[Adrenomyeloneuropathy: an adult form of adrenoleukodystrophy spastic paraparesis, and chronic adrenal insufficiency (concerning 3 cases) (author's transl)].
Three cases of adult males with spastic paraparesis and adrenal insufficiency are reported. The adrenal insufficiency is primary and in one case associated with Leydig cell insufficiency. Ultrastructural examination of peripheral nerve revealed abnormal cytoplasmic inclusions in Schwann cells. A decrease in the percentage of linoleate to total fatty acids was found in the sera. A child with adrenoleucodystrophy and an adult with adreno myeloneuropathy were observed in the same family. It allows adrenomyeloneuropathy to be considered as an adult form of adrenoleucodystrophy.
D000224:Addison Disease / Q000097:blood / Q000473:pathology; D000304:Adrenal Cortex Function Tests; D000305:Adrenal Cortex Hormones / Q000097:blood; D000328:Adult; D005227:Fatty Acids / Q000097:blood; D005231:Fatty Acids, Unsaturated / Q000097:blood; D006801:Humans; D008297:Male; D008875:Middle Aged; D009128:Muscle Spasticity / Q000097:blood / Q000473:pathology; D010264:Paraplegia / Q000097:blood / Q000473:pathology; D010525:Peripheral Nerves / Q000648:ultrastructure; D013577:Syndrome; D013739:Testosterone / Q000097:blood
D000305:Adrenal Cortex Hormones; D005227:Fatty Acids; D005231:Fatty Acids, Unsaturated; D013739:Testosterone
Adrénomyéloneuropathie: forme adulte d'adrénoleucodystrophie. Paraparésie spastique et insuffisance surrénale chronique. A propos de 3 cas.