POEMS syndrome with central nervous system involvement: a case report

POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) syndrome was found to be associated with a disorder of the central nervous system (CNS) in a 56-year-old woman. Elevated intracranial pressure, neuroimaging evidence of diffuse white matter edema and papilledema point to a disease process affecting the CNS. Brainstem auditory (BAEPs) and visual evoked potentials (VEPs) also provided evidence of reduced central conduction velocities without, however, apparent clinical deficits attributable to CNS involvement. The intracranial hypertension is thought to be a part of a generalized tendency toward edema characteristic of this particular syndrome. We speculate that POEMS syndrome is an autoantibody-mediated immunological disorder in which cerebral vascular endothelium is one of the targets of the immunopathogenetic process and undergoes subsequent enhancement of vascular permeability.