**1. Introduction**

172 Novel Aspects on Epilepsy

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949.

Delhi.

#### **1.1 Background**

Neurocysticercosis is eradicable parasitic zoonoses of the brain if it is manages by public health sector and agricultural sector with the dedicated support of veterinarian doctors. Neurocysticercosis is the only zoonotic infection which has been considered as eradicable by the World Health Assembly. Therefore, we will focus on that, and other parasitic zoonoses causing epilepsy will be discussed in another chapter. In this chapter new aspect about insular epilepsy secondary to NC will be introduced as well.

Like other pathological disorders, early diagnosis and treatment can significantly decrease morbidity and mortality rates of parasitic infections. Diseases that have their origins in infected animals, such as H1N1 influenza or SARS have highlighted the need for a better understanding of their origin on an affected animal.

The ease and speed of modern travel facilitates the spread of diseases once confined to specific geographic areas, as recently occurred with the widely publicized H1N1 influenza. Animal migration and trade pose a similar threat, as was shown by the outbreaks in the United States of West Nile fever, and monkey-pox, two diseases haven't previously known in the Western Hemisphere. Each of these examples highlights the need for accurate, up-todate information and ongoing research on those public health problems. (Carabin, personal communication, 2010)

Pig farming has increased considerably during the past decade in Eastern and Southern Africa (ESA); especially in rural, resource-poor, smallholder communities where sanitation is poor. Hence, it is highly suspected that the frequency of epilepsy secondary to NC in the region may further increase in the foreseeable future. We see a lot of pigs affected by cysticercosis free of neurological signs but when there is a sign it can indicate the etiology of human's disease. Let us to address it in a better way, for example pigs with NC do not suffer of epilepsy, however presence of cysticercosis of pigs (intermediate host) indicates that NC is the most likely cause of epilepsy of peoples living around, being yet another reason to support that health worker and agriculture worker should work together in this field. In places where there are not clinical health laboratory facilities and CT/MR images or simply patients have not free to these investigations and the prevalence of epilepsy is considerably high we do suggest to confirm diagnosis of cysticercosis on pig's population by physical inspection of the tongue (Figure 1).

Clinical Features of Epilepsy Secondary to Neurocysticercosis at the Insular Lobe 175

*Taenia solium* cysticercosis' life cycle starts when humans become infected by eating undercooked pork containing cysticerci (See figure 3) and later they develop taeniosis. People with taeniosis pass eggs with their faeces which are ingested by humans and pigs.

Fig. 3. Shows a maseter muscle from infected pig containing numerous cysticerci. Photo

When only a few measles are found the meat is sweet, but when numerous, the meat

Seems to be that measly pork meat has been described long ago according to this sentence

Eggs develop into larval cysts causing human and porcine cysticercosis. Risk factors for taeniosis include the consumption of undercooked infected pork meat and inadequate meat inspection. Risk factors of cysticercosis include free-range pig farming, poor sanitation, close contact of humans and pigs and inadequate hygiene of food handlers. (See graphic 1)

Graphic 1. Shows the life cycle of T Solium cysticercosis and the path toward to NC. Graphic

taken by Prof. A Lee (CWGESA).

found in ancient documents:

In 384-323 BC, Aristotle said:

becomes watery and unpalatable."

modified from by Prof. Carabin. (CWGESA)

Fig. 1. A cystic lesion on the tongue in a pig affected by cysticercosis is pointed by the yellow arrow. Photo taken by Prof. RC Krecek (CWGESA)

The other choice is to take blood samples from jugular veins (Figure 2) and request ELISA for cysticercosis at the nearest veterinarian laboratory. It may help suspecting the etiological diagnosis of epilepsy in these patients and to support their treatment.

Fig. 2. Shows agricultural workers and Prof. Foyaca taken a blood sample from the jugular veins in infected pig. (Photo taken by Prof RC Krecek)

*Taenia solium* cysticercosis' life cycle starts when humans become infected by eating undercooked pork containing cysticerci (See figure 3) and later they develop taeniosis. People with taeniosis pass eggs with their faeces which are ingested by humans and pigs.

