Efficacy of levetiracetam in a patient with Unverricht-Lundborg progressive myoclonic epilepsy

The progressive myoclonic epilepsies (PME) are a group of symptomatic generalized epilepsies associated with severe myoclonus, intractable generalized tonic-clonic seizures, and variable cognitive impairment. Specific subtypes include mitochondrial disorders, the ceroid lipofuscinoses, Lafora body disease, and Unverricht-Lundborg disease. The latter is autosomal recessive and recently shown to be due to an underlying mutation in the gene encoding for cystatin-B ( CTSB ), a cysteine protease inhibitor, on chromosome 21q22.1 Correlation between the mutated protein and disease pathogenesis is poorly understood. Treatment of PME is difficult with a variable and usually unsatisfactory response to benzodiazepines, sodium valproate, and piracetam, and new therapies are needed. We treated a patient with the newly released anticonvulsant levetiracetam. This drug is a piracetam analogue with demonstrated efficacy …