The Italian Journal of Neurological Sciences

, Volume 15, Issue 7, pp 353–358

The POEMS syndrome: Report of six cases

Authors

  • Pareyson D. 
    • Istituto Nazionale Neurologico “C. Besta”IRCCS
  • Marazzi R. 
    • Istituto Nazionale Neurologico “C. Besta”IRCCS
  • Confalonieri P. 
    • Istituto Nazionale Neurologico “C. Besta”IRCCS
  • Mancardi G. L. 
    • Clinica NeurologicaUniversità di Genova
  • Schenone A. 
    • Clinica NeurologicaUniversità di Genova
  • Sghirlanzoni A. 
    • Istituto Nazionale Neurologico “C. Besta”IRCCS
Original Articles

DOI: 10.1007/BF02339932

Cite this article as:
Pareyson, D., Marazzi, R., Confalonieri, P. et al. Ital J Neuro Sci (1994) 15: 353. doi:10.1007/BF02339932

Abstract

We report six patients affected by POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes), a peculiar multiorgan disease frequently associated with osteosclerotic myeloma or other plasma cell disorders.

Sensorimotor polyneuropathy was associated with multisystem involvement in all of the patients, with osteosclerotic myeloma in 2 cases, monoclonal gammopathy of undetermined significance in 2 cases and Castleman's disease in the final two. In all of the patients, sural nerve biopsy findings were consistent with a mixed, axonal and demyelinating neuropathy. Increased levels of Interleukin-6 were found in two cases, but the pathogenesis of the disease is far from established.

Key Words

polyneuropathyPOEMS syndromemonoclonal gammopathyosteosclerotic myelomaCastleman's diseasecytokines

Sommario

Descriviamo 6 pazienti con sindrome POEMS (Polineuropatia, Organomegalia, Endocrinopatia, gammopatia Monoclonale, alterazioni cutanee [Skin changes]), una peculiare malattia ad interessamento multisistemico frequentemente associata a mieloma osteosclerotico o ad altre malattie plasmacellulari. Una polineuropatia sensitivo-motoria si accompagnava ad interessamento pluriviscerale in tutti i pazienti, a mieloma osteosclerotico in due casi, a gammopatia monoclonale di incerto significato in altri due, e a malattia di Castleman negli ultimi due pazienti. In tutti i 6 casi la biopsia del nervo surale dimostrava una neuropatia mista, demielinizzante ed assonale. In due casi sono stati trovati elevati livelli di Interleuchina-6, ma la patogenesi della malattia è ancora ignota.

Copyright information

© Masson S.p.A. 1994