Progressive myoclonic epilepsies.
The progressive myoclonic epilepsies are a rare but extremely debilitating group of disorders that are difficult to diagnose and even harder to treat. They represent a heterogeneous subgroup of those with secondary generalized epilepsy. Efficacy of treatment is often measured in terms of slowing a patient's inevitable decline. Reviewed here are the classification of progressive myoclonic epilepsies, features of myoclonic seizures, the five most prevalent progressive myoclonic epilepsy syndromes-Unverricht-Lundborg disease, myoclonus epilepsy with ragged red fibers (MERRF) mitochondrial disease, Lafora's disease, neuronal ceroid lipofuscinoses, and sialidoses-and current treatment options.
Conry
Joan A
JA
Children's National Medical Center, Washington, DC 20010, USA. jconry@cnmc.org
D000927:Anticonvulsants / Q000627:therapeutic use; D002648:Child; D006801:Humans; D020191:Myoclonic Epilepsies, Progressive / Q000145:classification / Q000175:diagnosis* / Q000628:therapy*; D011379:Prognosis; D016896:Treatment Outcome
D000927:Anticonvulsants