsy 295

generalized seizures, and occasionally Todd's paresis or focal neurological deficits. These lesions have been attributed mainly to cysticercosis or to tuberculosis; however, similar lesions have been reported in other inflammatory pathologies such as pyogenic abscess, histoplasmosis, blastomycosis and sarcoidiosis, post-infectious vasculitis, and to primary

The natural history of these lesions usually takes one of two forms: it becomes isodense on CT, or isointense on MR, and then either resolves entirely, or a punctuate calcification may be left as a residue [41]. The time till resolution of the lesion is quite variable ranging from a few weeks to more than a year. SECTL are benign and tend to resolve spontaneously, without anticysticercal drugs or surgery, since the parasite is already in the degenerative phase and will eventually disappear or become calcified [1]. Treatment should be limited to medication required to control the acute symptoms, such as antiepileptic medication [2].

Analytical studies designed to establish associations and determinants of epilepsy have been scarce in DC. Comparing results of studies of etiology is difficult because of differences in definitions and lack of diagnostic criteria [3]. Most studies lack information on the latency between the first acute symptomatic seizure and the first unprovoked seizure, as well as age at onset of seizures and age at diagnosis. In studying etiology, it is preferable to use incident cases, not prevalent cases, because one cannot distinguish the potential etiological factors that preceded the onset of epilepsy from those which occurred after the disease developed. Cause and effect become confused. This is why It is extremely difficult to compare results of studies of epilepsy due to NC. These studies are few, and are frequently targeted at all seizures, instead of epilepsy alone. There are broad differences in the definition of NC, as

Information available in developing countries shows that the proportion of idiopathic (60-70%) to symptomatic epilepsy (30-40%) is similar to that reported in studies from developed countries [8]. Among the symptomatic group, infection and parasitic diseases, particularly neurocysticercosis, perinatal brain damage, and head trauma are the most frequent disorders reported as a cause of epilepsy [5,42]. In a prospective cohort study of patients with newly-diagnosed epilepsy seen at the five main hospitals in the three major cities of Ecuador, perinatal brain damage (9%), neurocysticercosis (8.3%), central nervous system infections (4.2%), stroke (4.8%), and head trauma (4.2%) were the most frequent disorders reported as causes of epilepsy [42]. Although NCC is one of the most frequent antecedents among the symptomatic group, this disease is not necessarily the main cause of

Hospital-based studies to analyze NCC as etiology of epilepsy are shown in Table 1. Studies of highly selected patients with seizures in neurologic services of hospital settings from some DC reported NCC as the main cause of epilepsy, accounting for 30% to 50% of patients [11,43]. In a study carried out in 212 patients with epilepsy, a rural sub-Saharan Africa area

well as failure to define criteria for diagnosis of either seizures or epilepsy.

and metastatic brain tumors.

**5. Neurocysticercosis as etiology of epilepsy** 

epilepsy, as has been previously suggested [11,17,43,44].

So far, the mechanism by which the calcified neurocysticercal lesions (CNL) cause seizures or epilepsy is not known [1,33,34] This has been attributed to residual perilesional gliosis that results in chronic epileptogenic foci [33]**.** CNL are frequently encountered on CT scans of asymptomatic individuals, and studies from Latin American countries report that the majority are incidental lesions [30,35] These observations would question the epileptogenicity of CNL. On the contrary, based on epidemiological studies, patients with epilepsy have a higher prevalence of calcified lesions than controls [19].

Another potential evidence for the epileptogenicity of CNL is the episodic appearance of edema surrounding the CNL after seizures. Some studies have suggested that perilesional oedema is associated with episodic seizure activity in patients with CNL [36] . The authors argue that episodic release of cysticercal antigens from the calcified lesions can lead to inflammation, perilesional edema, and seizures. These studies have not been replicated so far. Anyway, it is not clear whether this edema is causal or a consequence of the seizure [34]. Transient cortical edema after seizures, a rare neuroimaging finding, was described more than 20 years ago [37,38] but its mechanism has not been entirely clarified. The empirical observation of neurologists from NCC endemic countries is that perilesional oedema around calcification in patients with epilepsy are extremely rare and, when it happens, perilesional oedema disappears in a few days without any additional treatment, except for antiepileptic drugs.

