Long-term observations of two siblings with Lafora disease treated with zonisamide

Abstract

We have reported long-term clinical follow-up for two siblings with Lafora disease, a brother and sister, one of whom autopsied. Both siblings had repeated attacks of severe myoclonus, tonic and tonic–clonic convulsions, and intractable status epilepticus. The addition of orally administered zonisamide brought about striking effective seizure control for about 12–14 years in both patients, relieving not only myoclonus and generalized tonic–clonic seizures but also intractable status epilepticus.