CASE REPORT


An Atypical Case of POEMS Syndrome Associated with Autonomic Dysfunction



Mariana B. Hämmerle1, Karina L. Pires1, *, 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
1 Gaffree Guinle University, Hospital Internal Medicine Rio de Janeiro, 5521996752660 Rua Mariz e Barros 775, Brazil


© 2019 Beiral Hämmerle et al.

open-access license: This is an open access article distributed under the terms of the Creative Commons Attribution 4.0 International Public License (CC-BY 4.0), a copy of which is available at: https://creativecommons.org/licenses/by/4.0/legalcode. This license permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

* Address correspondence to this author at the Gaffree Guinle University, Hospital Internal Medicine, Rio de Janeiro 5521996752660, Rua Mariz e Barros 775, Brazil; Email: karinalebeis1@gmail.com


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.

Keywords: POEMS, Cow-Fukase, Takatsuki, Polyneuropathy, Dysautonomia, Castleman disease.