[Adrenomyeloneuropathies].
An adrenomyeloneuropathy is observed in a patient died at 24 years old after an illness of five years duration. These case is peculiar by the presence of lamellar cytoplasmic inclusions in the Schwann cells. Adrenomyeloneuropathy is an adult variant of adrenoleucodystrophy. This sex linked recessive disorder is related to an excessive amount of long chain fatty acids.
D000309:Adrenal Insufficiency / Q000175:diagnosis*; D000328:Adult; D003711:Demyelinating Diseases / Q000175:diagnosis* / Q000378:metabolism; D005227:Fatty Acids / Q000032:analysis; D006801:Humans; D002479:Inclusion Bodies / Q000473:pathology; D008297:Male; D009186:Myelin Sheath / Q000032:analysis; D009422:Nervous System Diseases / Q000175:diagnosis / Q000473:pathology; D010264:Paraplegia / Q000175:diagnosis*; D010525:Peripheral Nerves / Q000648:ultrastructure; D013577:Syndrome
D005227:Fatty Acids
Les adrénomyéloneuropathies.