Another study designed to analyze epileptogenesis in calcified neurocysticercosis using dynamic contrast-enhanced MRI [39], reported quantitative differences between symptomatic and asymptomatic groups in various perfusion indices. Median values of the rate transfer constant and leakage volume were higher in symptomatic patients than in asymptomatic patients, indicating a higher degree of blood brain barrier permeability in symptomatic individuals. This study also showed increased MMP-9 (R279Q) gene polymorphisms in subjects with seizures compared with those in asymptomatic and control subjects. Therefore, the epileptogenicity in patients with CNL could be related to degree of inflammation, which also may be partly determined by genetic factors. This study was carried out with a small sample of patients and should be confirmed in the future.

Single enhancing lesion and seizures. A single enhancing lesion on CT (SECTL) or hyperintense lesion on MRI is a common finding in patients with newly identified seizures in developing countries [40,41]. The patients, mainly children and young adults, have some benign and transitory clinical manifestations, predominantly partial or partial secondary generalized seizures, and occasionally Todd's paresis or focal neurological deficits. These lesions have been attributed mainly to cysticercosis or to tuberculosis; however, similar lesions have been reported in other inflammatory pathologies such as pyogenic abscess, histoplasmosis, blastomycosis and sarcoidiosis, post-infectious vasculitis, and to primary and metastatic brain tumors.

The natural history of these lesions usually takes one of two forms: it becomes isodense on CT, or isointense on MR, and then either resolves entirely, or a punctuate calcification may be left as a residue [41]. The time till resolution of the lesion is quite variable ranging from a few weeks to more than a year. SECTL are benign and tend to resolve spontaneously, without anticysticercal drugs or surgery, since the parasite is already in the degenerative phase and will eventually disappear or become calcified [1]. Treatment should be limited to medication required to control the acute symptoms, such as antiepileptic medication [2].

## **5. Neurocysticercosis as etiology of epilepsy**

294 Novel Aspects on Cysticercosis and Neurocysticercosis

recurrent acute condition (transitional form) [3].

drugs.

Neurologists from developing countries frequently see patients in whom the first seizure occurred many years before consultation; and when the second seizure occurs at the time of consultation, the imaging study shows one or more calcifications and one cyst in the transitional form with perilesional edema. We can assume that when the first seizures occurred, the patient had cysts in a transitional form, which eventually became calcified, and currently the patient has new acute seizures [1]. According to the ILAE Commission we should categorize these patients as having isolated epileptic events associated with a

So far, the mechanism by which the calcified neurocysticercal lesions (CNL) cause seizures or epilepsy is not known [1,33,34] This has been attributed to residual perilesional gliosis that results in chronic epileptogenic foci [33]**.** CNL are frequently encountered on CT scans of asymptomatic individuals, and studies from Latin American countries report that the majority are incidental lesions [30,35] These observations would question the epileptogenicity of CNL. On the contrary, based on epidemiological studies, patients with

Another potential evidence for the epileptogenicity of CNL is the episodic appearance of edema surrounding the CNL after seizures. Some studies have suggested that perilesional oedema is associated with episodic seizure activity in patients with CNL [36] . The authors argue that episodic release of cysticercal antigens from the calcified lesions can lead to inflammation, perilesional edema, and seizures. These studies have not been replicated so far. Anyway, it is not clear whether this edema is causal or a consequence of the seizure [34]. Transient cortical edema after seizures, a rare neuroimaging finding, was described more than 20 years ago [37,38] but its mechanism has not been entirely clarified. The empirical observation of neurologists from NCC endemic countries is that perilesional oedema around calcification in patients with epilepsy are extremely rare and, when it happens, perilesional oedema disappears in a few days without any additional treatment, except for antiepileptic

