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Title: Progressive myoclonus epilepsies: clinical and neurophysiological diagnosis.
Austin Authors: Berkovic, Samuel F ;So, N K;Andermann, Frederick
Affiliation: Department of Neurology, Austin Hospital, Melbourne, Australia
Issue Date: 1-Jul-1991
Publication information: Journal of Clinical Neurophysiology : Official Publication of the American Electroencephalographic Society; 8(3): 261-74
Abstract: Diagnosis and management of the progressive myoclonus epilepsies (PMEs) provides a challenge to the clinician and neurophysiologist. Over 15 specific disorders can cause the PME syndrome; all are rare, and individual physicians are unlikely to have experience in all of them. Accurate diagnosis is essential to provide a prognosis, optimal therapy, and genetic counseling. The major causes are PME of the Unverricht-Lundborg type, Lafora disease, neuronal ceroid lipofuscinoses (three forms), MERRF (myoclonus epilepsy and ragged red fibers), and sialidoses (two forms), in addition to a number of even rarer disorders. Here we review the clinical aspects and neurophysiology of these disorders, which can now be diagnosed in life by relatively simple methods in the vast majority of cases.
Gov't Doc #: 1918332
Journal: Journal of clinical neurophysiology : official publication of the American Electroencephalographic Society
Type: Journal Article
Subjects: Electroencephalography
Epilepsies, Myoclonic.diagnosis.physiopathology.therapy
Evoked Potentials
Sleep, REM.physiology
Appears in Collections:Journal articles

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