progressive myoclonus epilepsy

progressive myoclonus epilepsy

Neurology A heterogeneous group of disorders that share clinical features, and thus generically termed PME syndrome Clinical Prominent sensitivity of myoclonus to all stimuli–eg, passive movement of a limb might evoke a generalized convulsions Etiology Unverricht-Lundborg's disease, Lafora body disease, neuronal ceroid lipofuscinosis–late infantile, juvenile, and adult types, sialidosis–types I/II, and MERRF–mitochondrial encephalopathy; rare causes of PME include Gaucher disease, GM₂ gangliosidosis, biotin-responsive encephalopathy, Hallervorden-Spatz disease, Ekbom syndrome, May-White syndrome