Progressive myoclonus epilepsies

Abstract

The progressive myoclonus epilepsy (PME) syndrome is characterized by: • a combination of fragmentary or segmental, arrhythmic, asynchronous symmetric or asymmetric myoclonus, and massive myoclonias • other epileptic seizures, usually generalized tonicclonic or clonic seizures • abnormal neurologic signs, particularly cerebellar signs • mental deterioration, which may culminate in dementia. This is a less constant component of the syndrome.1