The natural history of a "benign" rib lesion in a patient with a demyelinating polyneuropathy and an unusual variant of POEMS syndrome.
Patients with POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome exhibit great clinical variability and often have osteosclerotic myeloma. We present a patient with an acquired demyelinating polyneuropathy who was eventually diagnosed as having POEMS syndrome. Her long period of observation permitted documentation of the natural history of a plasmacytoma, including its remarkably slow rate of growth and its transformation from a nonsclerotic to a sclerotic bone lesion. Her clinical and laboratory features emphasize the variability of this syndrome. Biopsy of benign-appearing bone lesions should be considered in patients with acquired demyelinating polyneuropathies who do not respond to standard treatment modalities. Serial x-rays may not distinguish benign lesions from plasmacytomas in this treatable condition.
Simmons
Z
Z
Division of Neurology, Pennsylvania State University College of Medicine, Milton S. Hershey Medical Center, Hershey 17033.
Wald
J
J
Albers
J W
JW
Feldman
E L
EL
D001847:Bone Diseases / Q000150:complications / Q000000981:diagnostic imaging / Q000503:physiopathology*; D003711:Demyelinating Diseases / Q000150:complications / Q000503:physiopathology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008475:Median Nerve / Q000503:physiopathology; D008875:Middle Aged; D009046:Motor Neurons / Q000502:physiology; D009431:Neural Conduction; D009475:Neurons, Afferent / Q000502:physiology; D016878:POEMS Syndrome / Q000150:complications / Q000503:physiopathology*; D010543:Peroneal Nerve / Q000503:physiopathology; D011859:Radiography; D012272:Ribs / Q000000981:diagnostic imaging; D013979:Tibial Nerve / Q000503:physiopathology