TY - JOUR
T1 - Adrenomyeloneuropathy
T2 - A probable variant of adrenoleukodystrophy: II. General pathologic, neuropathologic, and biochemical aspects
AU - Schaumburg, Herbert H.
AU - Powers, James M.
AU - Raine, Cedrlcs
AU - Spencer, Peters
AU - Griffin, John W.
AU - Prineas, John W.
AU - Boehme, Diethelm M.
PY - 1977/12
Y1 - 1977/12
N2 - 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.
AB - 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.
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U2 - 10.1212/wnl.27.12.1114
DO - 10.1212/wnl.27.12.1114
M3 - Article
C2 - 200862
AN - SCOPUS:0017593418
SN - 0028-3878
VL - 27
SP - 1114
EP - 1119
JO - Neurology
JF - Neurology
IS - 12
ER -