Involvement of the central nervous system myelin in a POEMS patient

Abstract

POEMS (polyneuropathy, organomegaly, endocrinopathy, Mrotein and skin changes) syndrome is a rare cause of mixed emyelinating and axonal neuropathy with multi-organ involveent and a monoclonal plasma cell proliferative disorder [1]. ther important traits of the syndrome are sclerotic bone lesions, astleman disease, papilledema, peripheral edema, ascites, effuions, thrombocytosis, polycythemia, clubbing and increased level f the plasmacytomas-secreted vascular endothelial growth factor VEGF) [1]. The pathogenesis is not well understood but a role for VEGF has een suggested [2]. POEMS syndrome is a potentially fatal disease and patients’ uality of life deteriorates because of progressive neuropathy,masive pleural effusion, ascites or thromboembolic events. Cerebral vasculitis, multiple vascular abnormalities, hyperrophic cranial pachymeningitis, bilateral internal carotid rtery occlusion, amyloidosis, diffuse white matter edema and apilledema have been associated with POEMS syndrome [3–8]. n one case, cranial magnetic resonance imaging disclosed diffuse