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February 1984

Spongy Degeneration of the CNS in Infancy

Author Affiliations

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

Arch Neurol. 1984;41(2):164-170. doi:10.1001/archneur.1984.04050140062025

• 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.

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