Fig. 3. Shows a maseter muscle from infected pig containing numerous cysticerci. Photo taken by Prof. A Lee (CWGESA).

Seems to be that measly pork meat has been described long ago according to this sentence found in ancient documents:

In 384-323 BC, Aristotle said:

174 Novel Aspects on Epilepsy

Fig. 1. A cystic lesion on the tongue in a pig affected by cysticercosis is pointed by the

Fig. 2. Shows agricultural workers and Prof. Foyaca taken a blood sample from the

jugular veins in infected pig. (Photo taken by Prof RC Krecek)

The other choice is to take blood samples from jugular veins (Figure 2) and request ELISA for cysticercosis at the nearest veterinarian laboratory. It may help suspecting the etiological

yellow arrow. Photo taken by Prof. RC Krecek (CWGESA)

diagnosis of epilepsy in these patients and to support their treatment.

When only a few measles are found the meat is sweet, but when numerous, the meat becomes watery and unpalatable."

Eggs develop into larval cysts causing human and porcine cysticercosis. Risk factors for taeniosis include the consumption of undercooked infected pork meat and inadequate meat inspection. Risk factors of cysticercosis include free-range pig farming, poor sanitation, close contact of humans and pigs and inadequate hygiene of food handlers. (See graphic 1)

Graphic 1. Shows the life cycle of T Solium cysticercosis and the path toward to NC. Graphic modified from by Prof. Carabin. (CWGESA)

Clinical Features of Epilepsy Secondary to Neurocysticercosis at the Insular Lobe 177

Fig. 4. CT scan of the brain showing multiple active cystic lesions and their scolex inside.

evidence of household contact with peoples with cysticercosis

For the present study, definite NC will be defined as: 1 absolute criteria or 2 major criteria or 1 major, 2 minor and 1 epidemiologic criterion; probable NC as: 1 major and 2 minor criteria or 1 major, 1 minor and 1 epidemiologic criterion or three minor and one epidemiologic criterion; and possible NC as: 1 major criteria or 2 minor criteria or 1 minor and 1

The pilot study conducted by us at the Nelson Mandela Academic Hospital consisted of asking 57 epilepsy patients with confirmed NC, 52 epileptic patients without NC and 61 patients from the dermatology and ophthalmology clinics to answer a questionnaire interview about epilepsy and pig raising and management. A very preliminary analysis suggests that, using dermatology and ophthalmology clinic patients as the reference group, the POR of owning pigs and having NC was 6.8 (95% CI: 2.1-21.7) and the POR for consuming pork compared to never consume pork and having NC was 2.1 (0.7-6.2). Using the epilepsy patients without NC as the reference group, the POR of consuming pork in those with NC was 14.2 (5.1-39.5) and of owning pigs was 17.5 (5.4-56.2). Why the association between owning pigs and NC was stronger when the epilepsy patients were

Patient presenting tonic-clonic generalize and recurrent frontal lobe seizures.

lesions compatible with NC on neuroimaging studies

clinical manifestations of NC cysticercosis outside the CNS

residence in a cysticercosis endemic area visiting an endemic area for cysticercosis

**Minor criteria:** 

**Epidemiological criteria:** 

epidemiologic criterion.

Cysticercosis is thus strongly associated with poverty and other socio-economic-cultural problems (Del Rio & Foyaca-Sibat, 2005, 2005a, 2007, 20008; Foyaca-Sibat & Del Rio, 2004, 2005, 2005a). In both humans and pigs, cysts migrate mostly to the subcutaneous tissue, skeletal muscle, the eye, and the central nervous system (CNS). Currently, NC is not only the major cause of acquired epilepsy in many developing countries, but is also of growing concern in northern/western countries due to globalization and immigration of infected people as before-cited.

In South Africa, epilepsy secondary to NC is quite common in ECP particularly in the poor, former black homeland, rural areas of the former 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 contact of humans and pigs and inadequate hygiene of food handlers.

