POEMS syndrome: ultrastructural observations on the sural nerve.
POEMS is an acronym that stands for a multisystem disorder that includes progressive sensorimotor Polyneuropathy, Organomegaly, Endocrinopathy, an M component in blood and/or concentrated urine, and Skin changes. The morphology of the peripheral nerves was mentioned in some of these cases and ranged from normal to those with segmental demyelination and/or degeneration of myelinated axons. We analyzed quantitatively the sural nerves of two cases of POEMS syndrome. Both nerves showed a severe loss of myelinated fibers, with a minor degree of regeneration. Degeneration of unmyelinated axons was observed, but to a lesser degree. There was marked collagen deposition in the endoneurium. The pathogenesis of the changes in the peripheral nerves and other organs in POEMS syndrome is still to be elucidated.
Teixeira
F
F
Department of Experimental Neuropathology, National Institute of Neurology, Mexico City, Mexico D.F.
Aranda
F
F
Cabañas
A
A
Estañol
B
B
D000328:Adult; D001369:Axons / Q000648:ultrastructure; D003094:Collagen / Q000032:analysis; D004700:Endocrine System Diseases / Q000150:complications*; D006529:Hepatomegaly / Q000150:complications; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D008206:Lymphatic Diseases / Q000150:complications; D008297:Male; D009186:Myelin Sheath / Q000648:ultrastructure; D009410:Nerve Degeneration; D010523:Peripheral Nervous System Diseases / Q000150:complications* / Q000473:pathology; D012583:Schwann Cells / Q000648:ultrastructure; D012871:Skin Diseases / Q000150:complications*; D013127:Spinal Nerves / Q000648:ultrastructure*; D013497:Sural Nerve / Q000648:ultrastructure*; D013577:Syndrome
D007074:Immunoglobulin G; D003094:Collagen