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Japanese

Progressive myoclonus epilepsies and DRPLA. Haruhiko NAITO 1 1Department of Psychiatry, School of Medicine, Niigata University pp.56-67
Published Date 1990/2/10
DOI https://doi.org/10.11477/mf.1431900006
  • Abstract
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 The Progressive Myoclonus Epilepsies (PMEs) are a group of rare genetic disorders characterised by myoclonus, epileptic seizures and progressive neurological deterioration, particularly dementia and ataxia. A large number of specific diseases can cause the PME syndrome.

 Reviewing literatures, in the 1950's PME syndrome was categorized into 3 major groupes, Lafora disease, lipidosis type, and degenerative type, on the basis of neuropathological findings. However, the nosological concept of the degenerative type of PME has not been clear because of the lack of distinct pathological hallmarks such as Lafora bodies or lipid inclusions, and the cases reported as such have shown only neuronal abiotrophic or nonspecific changes involving diverse portions of the central nervous system.


Copyright © 1990, Igaku-Shoin Ltd. All rights reserved.

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電子版ISSN 1882-1243 印刷版ISSN 0001-8724 医学書院

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