Consumption of uninspected pork meat is undoubtedly a major source of human taeniasis. The transmission of *T. solium* to pigs, the essential partner in the pig-man-pig cycle, requires that pigs have access to human feces and that people consume improperly cooked pork.

The major risk factors related to transmission of eggs to pigs can be summarized as follows:


Outdoor human defecation near or in pig rearing areas

Use of pigs to scavenge and eat human feces ("sanitary policeman")

Deliberate use of human feces as pig feed

Connection of pig pens to human latrines ("pig sty privies");

Use of sewage effluent, sludge or "night soil" to irrigate and/or fertilize pig pastures and food crops

Involvement of humans' carriers in pig rearing and care.

The diagnostic assessment for NC was proposed by Del Brutto et al., (2001), and we used an abbreviated set of these criteria for the definition of NC in our studies.

#### **Absolute criteria include:**


Other criteria are:

#### **Major criteria:**


Fig. 4. CT scan of the brain showing multiple active cystic lesions and their scolex inside. Patient presenting tonic-clonic generalize and recurrent frontal lobe seizures.

#### **Minor criteria:**

176 Novel Aspects on Epilepsy

Cysticercosis is thus strongly associated with poverty and other socio-economic-cultural problems (Del Rio & Foyaca-Sibat, 2005, 2005a, 2007, 20008; Foyaca-Sibat & Del Rio, 2004, 2005, 2005a). In both humans and pigs, cysts migrate mostly to the subcutaneous tissue, skeletal muscle, the eye, and the central nervous system (CNS). Currently, NC is not only the major cause of acquired epilepsy in many developing countries, but is also of growing concern in northern/western countries due to globalization and immigration of infected

In South Africa, epilepsy secondary to NC is quite common in ECP particularly in the poor, former black homeland, rural areas of the former 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

Consumption of uninspected pork meat is undoubtedly a major source of human taeniasis. The transmission of *T. solium* to pigs, the essential partner in the pig-man-pig cycle, requires that pigs have access to human feces and that people consume improperly cooked pork. The major risk factors related to transmission of eggs to pigs can be summarized as follows:

Use of sewage effluent, sludge or "night soil" to irrigate and/or fertilize pig pastures and

The diagnostic assessment for NC was proposed by Del Brutto et al., (2001), and we used an

cystic lesions showing the scolex on CT or MRI (See figure 4)

demonstration of T solium from biopsied specimens

cystic lesions without scolex, enhancing lesions

raising pigs contact of humans and pigs and inadequate hygiene of food handlers.

people as before-cited.

food crops

**Absolute criteria include:** 

Other criteria are: **Major criteria:** 

Extensive or free-range pig rearing

Deliberate use of human feces as pig feed

Outdoor human defecation near or in pig rearing areas

Connection of pig pens to human latrines ("pig sty privies");

Involvement of humans' carriers in pig rearing and care.

Use of pigs to scavenge and eat human feces ("sanitary policeman")

abbreviated set of these criteria for the definition of NC in our studies.

visualization of *T solium* on fundoscopy

typical parenchyma brain calcifications positive serum Ag-ELISA for cysticercosis


#### **Epidemiological criteria:**


For the present study, definite NC will be defined as: 1 absolute criteria or 2 major criteria or 1 major, 2 minor and 1 epidemiologic criterion; probable NC as: 1 major and 2 minor criteria or 1 major, 1 minor and 1 epidemiologic criterion or three minor and one epidemiologic criterion; and possible NC as: 1 major criteria or 2 minor criteria or 1 minor and 1 epidemiologic criterion.

The pilot study conducted by us at the Nelson Mandela Academic Hospital consisted of asking 57 epilepsy patients with confirmed NC, 52 epileptic patients without NC and 61 patients from the dermatology and ophthalmology clinics to answer a questionnaire interview about epilepsy and pig raising and management. A very preliminary analysis suggests that, using dermatology and ophthalmology clinic patients as the reference group, the POR of owning pigs and having NC was 6.8 (95% CI: 2.1-21.7) and the POR for consuming pork compared to never consume pork and having NC was 2.1 (0.7-6.2). Using the epilepsy patients without NC as the reference group, the POR of consuming pork in those with NC was 14.2 (5.1-39.5) and of owning pigs was 17.5 (5.4-56.2). Why the association between owning pigs and NC was stronger when the epilepsy patients were

Clinical Features of Epilepsy Secondary to Neurocysticercosis at the Insular Lobe 179

Forced blinking and eyelid flutter follow by horizontal gaze deviation.

