Neurol. praxi. 2023;24(3):232-235 | DOI: 10.36290/neu.2023.006

A rare syndrome where our multidisciplinary approach significantly helped the patient

MUDr. Alexandra Jungová, Ph.D.¹, Dr. Evgeni D. Kavrakov²

¹ Hematoonkologické oddělení, FN Plzeň

² Neurologické oddělení, Karlovarská krajská nemocnice, Karlovy Vary

In this case report we present the diagnostic process and treatment of a patient with severe chronic polyneuropathy (lasting around 3months), lymfadenopathy. hepatosplenomegaly, hypertrichosis and other symptoms. The patient´s condition required consultations with a neurologist, a hematologist, an ophtalmologist and also with a rehabilitation specialist and infectologists. The diagnose classic chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) was considered in the beginning due to relevant clinical manifestations. Intravenous immunoglobulins did not lead to significant clinical improvement of the polyneuropathy. Afterwards, it was found that our patient suffers from POEMS syndrome - a rare diagnosis where the hematological examination is crucial. This acronym means Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, Skin changes. Our patient did exhibit all five of those symptoms. In the end, the neurological manifestations improved significantly after specialized treatment (including autologous transplantation of stem cells). The patient´s quality of life improved, i.e. disability disappeared almost completely thanks to multidisciplinary care. We discuss different aspects of the diagnostic process in POEMS syndrome which included hospitalization in our neurology clinic. The treatment plan is also presented here. POEMS syndrome is a rare and complex disease where the neurologist plays an important role in the differential diagnostic clarification.

Keywords: POEMS, CIDP, autologous stem cell trasplantation, multidisciplinary care.

Received: January 4, 2023; Revised: January 30, 2023; Accepted: February 4, 2023; Prepublished online: February 4, 2023; Published: June 14, 2023  Show citation