Another study designed to analyze epileptogenesis in calcified neurocysticercosis using dynamic contrast-enhanced MRI [39], reported quantitative differences between symptomatic and asymptomatic groups in various perfusion indices. Median values of the rate transfer constant and leakage volume were higher in symptomatic patients than in asymptomatic patients, indicating a higher degree of blood brain barrier permeability in symptomatic individuals. This study also showed increased MMP-9 (R279Q) gene polymorphisms in subjects with seizures compared with those in asymptomatic and control subjects. Therefore, the epileptogenicity in patients with CNL could be related to degree of inflammation, which also may be partly determined by genetic factors. This study was

carried out with a small sample of patients and should be confirmed in the future.

Single enhancing lesion and seizures. A single enhancing lesion on CT (SECTL) or hyperintense lesion on MRI is a common finding in patients with newly identified seizures in developing countries [40,41]. The patients, mainly children and young adults, have some benign and transitory clinical manifestations, predominantly partial or partial secondary

epilepsy have a higher prevalence of calcified lesions than controls [19].

Analytical studies designed to establish associations and determinants of epilepsy have been scarce in DC. Comparing results of studies of etiology is difficult because of differences in definitions and lack of diagnostic criteria [3]. Most studies lack information on the latency between the first acute symptomatic seizure and the first unprovoked seizure, as well as age at onset of seizures and age at diagnosis. In studying etiology, it is preferable to use incident cases, not prevalent cases, because one cannot distinguish the potential etiological factors that preceded the onset of epilepsy from those which occurred after the disease developed. Cause and effect become confused. This is why It is extremely difficult to compare results of studies of epilepsy due to NC. These studies are few, and are frequently targeted at all seizures, instead of epilepsy alone. There are broad differences in the definition of NC, as well as failure to define criteria for diagnosis of either seizures or epilepsy.

Information available in developing countries shows that the proportion of idiopathic (60-70%) to symptomatic epilepsy (30-40%) is similar to that reported in studies from developed countries [8]. Among the symptomatic group, infection and parasitic diseases, particularly neurocysticercosis, perinatal brain damage, and head trauma are the most frequent disorders reported as a cause of epilepsy [5,42]. In a prospective cohort study of patients with newly-diagnosed epilepsy seen at the five main hospitals in the three major cities of Ecuador, perinatal brain damage (9%), neurocysticercosis (8.3%), central nervous system infections (4.2%), stroke (4.8%), and head trauma (4.2%) were the most frequent disorders reported as causes of epilepsy [42]. Although NCC is one of the most frequent antecedents among the symptomatic group, this disease is not necessarily the main cause of epilepsy, as has been previously suggested [11,17,43,44].

Hospital-based studies to analyze NCC as etiology of epilepsy are shown in Table 1. Studies of highly selected patients with seizures in neurologic services of hospital settings from some DC reported NCC as the main cause of epilepsy, accounting for 30% to 50% of patients [11,43]. In a study carried out in 212 patients with epilepsy, a rural sub-Saharan Africa area endemic for porcine cysticercosis [45], 2.4% were identified as definitive and 11% had lesions highly suggestive of NCC, using CT scan as part of the diagnoses. In another study from Africa, 37% of patients with epilepsy had NCC [46]. In studies from India, in which acute symptomatic seizures were excluded, only 11% (47) cases with epilepsy had NC; conversely, in a study of 572 patients with acute symptomatic seizures 67% had SECTL [48]. Another study was carried out in India [49] to determine the etiologic role of NCC in a hospital-based sample in 1026 patients with epilepsies divided according to the ILAE epidemiological criteria. NCC was diagnosed in imaging studies in 59% of those with acute symptomatic seizures, but only 2.0% with prevalent epilepsy, and none of the cases of incident epilepsy.