Calcified NC with or without perilesional edema is the most common cause. This presentation is uncommon despite it has been underestimated importantly. It can be associated to insula epilepsy or other types of epilepsy mainly in multiple calcified NC. Seems to be that measly pork meat has been described long ago according to this sentence.

Menon (2007) reported two young patients with symptomatic occipital lobe epilepsy due to discrete lesions of cysticercosis were misdiagnosed and treated for 2 years as migraine with visual aura. The patients suffered from frequent visual seizures often followed by migrainelike headache. Seizures manifested with colored and mainly circular elementary visual hallucinations of up to 1 minute duration. Headache, often severe and of long duration, was frequently associated with nausea, photophobia, and phonophobia. Both patients became seizure-free with appropriate treatment of the underlying disease and epileptic seizures.

Clinical features of epileptic seizures may help to identify the specific frontal region of

*Supplementary motor area*= Unilateral or asymmetric bilateral tonic posturing and facial grimacing, vocalization, or speech arrest; somatosensory aura; and complex automatisms

*Primary motor cortex=* focal simple motor seizures with clonic or myoclonic movements *Medial frontal, cingulate gyrus, orbitofrontal, or frontopolar regions=*Complex behavioural events (motor agitation and gestural automatisms) ; viscerosensory symptoms and strong emotional

*Operculum=* Swallowing, salivation, mastication, epigastric aura, fear, and speech arrest

This modality of seizures often bizarre and diagnosed incorrectly as psychogenic and can be associated to insular seizures. Despite calcified NC is the commonest cause no always locations of the lesions cause same clinical manifestations and quite often some lesions

Dystonic posturing, jerking, bending, and rocking; difficult to distinguish from

feelings; pelvic thrusting, pedalling, or thrashing, vocalizations, laughter, or crying. *Dorsolateral cortex=*- Tonic posturing or clonic movements often associated with either

contralateral head and eye deviation, or less commonly, ipsilateral head turn

often associated with clonic facial movements and gustatory hallucinations.

Patients presenting nocturnal occipital lobe epilepsy (NFLE) usually report

The frenetic or agitated appearance of onset and normal intelligent

The history of similar events with other family members (Autosomal dominant)

Structured visual hallucinations and moving colored shapes

Flashes light and transitory cortical blindness

**2.2 Frontal lobe epilepsy secondary to neurocysticercosis** 

*Dominant hemisphere involvement*= Prominent speech disturbances

onset. (So, 1998; Kotagal & Arunkumar, 1998) as follow:

such as kicking, laughing, or pelvic thrusting

remain silent for years.

parasomnias

**2.2.1 Nocturnal frontal lobe epilepsy** 

Seizure clusters occurring only during sleep

The medication of choice is carbamazepine.

used as a reference group needs further investigation, but it is possible that the NC and 2 the non-epilepsy groups came from rural areas whereas the epilepsy non-NC group came from more urban areas with less exposure to infected pigs. Further analyses will determine whether these results are confounded by the age of the patients or where they live (i.e. Persons who only had epilepsy may be more likely to live in more urbanized areas). We also participated in the pilot study conducted at the St-Elisabeth hospital from ECP in SA. We studied 433 consecutive patients consulting the outpatient clinic for suspected new-onset seizures or existing epilepsy cases. (Foyaca-Sibat et al., 2009)

