Amylaceous (polyglucosan) bodies in familial cerebral atrophy of early onset

Guillermo A. De León*, Susan E. Crawford, Cynthia Stack, Crystal F. Darling, G. Stephan Johnson

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

1 Scopus citations


An 8-year-old girl and her 4-year-old sister presented with psychomotor retardation during the 1st year of life. This was followed 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.

Original languageEnglish (US)
Pages (from-to)58-62
Number of pages5
JournalJournal of child neurology
Issue number1
StatePublished - Jan 1996
Externally publishedYes

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health


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