AMYLACEOUS (POLYGLUCOSAN) BODIES IN FAMILIAL CEREBRAL ATROPHY OF EARLY-ONSET

An 8-year-old girl and her 4-year-old sister presented with psychomoto r retardation during the Ist year of life. This was followed by spasti city, seizures, and in the older patient, progressive loss of facultie s and death. Computed tomographic and magnetic resonance imaging scans demonstrated progressive cerebral atrophy in both patients, Postmorte m examination in the older sibling showed diffuse atrophy of the supra granular layers of the cerebral cortex, atrophy and gliosis of the whi te matter, and accumulation of numerous amylaceous (polyglucosan) bodi es within tile cytoplasm of cell processes. The inclusions were closel y associated with atrophy of the parenchyma. The findings suggest that this disorder is different from other childhood diseases in which amy laceous bodies accumulate within the brain.