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Canavan Disease (Spongy Cerebral Degeneration) - A Case Report -

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Authors
Oh, Mee Hye; Kim, Chong Jai; Chi, Je G.; Kim, In-One; Hwang, Yong Seung; Wang, Kyu Chang; Cho, Byung Kyu
Issue Date
1995-06
Publisher
Seoul National University College of Medicine
Citation
Seoul J Med 1995;36(2):85-91
Keywords
Canavan diseaseSpongy cerebral degenerationN-acetylaspartic acidAspartoacylase
Abstract
We report a case of Canavan disease in a 6 month-old male Korean
baby who presented with aggravating tonic-clonic seizure for 5 days. Pathologic'
findings could be summarized as follows; increase in brain volume and weight,
spongy degeneration in the deep layers of the cerebral cortex and subcortical
white matter, and hyperplasia of Alzheimer type II astrocytes throughout the cerebral
cortex. Ultrastructurally, there was an abnormal accumulation of fluid in
astrocytes and between splitting myelin lamellae.
ISSN
0582-6802
Language
English
URI
http://hdl.handle.net/10371/6810
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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. 36 No.2 (1995)
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