This Article  • References  • Full text PDF  • Reply to article  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citation map  • Citing articles on HighWire  • Citing articles on Web of Science (14)  • Contact me when this article is cited  Related Content  • Similar articles in this journal  Social Bookmarking   What's this?

Yi Luo, MD; Kewei Huang, MD

Arch Neurol. 1984;41(2):164-170.

Abstract
• We studied six cases of spongy degeneration of the CNS in infancy among Chinese. The main clinical features were cyclical vomiting and wailing, blindness, megalocephaly, convulsions, lethargy, and coma. Chief findings from light microscopic studies were spongiform change, moderate outfall of neurons, and marked astrocytosis, with formation of Alzheimer's cells type 2. Demyelination was only slight, if present. Electron microscopic studies revealed membrane-bound vacuoles, often containing membranous fragments or blisters and electron-dense granules.

Author Affiliations
From the Neuropathological Laboratory, Chinese PLA General Hospital, Beijing.

Footnotes
Accepted for publication March 3, 1983.

Reprint requests to Neuropathological Laboratory, Chinese PLA General Hospital, Beijing, China (Dr Luo).

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter     What's this?

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

Molecular Basis of Canavan's Disease: From Human to Mouse
Surendran et al.
J Child Neurol 2003;18:604-610.
ABSTRACT  

Spongy Degeneration of the Central Nervous System (Van Bogaert-Bertrand Disease): Report of a Case in a Saudi Family
Mahdi et al.
J Child Neurol 1986;1:61-63.
ABSTRACT