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