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Progressive myoclonic epilepsy

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Abstract

Progressive myoclonic epilepsies (PMEs) are a group of rare disorders characterized by the occurrence of seizures, myoclonus, and progressive neurological dysfunction. This article discusses epidemiology, genetics, pathology, clinical manifestations, EEG characteristics, methods of diagnosis and treatment of the most common causes of PME, including Unverricht-Lundborg Disease (Baltic Myoclonus), MERRF, neuronal ceroid lipofuscinosis, dentatorubropallidoluysan atrophy, Gaucher disease, Lafora disease, and sialidosis. The aim of this paper is to provide clinicians with useful clinical information in order to facilitate the diagnosis and treatment of these rare diseases.

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  1. Benjamin Legros

    Present address: Service de neurologie-Hôpital Erasme, Centre de référence pour le traitement de l’épilepsie réfractaire, 808 troute de Lennik, 1070, Brussels, Belgium

Authors and Affiliations

  1. Department of Neurology and Pediatrics, Children’s Hospital of Wisconsin, Milwaukee, Wisconsin, USA

    Mary L. Zupanc

  2. Department of Neurology, Erasme Hospital, Brussels, Belgium

    Benjamin Legros

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  1. Mary L. Zupanc
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Zupanc, M.L., Legros, B. Progressive myoclonic epilepsy. Cerebellum 3, 156–171 (2004). https://doi.org/10.1080/14734220410035356

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