POEMS syndrome with opticneuritis as initial symptom: a case report

Abstract

Polyneuropathy, Organomegaly, Endocrinopathy, M-protein and Skin changes syndrome is a rare paraneoplastic syndrome from a plasma cell dyscrasia, which most commonly presents with peripheral neuropathy.

We report a 30-year-old female with blurred vision and vision loss as reasons for her initial treatment; the patient was diagnosed with bilateral opticneuritis. Five months later, the patient started to have lower extremity numbness and fatigue. Initial laboratory tests did not reveal positive monoclonal immunoglobulin. Therefore, simple peripheral neuropathy was considered for diagnosis, and the patient was treated in the Department of Neurology in our hospital; however, the symptom was not alleviated. Seven months later, the patient showed symptoms of cough, dyspnea, along with rough skin, pigmentation and drooping of both feet. Chest CT revealed bronchiectasis and infection of lungs, destruction of thoracic bones and ribs. Further examination indicated that the patient had splenomegaly, hypothyroidism and monoclonal IgA-λ chain zone, which led to a clear diagnosis of Polyneuropathy, Organomegaly, Endocrinopathy, M-protein and Skin changes syndrome.