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PubMed · 6264714

[Adrenoleukodystrophy (author's transl)].

J Lehmann·B Künnert·E Keller
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Abstract

A report on clinical course and morphological findings of a case of adrenoleukodystrophy in a 10-years-old boy is given. The brain showed the typical changes of a sudanophilic, inflammatory leukodystrophy in all lobes. Pathognomic intracytoplasmatic inclusions in the perivascular macrophages were found by electron microscopic examination. The adrenal glands showed a severe atrophy, but it was impossible to detect typical cytolpasmatic inclusions within the adrenocortical cells. By thin-layer chromatography an accumulation of cholesterol esters with very long chain fatty acids was found in the demyelinated areas. Findings diagnostic facilities and theories of pathogenesis are discussed.

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Addison DiseaseAutopsyChildCholesterolDiffuse Cerebral Sclerosis of SchilderHumansInclusion BodiesMacrophagesMaleMicroscopy, ElectronCase ReportsEnglish AbstractJournal Article

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J Lehmann, B Künnert, E Keller. 1981. [Adrenoleukodystrophy (author's transl)].. https://pubmed.ncbi.nlm.nih.gov/6264714/

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