Leukoencephalopathic alteration in membranous lipodystrophy

  • J. Tanaka
  • Published 1980 in Acta Neuropathologica

Abstract

Leukoencephalopathic alteration in a case of membranous lipodystrophy which involves the systemic adipose tissue has been described. A 44-year-old Japanese man whose parents were consanguinous developed multiple cystic lesions in the limb bones with a pathological fracture, followed by progressive neuropsychiatric symptoms, and died 16 years after the onset of his illness. Brain showed diffuse and symmetrical demyelination of the cerebral white matter, particularly in the fronto-temporal lobes, with preservation of subcortical arcuate fibers, associated with the occurrence of sudanophilic granules within perivascular macrophages and conspicuous fibrous gliosis. The alteration suggests it to be a sudanophilic leukodystrophy. Deposition of calcospherites in the basal ganglia was another interesting remark. This disorder is thought to be associated with an impairment in systemic lipid metabolism affecting both cerebral myelin and fat cell membranes on a heredofamilial backgound.

DOI: 10.1007/BF00688753

8 Figures and Tables

Statistics

05001000'87'90'93'96'99'02'05'08'11'14'17
Citations per Year

1,925 Citations

Semantic Scholar estimates that this publication has 1,925 citations based on the available data.

See our FAQ for additional information.

Cite this paper

@article{Tanaka1980LeukoencephalopathicAI, title={Leukoencephalopathic alteration in membranous lipodystrophy}, author={J. Tanaka}, journal={Acta Neuropathologica}, year={1980}, volume={50}, pages={193-197} }