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One Case of Leucodystrophy with Deposits Resembling Rosenthal Fibres Susumu Ando 1 , Minoru Ueda 2 , Yoshisuke Nakagawa 2 , Naohiko Kato 2 , Yutaka Tojima 3 1National Musashi Sanatrium 2Department of Psychiatry Faculty of Medicine, University of Tokyo 3Neuro-Psychiatric Institute pp.765-774
Published Date 1967/12/25
DOI https://doi.org/10.11477/mf.1431904467
  • Abstract
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1) The clinical and pathological findings are described in a case of Alexander's disease (leucodystrophy with deposits resembling Rosenthal fibres), late infantile type, which began at 9 years and died at 21. Main clinical symptomes were speach disturbances, ataxia, bulbar palsy, pyramidal signs and mental deterioration.

2) Pathologiaclly the findings coinside with Alexander's disease. In additon to the changes in cerebrum, coalescent or patchy, dysmyelinated foci were disseminated in the various portions of the brain stem: these were lesions in the internal capsule, thalamus, cerebral peduncle, a part of red nucleus, pyramis, and around the olivary and dentate nuclei.

3) Clinical and neuropathological features were discussed with the reference to the cases hitherto reported.

4) Rosenthal fibres were studied with light and electron-microscops. Electron-microscopic examination disclosed two new findings in neurofibril and perivascural areas. Close relation of Rosenthal fiber to astrocytes was suspected through its figure and location, but confirmable evidence of its relation to thier cell bodies was not shown.

5) It was suspected that the luncodystrophy in this case was dysmelinogenic one.


Copyright © 1967, 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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