Adrenomyeloneuropathy: demonstration of inclusions at the level of the peripheral nerve.
A patients is reported with a progressive neurological disease which started at the age of 19 years and included spasmodic paraplegia, urinary disorders, impotence and neuropathic signs. The neurological syndrome was associated with adrenal failure. The disease led to death within 5 years. In this characteristic picture of adrenomyeloneuropathy, biopsy of peripheral nerve disclosed lamellar inclusions in the Schwann cell cytoplasm. Such structures, which have already been observed in the sural nerve of some adrenoleukodystrophy cases reported previously, are described for the first time in a case of adrenomyeloneuropathy.
D000309:Adrenal Insufficiency / Q000150:complications / Q000473:pathology*; D000328:Adult; D001706:Biopsy; D003593:Cytoplasm / Q000648:ultrastructure; D006801:Humans; D002479:Inclusion Bodies / Q000648:ultrastructure*; D008264:Macrophages / Q000648:ultrastructure; D008297:Male; D008854:Microscopy, Electron; D010264:Paraplegia / Q000150:complications / Q000473:pathology*; D010543:Peroneal Nerve / Q000473:pathology* / Q000648:ultrastructure; D012583:Schwann Cells / Q000648:ultrastructure