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Alexander's Disease: A Case Report with Ultrastructural, Immunohistochemical and MRI Findings
Alexander병 - 1증례보고

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Authors
Kim, Chong-Jai; Chi, Je G.; Park, In-Sung; Cho, Byung-Kyu; Kim, In-One; Chang, Kee-Hyun
Issue Date
1989-03
Publisher
Seoul National University College of Medicine
Citation
Seoul J Med, Vol.30 No.1, pp. 47-57
Keywords
Alexander's diseaseRosenthal fiberWhite matter disease
Abstract
Alexander's disease is an unusual type of disorder which is characterized by a
progressive neurological deterioration and the diffuse deposition of Rosenthal fibers. In this
study, a case of 7 years old boy with Alexander's disease is evaluated in detail with full
emphasis on its clinical and morphological characteristics. Clinical and pathological findings
of the patient coincide with the classic features of Alexander's disease. From the findings, the
basic pathogenetic mechanism was postulated. Current knowledge of this disorder strongly
supports a possibility of a metabolic defect in the degradation of glial filament proteins of
astrocytes and therefore, it is our belief that the term Alexander's disease should be used
more strictly for the cases with an early onset and typical clinical manifestations.
ISSN
0582-6802
Language
English
URI
https://hdl.handle.net/10371/5534
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College of Medicine/School of Medicine (의과대학/대학원)Dept. of Medicine (의학과)The Seoul Journal of MedicineThe Seoul Journal of Medicine Vol. 30 No.1 (1989)
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