Neurocysticercosis and Epilepsy 297

No. (%)

15 (38)

5 (26)

Inclusion criteria Diagnosis of NCC NCC

CT scan, > 50% only 1 calcification

CT scan, All pts had only 1 calcification

CT scan, 37(37)

NCC were more frequent in individuals with seizures and in those with positive EITB for cysticercosis. Most of the patients who were diagnosed with NCC in this study had only calcifications (half of them had just one calcification); however, it is well known that brain calcifications do not necessarily mean NC. The small population samples of the above

39 All seizures

19 Recurrent

100 Recurrent

There are several studies in the medical literature that have reported an association between epilepsy and NCC based on positive serum antibodies to Taenia solium/cysticercosis [17,50,51]. Unfortunately, the presence of antibodies may indicate only previous exposure to or infection with the parasite, but not necessarily brain infection. This information has created some distortion in the current perception of NCC epidemiology. A systematic review of the literature on the frequency of NCC, diagnosed with neuroimaging, has been published [15]. Overall, 565 articles were retrieved and only 26 had reliable information to estimate the frequency of NCC in various populations worldwide. The authors concluded that the prevalence of NCC worldwide remains unknown; however, the proportion of NCC among persons with epilepsy was very consistent and estimated at 29.6% (95%CI: 23.5%– 36.1%) from 12 studies conducted in Latin America, Sub-Saharan Africa, and Southeast Asia.

**6. Inconsistencies in the relationship between neurocysticercosis and** 

with multiple cysts or calcifications but no seizures.

There are clinical inconsistencies in the link between epilepsy and NCC. Parasite location may be remote from the apparent epileptogenic region [3]. There is also no correlation between the NCC burden of lesions and the severity of the epilepsy. Patients with severe refractory seizures may have only one calcified lesion; on the other hand, there are patients

EEG has been found to be abnormal in 30-50% of patients with seizures due to NC. It is assumed that EEG findings have poor correlation with symptoms and CT lesions in patients with NCC [52.53]. A positive correlation between CT lesions and localizing or lateralizing

Prevalent cases

seizures Prevalent cases

seizures Prevalent cases

Patients with epilepsy

**Table 2.** Neurocysticercosis as an etiology of epilepsy: Community-based studies

studies do not allow generalizations.

Author Country/year

Montano, et al Perú/2005 (44)

> Del Brutto, et al Ecuador/2005 (43)

Medina et al Honduras/2005 (11)

**epilepsy** 


**Table 1.** Neurocysticercosis as etiology of epilepsy: Hospital-based studies

The above mentioned studies confirm the necessity to differentiate between acute seizure and recurrent unprovoked seizures (epilepsy). It is likely that most of the patients with NCC have acute symptomatic seizures which do not necessarily evolve into epilepsy. Similarly, it is crucial to determine NCC as etiology of epilepsy in incident cases instead of prevalent ones. These are probably some of the reasons of over diagnosis of epilepsy in some hospitalbased studies [3]

Community-based studies in which CT scan was used to diagnose NCC are shown in Table 2. In a study carried out in Ecuador [43], the authors concluded that NCC is associated with one in three cases of epilepsy and was possibly the cause of the excessive proportion of epilepsy in that population. However, only three (8%) of 24 people with epilepsy had "definitive" NCC. Another community survey [44] carried out in Peru showed that seroprevalence (using the enzyme-linked immunoelectrotransfer blot (EITB) assay] was positive in 24.2% (200/825); 15 of 39 individuals with seizures (38.5%) had lesions compatible with NCC on CT scan. The authors concluded that brain CT abnormalities compatible with NCC were more frequent in individuals with seizures and in those with positive EITB for cysticercosis. Most of the patients who were diagnosed with NCC in this study had only calcifications (half of them had just one calcification); however, it is well known that brain calcifications do not necessarily mean NC. The small population samples of the above studies do not allow generalizations.


**Table 2.** Neurocysticercosis as an etiology of epilepsy: Community-based studies

296 Novel Aspects on Cysticercosis and Neurocysticercosis
