POEMS Syndrome Accompanied by Papilledema Identified in a Patient with Bilateral Visual Loss

Kim, Mi Jin; Kim, Seong-Joon

Case Report

Annals of Optometry and Contact Lens 2017;16(1):31-35.
Published online March 25, 2017.

POEMS Syndrome Accompanied by Papilledema Identified in a Patient with Bilateral Visual Loss

Department of Ophthalmology, Seoul National University College of Medicine¹, Seoul, Korea.
Seoul Artificial Eye Center, Seoul National University Hospital Clinical Research Institute², Seoul, Korea

Received: 20 September 2016 • Revised: 6 November 2016 • Accepted: 7 November 2016

Abstract

Purpose: POEMS refers to a syndrome characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. This report describes a patient who had been incidentally diagnosed with papilledema for bilateral reduced visual acuity and showed lower limb weakness that eventually led to a diagnosis of POEMS syndrome.
Case summary: A 63-year-old woman presented with 5-year history of bilateral reduced visual acuity. On initial examination, her best-corrected visual acuity was 1.0 in the right eye and 0.5 in the left eye with an intraocular pressure of 11 mmHg for both eyes. She had no afferent pupillary defect and had normal color vision. Only mild overall reduction of peripheral visual field was detected via the Goldman visual field test. Fundal examination revealed bilateral optic disc swelling and lumbar puncture verified increased intracranial pressure. Brain magnetic resonance imaging showed no abnormal findings other than pachymeningeal enhancement. An electromyogram performed to examine lower extremity weakness indicated sensorimotor polyneuropathy. Examination of bone marrow showed increased lambda monoclonal plasma cells and lymphoid cells. The patient also had splenomegaly, increased skin pigmentation, and pitting edema. Based on these findings, she was diagnosed with POEMS syndrome. She was treated with acetazolamide for bilateral papilledema caused by increased intracranial pressure of unknown etiology. She also underwent chemotherapy and steroids and is currently under follow-up.
Conclusion: Although rare, the possibility of POEMS syndrome should be taken into consideration in differential diagnosis of patients with optic disc swelling due to increased intracranial pressure of unknown etiology when accompanied by characteristic systemic manifestations.

Key Words: Optic disc swelling; Papilledema; POEMS syndrome