Of these, 281 were diagnosed as recurrent focal or generalized motor seizures due to secondary epilepsy. Each consenting participant was administered the questionnaire and asked to provide a blood sample for a serological analysis of antibodies to and antigens of the larval stage of *T. solium*. Additionally, each week for consenting, randomly selected patients with the confirmed diagnosis of seizures disorder were transported to Mthatha for a CT scan of the brain and EEG. Among these 281 patients, the prevalence of seropositivity of antibodies to the larval stage of *T. solium* was 33% (95% CI: 27%-38%) in the 273 tested. Serological antigens were available for 189 patients with confirmed seizures or epilepsy. In this group the prevalence of seropositivity to antigens to *T. solium* was 8% (95%CI: 4.5%- 13%). Modified from the original one done by Prof. Carabin. A total of 92 patients with recurrent seizures and who also completed a questionnaire were referred to Mthatha for a CT-scan. Of these, 34 (37.0%, 95%CI: 27.1%-47.7%) had a definite diagnosis of NC, 14 of whom had active lesions visible on CT, 39 (42%) had no CT abnormality, and 19 (21%) had other, undefined non-NC calcifications. The age of the NC cases ranged from 5 to 67 years old whereas the epilepsy cases ranged from 5 to 76 years old. Antibody ELISA results were available for 33 of the 34 patients classified as probable NC and for all 39 without NC. The predictive value of a positive antibody test in identifying NC in persons with epilepsy was 60% (95% CI: 41%-77%). Serological results for antigen ELISA were available for 23 confirmed NC cases and 22 non-NC cases. The predictive value of a positive antigen test in identifying NC in persons with epilepsy was 67% (95% CI: 22%-96%). Thus, it is clear that serology alone cannot be used to diagnose NC in this population. HIV status was available from 50 patients with epilepsy. Among the 47 patients with antibody ELISA results available, the antibody seroprevalence of T. solium was 30.0% among HIV positive patients and 48.1% among HIV negative patients. Interestingly, among the 33 patients with antigen ELISA results, the antigen seroprevalence of *T. solium* was 16.7% among the HIV positive patients but only 9.5% among the HIV negative patients. Even though these results are based on a very small sample, it does suggest that HIV patients may be less able to mount a detectable antibody response to cysticercosis but might be more likely to be infected with active cysts. A total of 22 of these patients (13 HIV negative and 9 HIV positive) were referred for a CT-scan. Of these, five HIV negative and seven HIV positive patients had CT evidence of NC with two HIV negative and five HIV positive patients harboring active cysts. These very preliminary and imprecise results do suggest that there may be an association between NC and HIV infection. (Foyaca-Sibat et al., 2009)

#### **2. Other types of epilepsy secondary to neurocysticercosis**

#### **2.1 Occipital lobe epilepsy secondary to neurocysticercosis**

In our series patients presenting occipital lobe epilepsy usually is reporting

used as a reference group needs further investigation, but it is possible that the NC and 2 the non-epilepsy groups came from rural areas whereas the epilepsy non-NC group came from more urban areas with less exposure to infected pigs. Further analyses will determine whether these results are confounded by the age of the patients or where they live (i.e. Persons who only had epilepsy may be more likely to live in more urbanized areas). We also participated in the pilot study conducted at the St-Elisabeth hospital from ECP in SA. We studied 433 consecutive patients consulting the outpatient clinic for suspected new-onset

