J Pediatr Epilepsy 2015; 04(03): 109-117
DOI: 10.1055/s-0035-1556734
Review Article
Georg Thieme Verlag KG Stuttgart · New York

Progressive Myoclonus Epilepsy with Adolescent Onset: Clinical Features and Diagnosis

Aimee F. Luat
1  Department of Pediatrics and Neurology, Wayne State University School of Medicine, Detroit Medical Center, Detroit, Michigan, United States
› Author Affiliations
Further Information

Publication History

25 September 2014

22 December 2014

Publication Date:
21 August 2015 (eFirst)

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.