Adrenomyeloneuropathy: A probable variant of adrenoleukodystrophy: II. General pathologic, neuropathologic, and biochemical aspects

Herbert H. Schaumburg, James M. Powers, Cedrlcs Raine, Peters Spencer, John W. Griffin, John W. Prineas, Diethelm M. Boehme

Research output: Contribution to journalArticle

124 Scopus citations

Abstract

Histopathologic features were studied in postmortem examination of two men with adrenomyeloneuropathy, and biochemical studies were performed on one of these individuals. The histopathologic picture of one case included dying-back features in the nervous system and lamellar cytoplasmic inclusions in the brain, adrenal gland, and testis similar to those in adrenoleukodystrophy. Biochemical studies of the cerebral white matter of this individual revealed increased amounts of long-chain saturated fatty acids in cholesterol esters, an abnormality characteristic of adrenoleukodystrophy. Despite differences in clinical presentation and neuropathology, adrenomyeloneuropathy probably represents a distinct variant of adrenoleukodystrophy.

Original languageEnglish (US)
Pages (from-to)1114-1119
Number of pages6
JournalNeurology
Volume27
Issue number12
DOIs
StatePublished - Dec 1977

ASJC Scopus subject areas

  • Clinical Neurology

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