Of these, 281 were diagnosed as recurrent focal or generalized motor seizures due to secondary epilepsy. Each consenting participant was administered the questionnaire and asked to provide a blood sample for a serological analysis of antibodies to and antigens of the larval stage of *T. solium*. Additionally, each week for consenting, randomly selected patients with the confirmed diagnosis of seizures disorder were transported to Mthatha for a CT scan of the brain and EEG. Among these 281 patients, the prevalence of seropositivity of antibodies to the larval stage of *T. solium* was 33% (95% CI: 27%-38%) in the 273 tested. Serological antigens were available for 189 patients with confirmed seizures or epilepsy. In this group the prevalence of seropositivity to antigens to *T. solium* was 8% (95%CI: 4.5%- 13%). Modified from the original one done by Prof. Carabin. A total of 92 patients with recurrent seizures and who also completed a questionnaire were referred to Mthatha for a CT-scan. Of these, 34 (37.0%, 95%CI: 27.1%-47.7%) had a definite diagnosis of NC, 14 of whom had active lesions visible on CT, 39 (42%) had no CT abnormality, and 19 (21%) had other, undefined non-NC calcifications. The age of the NC cases ranged from 5 to 67 years old whereas the epilepsy cases ranged from 5 to 76 years old. Antibody ELISA results were available for 33 of the 34 patients classified as probable NC and for all 39 without NC. The predictive value of a positive antibody test in identifying NC in persons with epilepsy was 60% (95% CI: 41%-77%). Serological results for antigen ELISA were available for 23 confirmed NC cases and 22 non-NC cases. The predictive value of a positive antigen test in identifying NC in persons with epilepsy was 67% (95% CI: 22%-96%). Thus, it is clear that serology alone cannot be used to diagnose NC in this population. HIV status was available from 50 patients with epilepsy. Among the 47 patients with antibody ELISA results available, the antibody seroprevalence of T. solium was 30.0% among HIV positive patients and 48.1% among HIV negative patients. Interestingly, among the 33 patients with antigen ELISA results, the antigen seroprevalence of *T. solium* was 16.7% among the HIV positive patients but only 9.5% among the HIV negative patients. Even though these results are based on a very small sample, it does suggest that HIV patients may be less able to mount a detectable antibody response to cysticercosis but might be more likely to be infected with active cysts. A total of 22 of these patients (13 HIV negative and 9 HIV positive) were referred for a CT-scan. Of these, five HIV negative and seven HIV positive patients had CT evidence of NC with two HIV negative and five HIV positive patients harboring active cysts. These very preliminary and imprecise results do suggest that there may be an

seizures or existing epilepsy cases. (Foyaca-Sibat et al., 2009)

association between NC and HIV infection. (Foyaca-Sibat et al., 2009)

**2. Other types of epilepsy secondary to neurocysticercosis** 

In our series patients presenting occipital lobe epilepsy usually is reporting

**2.1 Occipital lobe epilepsy secondary to neurocysticercosis** 


Calcified NC with or without perilesional edema is the most common cause. This presentation is uncommon despite it has been underestimated importantly. It can be associated to insula epilepsy or other types of epilepsy mainly in multiple calcified NC. Seems to be that measly pork meat has been described long ago according to this sentence. The medication of choice is carbamazepine.

Menon (2007) reported two young patients with symptomatic occipital lobe epilepsy due to discrete lesions of cysticercosis were misdiagnosed and treated for 2 years as migraine with visual aura. The patients suffered from frequent visual seizures often followed by migrainelike headache. Seizures manifested with colored and mainly circular elementary visual hallucinations of up to 1 minute duration. Headache, often severe and of long duration, was frequently associated with nausea, photophobia, and phonophobia. Both patients became seizure-free with appropriate treatment of the underlying disease and epileptic seizures.

### **2.2 Frontal lobe epilepsy secondary to neurocysticercosis**

Clinical features of epileptic seizures may help to identify the specific frontal region of onset. (So, 1998; Kotagal & Arunkumar, 1998) as follow:

*Dominant hemisphere involvement*= Prominent speech disturbances

*Supplementary motor area*= Unilateral or asymmetric bilateral tonic posturing and facial grimacing, vocalization, or speech arrest; somatosensory aura; and complex automatisms such as kicking, laughing, or pelvic thrusting

*Primary motor cortex=* focal simple motor seizures with clonic or myoclonic movements

*Medial frontal, cingulate gyrus, orbitofrontal, or frontopolar regions=*Complex behavioural events (motor agitation and gestural automatisms) ; viscerosensory symptoms and strong emotional feelings; pelvic thrusting, pedalling, or thrashing, vocalizations, laughter, or crying.

*Dorsolateral cortex=*- Tonic posturing or clonic movements often associated with either contralateral head and eye deviation, or less commonly, ipsilateral head turn

*Operculum=* Swallowing, salivation, mastication, epigastric aura, fear, and speech arrest often associated with clonic facial movements and gustatory hallucinations.

