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

Publication ,  Journal Article
de León, GA; Crawford, SE; Stack, C; Darling, CF; Johnson, GS
Published in: J Child Neurol
January 1996

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.

Duke Scholars

Published In

J Child Neurol

DOI

ISSN

0883-0738

Publication Date

January 1996

Volume

11

Issue

1

Start / End Page

58 / 62

Location

United States

Related Subject Headings

  • Tomography, X-Ray Computed
  • Neurology & Neurosurgery
  • Muscle, Skeletal
  • Humans
  • Female
  • Child, Preschool
  • Child
  • Brain Diseases
  • Brain
  • Atrophy
 

Citation

APA
Chicago
ICMJE
MLA
NLM
de León, G. A., Crawford, S. E., Stack, C., Darling, C. F., & Johnson, G. S. (1996). Amylaceous (polyglucosan) bodies in familial cerebral atrophy of early onset. J Child Neurol, 11(1), 58–62. https://doi.org/10.1177/088307389601100115
León, G. A. de, S. E. Crawford, C. Stack, C. F. Darling, and G. S. Johnson. “Amylaceous (polyglucosan) bodies in familial cerebral atrophy of early onset.J Child Neurol 11, no. 1 (January 1996): 58–62. https://doi.org/10.1177/088307389601100115.
de León GA, Crawford SE, Stack C, Darling CF, Johnson GS. Amylaceous (polyglucosan) bodies in familial cerebral atrophy of early onset. J Child Neurol. 1996 Jan;11(1):58–62.
de León, G. A., et al. “Amylaceous (polyglucosan) bodies in familial cerebral atrophy of early onset.J Child Neurol, vol. 11, no. 1, Jan. 1996, pp. 58–62. Pubmed, doi:10.1177/088307389601100115.
de León GA, Crawford SE, Stack C, Darling CF, Johnson GS. Amylaceous (polyglucosan) bodies in familial cerebral atrophy of early onset. J Child Neurol. 1996 Jan;11(1):58–62.
Journal cover image

Published In

J Child Neurol

DOI

ISSN

0883-0738

Publication Date

January 1996

Volume

11

Issue

1

Start / End Page

58 / 62

Location

United States

Related Subject Headings

  • Tomography, X-Ray Computed
  • Neurology & Neurosurgery
  • Muscle, Skeletal
  • Humans
  • Female
  • Child, Preschool
  • Child
  • Brain Diseases
  • Brain
  • Atrophy