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Japanese

A clinicopathological study on autosomal dominant hereditary dentatorubro-pallidoluysian atrophy (Naitoh-Oyanagi's disease). Kiyoshi IWABUCHI 1 , Saburo YAGISHITA 2 , Naoji AMANO 3 , Harutada SAKAI 3 , Megumi SASAKI 1,4 , Tatsuji TANABE 5 , Kenji KOSAKA 6 1Department of Neuropathology, Tokyo Institute of Psychiatry 2Department of Pathology, Kanagawa Rehabilitation Center 3Department of Psychiatry, Kanagawa Rehabilitation Center 4Department of Psychiatry, Tsukuba University, School of Medicine 5Takanedai Hospital 6Department of Psychiatry, Yokohama City University, School of Medicine pp.678-692
Published Date 1993/8/10
DOI https://doi.org/10.11477/mf.1431900359
  • Abstract
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 We studied the autosomal dominant hereditary dentatorubro-pallidoluysian atrophy (AD-DRPLA) clinicopathologically with special reference to its relation with the age at onset.

 Materials and methods: Seventeen autopsied cases from 13 families, including three couples and their children, were examined. The age at onset ranged from four to fifty-seven years of age. We divided them into three groups according to the age at onset; 5 cases in the juvenile form (the first and second decades), 7 cases in the early adult form (the third and forth decades), and 5 cases in the late adult form (above the fifth decade).


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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