[Recurrent cerebral ischemias due to cerebral vasculitis within the framework of incomplete POEMS syndrome with Castleman disease]

Abstract

The POEMS syndrome is a multisystem disorder characterised by polyneuropathy, organomegaly, endocrinopathy, plasma cell dyscrasia and skin changes. A coincidence with angiofollicular lymphoid hyperplasia (Castleman's disease) exists in about 60% of all cases. The POEMS syndrome may be associated with macroangiopathy and acute vascular obliterations. Most case reports refer to involvement of the coronary and lower limb arteries. There are only few reports dealing with cerebral strokes and POEMS syndrome. We report on a 32-year-old female with an incomplete form of POEMS syndrome with Castleman's disease and associated cerebral vasculitis. After sufficient treatment of the plasma cell dyscrasia, recurrent cerebral ischemias occurred. A stable state was finally reached after primary treatment of the vasculitis with cyclophosphamide.