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Two Autopsy Cases of Sporadic Amyotrophic Lateral Sclerosis with 20-year-clinical Course without Respirators Shuichi Kato 1 , Masaya Oda 2 , Hideaki Hayashi 1 , Tetsuo Komori 1 , Kazuhiko Hirose 1 , Hitoshi Tanabe 3 , Nobutaka Arai 1Departments of Neurology Tokyo Metropolitan Neurological Hospital 2Departments of Pathology, Tokyo Metropolitan Neurological Hospital 3Department of Clinical Neuropathology, Tokyo Metropolitan Institute for Neuroscience Keyword: amyotrophic lateral sclerosis , pyramidal tract , medullary reticular formation pp.267-272
Published Date 1993/3/1
DOI https://doi.org/10.11477/mf.1406900459
  • Abstract
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The present paper concerns the clinicopatho-logical study of two patients with sporadic amyotro-phic lateral sclerosis (ALS) with 21 or 22 years' clinical course without respirators. The two cases developed marked upper motor neuron signs and continually progressive paralysis. Involvement of the facial and bulbar muscles remained fairly mild. At autopsy, the anterior horns showed marked loss of motor neurons associated with numerous reactive astrocytes. On the other hand, the motor cortex and the pyramidal tract showed diminution of Betz cells and large myelinated fibers but no positive mate-rials by fat stain. In addition, the anterolateral funiculi of the spinal cords revealed widespread myelin pallor. One of the case showed intracytoplas-mic eosinophilic inclusions in some neurons of the medullary reticular formation.

Recent articles have reported several rapidly progressive ALS patients who developed ophthal-moplegia, while on respirators, and widespread degeneration in the CNS. By contrast, our extreme ly slowly progressive ALS patients developed alte-rations confined to upper and lower motor neurons. There may exist some diversities in ALS.


Copyright © 1993, Igaku-Shoin Ltd. All rights reserved.

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電子版ISSN 2185-405X 印刷版ISSN 0006-8969 医学書院

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