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Publication Details
AFRICAN RESEARCH NEXUS
SHINING A SPOTLIGHT ON AFRICAN RESEARCH
medicine
POEMS syndrome with plasmocytoma lytic bone lesion
Revue Neurologique, Volume 164, No. 8-9, Year 2008
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Description
Introduction: Crow-Fukase or Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, and Skin changes syndrome (POEMS) is a rare multisystemic affection with incompletely elucidated etiopathogenesis. Case report: We report a case of POEMS syndrome in a 48-year-old adult revealed four months before admission by areflexic flask tetraparesis prevalent on the lower limbs in connection with demyelinating and axonal CIDP "like" sensoriomotor neuropathy of the four limbs electroneuromyographically. The patient presented elevated protein level in the CSF with monoclonal standard IgG gammapathy associated with a narrow band lambda, suggesting POEMS syndrome. Further explorations revealed skin lesions with glomeruloid angiomas, edematous vasomotor disorders as well as erythrocyanose, hypogonadism, papillar edema and a lytic bone lesion of the left scapula. Radiotherapy was associated with corticosteroids and plasma exchanges. Outcome was good with resolution of the symptoms and stabilization of the neuropathy. Discussion: POEMS syndrome is rare; the diagnosis is based on necessary criteria, the presence of a demyelinating and axonal polyneuropathy associated with an IgA or IgG monoclonal gammapathy, the light chain being almost entirely lambda, associated to other characteristic elements, in particular glomeruloid angiomas, endocrinopathy, sclerosing plasmocytoma which must be carefully required. Treatment is based on surgical cure or radiotherapy for bone lesion and non specific treatments such as corticosteroid therapy, plasma exchanges and IVIG. © 2008 Elsevier Masson SAS. All rights reserved.
Authors & Co-Authors
Abdoh-Rafai, Mohammed Abdoh
Morocco, Casablanca
Centre Hospitalier Universitaire Ibn Rochd
Fadel, Hicham
Morocco, Tanger
Hôpital al Kortobi
Boulaajaj, Fatima Zohra
Morocco, Casablanca
Centre Hospitalier Universitaire Ibn Rochd
Sibai, Manal
Morocco, Casablanca
Centre Hospitalier Universitaire Ibn Rochd
Rafai, M.
Morocco, Casablanca
Centre Hospitalier Universitaire Ibn Rochd
El Moutawakkil, Bouchra
Morocco, Casablanca
Centre Hospitalier Universitaire Ibn Rochd
Bourezgui, Meryem
Morocco, Casablanca
Centre Hospitalier Universitaire Ibn Rochd
Trafeh, Mustapha
Morocco, Casablanca
Centre Hospitalier Universitaire Ibn Rochd
Slassi, Ilham
Morocco, Casablanca
Centre Hospitalier Universitaire Ibn Rochd
Statistics
Citations: 9
Authors: 9
Affiliations: 2
Identifiers
Doi:
10.1016/j.neurol.2007.11.001
ISSN:
00353787
Research Areas
Cancer
Health System And Policy