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

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

Research output: Contribution to journalArticle

123 Citations (Scopus)

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
StatePublished - 1977
Externally publishedYes

Fingerprint

Adrenoleukodystrophy
Cholesterol Esters
Inclusion Bodies
Adrenal Glands
Nervous System
Testis
Autopsy
Fatty Acids
Cholesterol
Inclusion
Neuropathology
Dying
Brain

ASJC Scopus subject areas

  • Arts and Humanities (miscellaneous)
  • Clinical Neurology
  • Neuroscience(all)

Cite this

Schaumburg, H. H., Powers, J. M., Raine, C., Spencer, P., Griffin, J. W., Prineas, J. W., & Boehme, D. M. (1977). Adrenomyeloneuropathy: A probable variant of adrenoleukodystrophy: II. General pathologic, neuropathologic, and biochemical aspects. Neurology, 27(12), 1114-1119.

Adrenomyeloneuropathy : A probable variant of adrenoleukodystrophy: II. General pathologic, neuropathologic, and biochemical aspects. / Schaumburg, Herbert H.; Powers, James M.; Raine, Cedrlcs; Spencer, Peter; Griffin, John W.; Prineas, John W.; Boehme, Diethelm M.

In: Neurology, Vol. 27, No. 12, 1977, p. 1114-1119.

Research output: Contribution to journalArticle

Schaumburg, HH, Powers, JM, Raine, C, Spencer, P, Griffin, JW, Prineas, JW & Boehme, DM 1977, 'Adrenomyeloneuropathy: A probable variant of adrenoleukodystrophy: II. General pathologic, neuropathologic, and biochemical aspects', Neurology, vol. 27, no. 12, pp. 1114-1119.
Schaumburg, Herbert H. ; Powers, James M. ; Raine, Cedrlcs ; Spencer, Peter ; Griffin, John W. ; Prineas, John W. ; Boehme, Diethelm M. / Adrenomyeloneuropathy : A probable variant of adrenoleukodystrophy: II. General pathologic, neuropathologic, and biochemical aspects. In: Neurology. 1977 ; Vol. 27, No. 12. pp. 1114-1119.
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