ABSTRACT

Progressive myoclonic epilepsy (PME) is a syndrome (not a specific disease) with myoclonic seizures and progressive neurological decline. The diagnosis of PME is based on the presence of a degenerative process which includes myoclonic seizures and progressive neurological dysfunction and which does not fit into any of the other myoclonic epilepsy syndromes. (Table 1). Myoclonic seizures are seen in a variety of epileptic syndromes, some benign and some malignant.