Spongy degeneration of the CNS in infancy
Y. Luo and K. Huang
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.