This modality of seizures often bizarre and diagnosed incorrectly as psychogenic and can be associated to insular seizures. Despite calcified NC is the commonest cause no always locations of the lesions cause same clinical manifestations and quite often some lesions remain silent for years.

#### **2.2.1 Nocturnal frontal lobe epilepsy**

Patients presenting nocturnal occipital lobe epilepsy (NFLE) usually report


Clinical Features of Epilepsy Secondary to Neurocysticercosis at the Insular Lobe 181

of the mouth, and oropharyngeal swallowing (anterior insular) are not well known neither, and almost nothing has been demonstrated about the role of the insular lobe over the amygdala complex and emotional behavior. The human IL is also considered as paralimbic cortex, because of its connections with limbic and sensorimotor cortices, the IL is believed to play a role in affective and attention aspects of human behavior as well. Paralimbic insular regions have functional specialization for behaviors requiring integration between extra personal stimuli and the internal milieu. Based on these connections, one might expect that lesions of the insular cortex may result in disorders of neglect (Foyaca-Sibat & Ibañez-

Insular lobe epilepsy (ILE) and insular lobe seizures (ILS) are still not included in the current classification for epileptic seizures, epilepsy or epileptic syndromes belong to "The International League Against Epilepsy" therefore most of neurologists, epileptologists, clinician pediatrician, and general practitioner do not include this entity in their list of differential diagnosis in patients presenting "aberrant" types of temporal lobe epilepsy (TLE), "stereotype" simple focal seizures and others. Insular seizures may mimic temporal,

The electroencephalographic (EEG) studies of the insula lobe (IL) are not confident because it is the only cortical part of the brain that is not accessible at the surface of the cerebral hemisphere, because it is totally covered by the fronto-parietal and temporal opercula,

The insula is one of the five cerebral lobes and its cortex is situated deep within each hemisphere. It is overlayed by the frontal and temporal neocortex and this explains how difficult it should be to get a reliable EEG sampling from the insular cortex and to define an "insular epileptic syndrome" as has been done with temporal lobe epilepsy. Adequate sampling from the insula can only be obtained by depth or subdural electrodes' implantation or acute intraoperative electrocorticography. Depth or subdural electrodes implantation of the insular faces some technical problems. There is substantial evidence that the insula is involved as a somesthetic area, including a major role in the process of nociceptive input. The role of the insula in some epileptic patients was recently investigated by means of depth electrode recordings made following Talairach's stereoelectroencephalography (SEEG) methodology. It appears that ictal signs associated with an insular discharge is very similar to those usually attributed to mesial temporal lobe seizures (Robles et al, 2009]) others authors reported: sensation of laryngeal constriction and paresthesiae, often unpleasant, affecting large cutaneous territories, most often at the onset of a complex partial seizure (five of the six patients) as a common presentation [ Isnard J, et al., 2004] while other said: the most common clinical feature associated with damage to the insula is the complex partial seizures with involvement of the visceral sensations (Duffau H, et al., 2002). Different authors reported ictal symptoms associated with insular discharges mainly made up of respiratory, viscerosensitive (chest or abdominal constriction), or oroalimentary (chewing or swallowing) manifestations. Unpleasant somatosensory manifestation always opposite the discharging side, are also frequent and they concluded that Ictal signs arising from the insula occur in full consciousness; these are always simple

parietal or frontal lobe seizures and may coexist with seizures from other lobes.

therefore accuracy of EEG made by surface electrode is uncertain.

Valdés, 2006).

**3.1 Insula lobe epilepsy 3.1.1 Background** 

With nocturnal frontal lobe epilepsy, seizures begin shortly after falling asleep or in the early hours before awakening with a gasp, grunt, hums, moan or word, and are followed by sudden thrashing movements. Patients remain conscious but can neither control the movements nor speak. Thrashing can be vigorous enough to throw the patient out of bed, which can result in possible injury. Nocturnal frontal lobe epilepsy is typically treated with carbamazepine and, in some cases, surgery. We did not identify this type of seizures in our series. If there is evidence of NC on imagenology then a diagnosis of NFLE is ruled out.
