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Further Postmortem Examination of a Case of Familial Ataxia with Cerebrospinal Fluid Abnormality: An Electron Microscopic Study of the Intracytoplasmic Eosinophilic Inclusion Bodies in the Central Nervous System
Author(s) -
Nakamura Ichiro,
Kurachi Masayoshi,
Fukutani Yuken,
Katsukawa Kazuhiko,
Kobayashi Katsuji,
Kawasaki Yasuhiro,
Suzuki Michio,
Yamaguchi Nariyoshi,
Torii Hosaku
Publication year - 1989
Publication title -
psychiatry and clinical neurosciences
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.609
H-Index - 74
eISSN - 1440-1819
pISSN - 1323-1316
DOI - 10.1111/j.1440-1819.1989.tb02574.x
Subject(s) - vacuole , pathology , inclusion bodies , cerebrospinal fluid , ataxia , ependymal cell , endoplasmic reticulum , biology , eosinophilic , central nervous system , cytoplasm , anatomy , chemistry , microbiology and biotechnology , medicine , biochemistry , neuroscience , escherichia coli , gene
As reported previously, the peculiar intracytoplasmic eosinophilic inclusion bodies (IEIBs) extensively appeared in the autopsied brain tissue from a 49‐year‐old man having familial ataxia with cerebrospinal fluid abnormality, and histochemically showed abundant proteins, but few lipids and carbohydrates. Ultrastructurally, many membrane‐bound vacuoles derived from the distended cisterns of rough‐surfaced endoplasmic re‐ticulum (RER) appeared in the neurons. They were filled with fine granular, less dense materials. The IEIBs, shown as a homogeneous dense core, were found in some of the vacuoles. Similar vacuoles also appeared in astrocytes, oligodendrocytes, vascular peri‐cytes, ependymal and choroidal epithelial cells. It is suggested that the vacuoles result from the accumulation of metabolic products in the distended RER cisterns of the cells in the central nervous system, presumably representing a genetically determined functional abnormality of the RER in protein synthesis and/or transport.