title: Ventriculoperitoneal shunt placement for POEMS syndrome.
abstract: 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.
authors.affiliation: Department of Neurosurgery, Massachusetts General Hospital, Harvard Medical School, 15 Parkman Street Wang 021, Boston, MA 02114, USA. Electronic address: calvarez-breckenridge@partners.org.
authors.forename: Christopher A
authors.initials: CA
authors.lastname: Alvarez-Breckenridge
authors.affiliation: Department of Neurosurgery, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
authors.forename: Mark A
authors.initials: MA
authors.lastname: Attiah
authors.affiliation: Department of Neurosurgery, Massachusetts General Hospital, Harvard Medical School, 15 Parkman Street Wang 021, Boston, MA 02114, USA.
authors.forename: Marcus
authors.initials: M
authors.lastname: Zachariah
authors.affiliation: Department of Neurosurgery, Yale School of Medicine, New Haven, CT, USA.
authors.forename: Abhijeet
authors.initials: A
authors.lastname: Gummadavelli
authors.affiliation: Department of Neurosurgery, Massachusetts General Hospital, Harvard Medical School, 15 Parkman Street Wang 021, Boston, MA 02114, USA.
authors.forename: Jimmy
authors.initials: J
authors.lastname: Yang
authors.affiliation: Department of Neurosurgery, Massachusetts General Hospital, Harvard Medical School, 15 Parkman Street Wang 021, Boston, MA 02114, USA.
authors.forename: Patrick J
authors.initials: PJ
authors.lastname: Codd
mesh_terms: D000328:Adult; D005260:Female; D006261:Headache / Q000150:complications / Q000175:diagnosis / Q000601:surgery; D006801:Humans; D019586:Intracranial Hypertension / Q000150:complications / Q000175:diagnosis / Q000601:surgery; D016878:POEMS Syndrome / Q000150:complications / Q000175:diagnosis* / Q000601:surgery*; D017287:Ventriculoperitoneal Shunt / Q000379:methods*
keywords: Cerebrospinal fluid diversion; Intracranial pressure; POEMS syndrome; Papilledema; Vascular endothelial growth factor