title: 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.
authors.affiliation: Department of Neuropsychiatry, Hirosaki University School of Medicine, 5 Zaifu-cho, Hirosaki 036-8562, Japan.
authors.forename: I
authors.initials: I
authors.lastname: Yoshimura
authors.forename: S
authors.initials: S
authors.lastname: Kaneko
authors.forename: N
authors.initials: N
authors.lastname: Yoshimura
authors.forename: T
authors.initials: T
authors.lastname: Murakami
mesh_terms: D000328:Adult; D000927:Anticonvulsants / Q000627:therapeutic use*; D017809:Fatal Outcome; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007555:Isoxazoles / Q000627:therapeutic use*; D020192:Lafora Disease / Q000188:drug therapy* / Q000503:physiopathology; D008297:Male; D009679:Nuclear Family; D013226:Status Epilepticus / Q000188:drug therapy / Q000503:physiopathology; D000078305:Zonisamide
chemical_list: D000927:Anticonvulsants; D007555:Isoxazoles; D000078305:Zonisamide