An Atypical Case of POEMS Syndrome Associated with Autonomic Dysfunction



Mariana Beiral Hämmerle1, Karina Lebeis Pires1, *, Márcia Beiral Hammerle1, Pedro de Mello Vianna Pedrosa Galvão1, Lavinia Lustosa Bergier1, Vitor Valviesse1, Monique de França Silva1, Marcelo Ribeiro Caetano1, Felix Perez1, Kevin Angel Reyes1, Camila Bastos Almenara1, Larissa Cristine de Souza Lopes1, Daiane Vieira Botelho1
1 Gaffree Guinle University Hospital Internal Medicine Rio de Janeiro 5521996752660 Rua Mariz e Barros 775, Brazil

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, gastrointestinal 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.


Abstract Information


Identifiers and Pagination:

Year: 2019
Volume: 13
Publisher Item Identifier: EA-TONEUJ-2019-6

Article History:

Received Date: 25/05/2019
Revision Received Date: 17/06/2019
Acceptance Date: 11/07/2019
Electronic publication date: 23/08/2019
Collection year: 2019

© 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