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An Atypical Case of POEMS Syndrome Associated with Autonomic Dysfunction
Márcia B. Hammerle1
Pedro de Mello Vianna P. Galvão1
Lavinia L. Bergier1
Vitor V.1
Monique de França Silva1
Marcelo R. Caetano1
Felix Perez1
Kevin A. Reyes1
Camila B. Almenara1
Larissa Cristine de Souza Lopes1
Daiane V. Botelho1
Authors Info & Affiliations
Abstract
A 52 year old patient showed, for two years, symptoms compatible with sensorimotor polyneuropathy preventing him from walking. An electroneuromyography found a demyelinating pattern suggesting Chronic Inflammatory Demyelinating Polyneuropathy (CIDP). The patient did not respond to corticosteroid therapy and later suffered sexual Dysfunction, Swelling, Lymphadenopathy, Hypotension, astrointestinal dysmotility, urinary retention and neuropathic pain. Analysis of the Cerebrospinal Fluid (CSF) revealed elevated protein levels and Computed Tomography (CT) scan found sclerotic bone lesions. High Vascular Endothelial Growth Factor (VEGF) levels and the results of Lambda light-Chain monoclonal gammopathy in urine protein Electrophoresis Suggested a Diagnosis of POEMS syndrome. The most striking feature, in this case, was the patient’s heightened and atypical polyneuropathy without axonal injury even after an extended period of time, and significant and atypical dysautonomia.
