Adrenoleukodystrophy. A clinical and pathological study of 17 cases

H. H. Schaumburg, J. M. Powers, C. S. Raine, K. Suzuki, E. P. Richardson

Research output: Contribution to journalArticle

392 Citations (Scopus)

Abstract

Adrenoleukodystrophy was diagnosed pathologically in 17 male patients. The diagnosis was suggested by clinical and laboratory signs of primary adrenal failure and by neurological signs referable to the degeneration of white matter. Neurological findings usually predominated over clinical stigmata of adrenal failure. Adrenal biopsy has proved to be the most reliable diagnostic test, while brain biopsy has often been misleading. The histological picture of the brain lesion differs substantially from that of the adrenal, but the presence of similar ultrastructural cytoplasmic inclusions suggests a common metabolic disorder. Morphological analysis of the cerebral lesion indicates that the destruction may spread in a caudal rostral direction. The intense inflammatory cell response occurs within the demyelinated areas, behind the area of active myelin breakdown, and appears to be a secondary feature of white matter degeneration.

Original languageEnglish (US)
Pages (from-to)577-591
Number of pages15
JournalArchives of Neurology
Volume32
Issue number9
StatePublished - 1975

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Adrenoleukodystrophy
Biopsy
Christianity
Inclusion Bodies
Brain
Myelin Sheath
Routine Diagnostic Tests
White Matter
Clinical Studies
Degeneration
Direction compound
Stigma
Morphological Analysis
Cells
Diagnostics
Lesion
Brain Lesion
Destruction
Inclusion

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Schaumburg, H. H., Powers, J. M., Raine, C. S., Suzuki, K., & Richardson, E. P. (1975). Adrenoleukodystrophy. A clinical and pathological study of 17 cases. Archives of Neurology, 32(9), 577-591.

Adrenoleukodystrophy. A clinical and pathological study of 17 cases. / Schaumburg, H. H.; Powers, J. M.; Raine, C. S.; Suzuki, K.; Richardson, E. P.

In: Archives of Neurology, Vol. 32, No. 9, 1975, p. 577-591.

Research output: Contribution to journalArticle

Schaumburg, HH, Powers, JM, Raine, CS, Suzuki, K & Richardson, EP 1975, 'Adrenoleukodystrophy. A clinical and pathological study of 17 cases', Archives of Neurology, vol. 32, no. 9, pp. 577-591.
Schaumburg HH, Powers JM, Raine CS, Suzuki K, Richardson EP. Adrenoleukodystrophy. A clinical and pathological study of 17 cases. Archives of Neurology. 1975;32(9):577-591.
Schaumburg, H. H. ; Powers, J. M. ; Raine, C. S. ; Suzuki, K. ; Richardson, E. P. / Adrenoleukodystrophy. A clinical and pathological study of 17 cases. In: Archives of Neurology. 1975 ; Vol. 32, No. 9. pp. 577-591.
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