**10. Conclusions**

Reducing the prevalence of epilepsy due to neurocysticercosis requires people working in the pig industry to make substantial behaviour changes. To interrupt the lifecycle of the *T. solium* parasite, pigs must be confined so that they cannot gain access to human stool; pork must be inspected to keep infected carcasses out of the human food chain; pork must be well cooked to ensure that cysts not observed by inspectors are killed; people who carry the tapeworm must be treated; and people must be encouraged to improve personal hygiene by using a pit latrine and washing their hands after defecating. Knowledge is power. People will be motivated to make behaviour changes when they understand the link between the pork tapeworm, pigs, pork, hygiene and epilepsy. There are financial implications to preventing epilepsy due to *T. solium* and interrupting the lifecycle. Farmers who keep their pigs confined must source feed for the pig. There is a cost to purchase some of the food and other food, such as weeds, will require the farmer to spend time collecting. The butchers must spend additional money to transport the pig to and from the slaughter slab, to use the slab and to have the government staff person inspect the carcass. If the carcass is condemned due to cysts, the butcher assumes the entire purchase price of the pig.

Workshops using a Training of the Trainers model by teaching the government staff and local community leaders who then teach the farmers are an effective way to increase knowledge and elicit long-term behaviour changes by the farmers. One-on-one training of the farmers increases the level of knowledge, in particular, for difficult concepts such as the lifecycle of the tapeworm. It is important that workshops are followed up by individual training sessions between farmers and researchers so that the material is presented more than once, in more than one training method and those farmers have an opportunity to ask questions in a safe and private environment. Workshops for the pork butchers in which the education is linked to business practice were particularly important. The butchers acquired useful information about how to track and understand the costs, income, and profits of their business. They learned about their role in preventing epilepsy due to neurocysticercosis and felt responsible for ensuring the safety of the pork they sell. The butchers did change their behaviour by having a higher proportion or all of the pork carcasses inspected prior to sale. Both farmers and butchers changed how they cooked the pork by boiling it for 20 minutes prior to consumption or sale. Together, the systematic changes across the entire pig industry are expected to reduce the prevalence of epilepsy in these communities. This model needs to be repeated around the world, wherever pigs are kept in a free range manner and neurocysticercosis occurs.

### **11. References**

218 Novel Aspects on Epilepsy

Four years after the study began, farmers and butchers were boiling the pork for 20 minutes before they fried the meat. The larval cyst will be killed if it is cooked at a boiling temperature for 10 minutes. Farmers said they purchased pork from butchers rather than from backyard operations because they wanted to buy meat that was inspected. Butchers regularly took the pigs to the slaughter slab to have them inspected prior to sale. The business education was important to the butchers because they kept track of their expenses and were sure to save enough money from each pig to pay for the inspection of the next pig. In a World Bank project in Kenya found that over 80% of farmers who are taught recommended practices choose to adopt them (Gautam 2000). This education had both a

Reducing the prevalence of epilepsy due to neurocysticercosis requires people working in the pig industry to make substantial behaviour changes. To interrupt the lifecycle of the *T. solium* parasite, pigs must be confined so that they cannot gain access to human stool; pork must be inspected to keep infected carcasses out of the human food chain; pork must be well cooked to ensure that cysts not observed by inspectors are killed; people who carry the tapeworm must be treated; and people must be encouraged to improve personal hygiene by using a pit latrine and washing their hands after defecating. Knowledge is power. People will be motivated to make behaviour changes when they understand the link between the pork tapeworm, pigs, pork, hygiene and epilepsy. There are financial implications to preventing epilepsy due to *T. solium* and interrupting the lifecycle. Farmers who keep their pigs confined must source feed for the pig. There is a cost to purchase some of the food and other food, such as weeds, will require the farmer to spend time collecting. The butchers must spend additional money to transport the pig to and from the slaughter slab, to use the slab and to have the government staff person inspect the carcass. If the carcass is

condemned due to cysts, the butcher assumes the entire purchase price of the pig.

