Ventriculoperitoneal shunt placement for POEMS syndrome

We report a 41-year-old woman with a history of an uncomplicated spinal hemangioma resection, who developed acute onset sensory-motor polyneuropathy following influenza vaccine administration. With extensive workup she was diagnosed with POEMS syndrome with progressive headaches, visual loss with papilledema, and repeated elevated lumbar puncture opening pressures despite treatment with acetazolamide and immunosuppressive therapy. Her symptoms dramatically improved following ventriculoperitoneal shunt placement. POEMS syndrome is a paraneoplastic disorder involving a constellation of clinical symptoms including polyneuropathy, organomegaly, endocrinopathy, monoclonal protein elevation, and skin changes. The progression of the disease involves a number of neurovascular sequelae, including symmetric sensory-motor polyneuropathy resembling chronic inflammatory demyelinating polyneuropathy, cerebrovascular accidents, and papilledema associated with increased intracranial pressure. Despite the association of POEMS with papilledema, treatment for this finding typically includes acetazolamide and therapeutic large volume lumbar punctures. To our knowledge, this is the first report of cerebrospinal fluid shunting for the symptomatic management of hydrocephalus associated with POEMS syndrome.