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Epileptic Disorders

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Managing Lafora body disease with vagal nerve stimulation Volume 19, numéro 1, March 2017

TEST YOURSELF

(1) What are the cardinal features of LBD?

 

(2) What treatments are used in the management of LBD-related seizures?

 

(3) For which epilepsy syndrome is VNS effective?

 

 

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Answers

(1) Multiple seizure types usually including: myoclonic seizures, generalized tonic-clonic seizures, photic-induced seizures, focal dyscognitive seizures accompanied by repetitive behaviours known as automatism, and absence seizures. In addition, progressive cognitive regression and worsening seizure control occur.


(2) Although the seizures are, as a rule, drug resistant, some reports suggest that the ketogenic diet and zonisamide are, at times, useful. Other medications that can be somewhat useful include: clonazepam, levetiracetam, piracetam, phenobarbitone, topiramate, and valproate. Of note is that zonisamide has been reported in some studies to have an excellent effect in controlling generalized tonic-clonic seizures and myoclonus for 2.5 years before failing.


(3) VNS is approved as adjunctive therapy for partial seizures in individuals above 12 years of age, but has been used for drug-resistant epilepsy syndromes of various types and patients of various ages based largely on uncontrolled data and anecdotal reports. Our case is the second reported case in the literature describing the effectiveness of VNS in the management of epilepsy types in Lafora body disease. Thus, VNS may prove to be an option in the treatment plan for LBD-related seizures.

 

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