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
