Abstract
Many of the progressive epilepsy syndromes with adolescent onset are classified as
progressive myoclonus epilepsies. It is a heterogenous group of epilepsy syndromes
associated with seizure, myoclonus, and progressive neurologic decline. This review
focuses on the different adolescent-onset progressive myoclonus epilepsies including
Unverricht–Lundborg disease, Lafora disease, juvenile-onset neuronal ceroid lipofuscinosis,
sialidosis (cherry-red spot myoclonus), dentatorubral–pallidoluysian atrophy, and
myoclonic epilepsy and ragged-red fibers. Their clinical presentations, neurophysiologic
and neuroimaging findings, genetics, pathology, and diagnosis are discussed.
Keywords
progressive myoclonus - epilepsy - adolescent