Workshops using a Training of the Trainers model by teaching the government staff and local community leaders who then teach the farmers are an effective way to increase knowledge and elicit long-term behaviour changes by the farmers. One-on-one training of the farmers increases the level of knowledge, in particular, for difficult concepts such as the lifecycle of the tapeworm. It is important that workshops are followed up by individual training sessions between farmers and researchers so that the material is presented more than once, in more than one training method and those farmers have an opportunity to ask questions in a safe and private environment. Workshops for the pork butchers in which the education is linked to business practice were particularly important. The butchers acquired useful information about how to track and understand the costs, income, and profits of their business. They learned about their role in preventing epilepsy due to neurocysticercosis and felt responsible for ensuring the safety of the pork they sell. The butchers did change their behaviour by having a higher proportion or all of the pork carcasses inspected prior to sale. Both farmers and butchers changed how they cooked the pork by boiling it for 20 minutes prior to consumption or sale. Together, the systematic changes across the entire pig industry are expected to reduce the prevalence of epilepsy in these communities. This model needs to be repeated around the world, wherever pigs are kept in a free range manner and

positive short- and long-term impact (Gautam 2000 ).

**9. Safe pork** 

**10. Conclusions** 

neurocysticercosis occurs.


**13** 

*Italy* 

**Myoclonic Epilepsy in Lysosomal** 

**Storage Disorders** 

Andrea Dardis and Bruno Bembi

*Regional Coorddinator Centre for Rare Diseases* 

*University Hospital "Santa Maria della Misericordia" of Udine Piazzale, Santa Maria della Misericordia, Udine,* 

Progressive myoclonic epilepsy (PME) constitutes an heterogeneous group of diseases, usually of genetic origin, which begins in childhood and adolescence and presents a variable evolution, ranging from slowly to rapidly progressive forms with refractory seizures and dead within few years (Marseille Consensus Group, 1990). Despite its broad spectrum of manifestations, patients affected with PME share some common specific clinical and electrophysiological features, such as: myoclonus, multiple type of seizures, delay or regression of psychomotor development, cerebellar ataxia, slow background activity on electroencephalogram (EEG), spikes and waves induced by intermittent photo-stimulation

From the genetic point of view, PME occurs in disorders presenting different genetic inheritance, including: the dentatorubralpallidolusyian atrophy (DRPLA), a disease of trinucleotide repeats, the myoclonic epilepsy with ragged red fibers (MERRF), a mitochondrial disease and autosomal recessive disorders, which may be divided in two main categories: non-lysosomal-related diseases such as Lafora disease and lysosomal-

Lysosomal storage disorders are severe genetic diseases caused by the defective activity of lysosomal proteins, cofactors or integral membrane proteins, which result in the intralysosomal accumulation of undegraded metabolites such as sphingolipids, cholesterol, glycoproteins, mucopolysaccharides or glycogen. Even if they are individually rare, the combined frequency of LSDs is estimated to be approximately 1 in 8000 live births (Meilke et al., 1999; Poorthuis et al., 1999; Applegarth et al., 2000; Dionisi-Vici et al., 2002; Pinto et al.,

More than 50 LSDs have been described to date (Staretz-Chacham et al., 2009). Although they are characterized by a wide spectrum of clinical phenotypes, many of these disorders present with severe progressive neurological impairment. Among the neurological symptoms, the presence of PME has been reported in different LSDs, including Gaucher disease, action myoclonus-renal failure syndrome, neuronal ceroid lipofuscinoses, sialidosis, Niemann Pick type C disease, and GM2 gangliosidosis. Each of these LSDs is characterized by a series of specific sings and symptoms. However, many of them share some clinical and biochemical features, such as the presence of signs of neurological impairments other than PME or organ disorders, which may be useful in the diagnosis of patients presenting with PME due to LSD.

and sensory evoked giant potentials (Marseille Consensus Group, 1990).

related-disease such as lysosomal storage disorders (LSDs).

**1. Introduction** 

2004; Poupetova et al., 2010).

