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Journal of Child Neurology
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Amylaceous (Polyglucosan) Bodies in Familial Cerebral Atrophy of Early Onset

Guillermo A. de León, MD

Department of Pathology

Susan E. Crawford, DO

Department of Pathology

Cynthia Stack, MD

Department of Neurology

Crystal F. Darling, MD

Department of Radiology, Children's Memorial Hospital and Northwestern University School of Medicine, Chicago, IL

G. Stephan Johnson, MD, PhD

Department of Pathology

An 8-year-old girl and her 4-year-old sister presented with psychomotor retardation during the 1st year of life. This was fol lowed by spasticity, seizures, and in the older patient, progressive loss of faculties and death. Computed tomographic and magnetic resonance imaging scans demonstrated progressive cerebral atrophy in both patients. Postmortem examination in the older sibling showed diffuse atrophy of the supragranular layers of the cerebral cortex, atrophy and gliosis of the white matter, and accumulation of numerous amylaceous (polyglucosan) bodies within the cytoplasm of cell processes. The inclusions were closely associated with atrophy of the parenchyma. The findings suggest that this disorder is different from other childhood diseases in which amylaceous bodies accumulate within the brain. (J Child Neurol 1996;11:58-62).

Journal of Child Neurology, Vol. 11, No. 1, 58-62 (1996)
DOI: 10.1177/088307389601100115
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