[POEMS syndrome: a contribution to differential diagnosis of polyneuropathy].
The POEMS syndrome is a synopsis of different symptoms such as polyneuropathy, organomegaly, endocrine disturbances, M-protein and skin changes. The leading symptoms are neuropathy and the skin symptoms. Additionally, a monoclonal light chain gammopathy is often found. The administration of immunosuppressive drugs yields a substantial improvement in some cases. We report here about a 72 year old lady who fell ill with a rapidly progressive neuropathy accompanied by hyperpigmentation and a morphea-like induration of the skin. A biopsy of the sural nerve showed a demyelinating axonal neuropathy and a focal vasculitis. Isoelectric focussing revealed oligoclonal bands in cerebrospinal fluid and serum. The cortisol serum level was very low and there were signs of a latent diabetes mellitus. These clinical features correspond to the POEMS syndrome. The prescription of initially 1 mg and later 0.5 mg prednisone improved the patient's condition dramatically.
Dressnandt
J
J
Neurologische Klinik, Technischen Universität München.
Näher-Noé
M
M
Drosner
M
M
Klingelhöfer
J
J
D000368:Aged; D001706:Biopsy; D004574:Electromagnetic Fields; D004576:Electromyography / Q000187:drug effects; D005260:Female; D006801:Humans; D007073:Immunoglobulin E / Q000032:analysis; D009132:Muscles / Q000294:innervation; D009460:Neurologic Examination / Q000187:drug effects; D016878:POEMS Syndrome / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology; D010525:Peripheral Nerves / Q000187:drug effects / Q000473:pathology / Q000503:physiopathology; D011239:Prednisolone / Q000008:administration & dosage
D007073:Immunoglobulin E; D011239:Prednisolone
Das POEMS-Syndrom: Ein Beitrag zur Differentialdiagnose der Polyneuropathie.