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A clinicopathological study on autosomal dominant hereditary olivo-ponto-cerebellar atrophy of which gene locus does not exist on chromosome 6p (Menzel's disease). Kiyoshi IWABUCHI 1 , Saburou YAGISHITA 2 , Masakatsu ANDOH 3 , Masuyuki NAMBA 4 , Toshio MIZUTANI 5 , Yoshio MORIMATSU 6 , Hideki NAGATOMO 7 , Tatsuji TANABE 8 , Naoji AMANO 9 , Kenji KOSAKA 10 1Department of Neuropathology, Tokyo Institute of Psychiatry 2Department of Pathology, Kanagawa Rehabilitation Center 3Department of Pathology, Asahikawa Red Cross Hospital 4Jikei Institute of Psychiatry 5Department of Neuropathology, Tokyo Metropolitan Institute of Gerontology 6Department of Clinicopathology, Tokyo Metropolitan Institute for Neuroscience 7Department of Neurology, Yokohama City University, School of Medicine 8Takanedai Hospital 9Department of Psychiatry, Kanagawa Rehabilitation Center 10Department of Psychiatry, Yokohama City University, School of Medicine pp.307-323
Published Date 1993/4/10
DOI https://doi.org/10.11477/mf.1431900331
  • Abstract
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 Recent advances in neurology have revealed three different diseases in dominant hereditary olivo-ponto-cerebellar atrophy (OPCA). They are spinocerebellar ataxia 1 (SCA 1) in which the gene locus is located in the short arm of chromosome 6, Menzel's disease in which degeneration in the olivo-ponto-cerebellar (OPC) system is identical with that of Menzel's original case in 1891, and hereditary OPCA with retinal degeneration.

 We examined nine autopsied Japanese cases of Menzel's disease from seven families. Their age at onset ranged from 17 to 37 years old.


Copyright © 1993, 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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