Work

Neurological Peculiarities of POEMS Syndrome: Experience From a Brazilian University Center

Year: 2025

Type: article

Abstract: AIM: Polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes (POEMS) syndrome is a rare paraneoplastic syndrome associated with significant neurologic morbidity. Better understanding of the manifestations of this disease is crucial to early diagnosis and improvement of prognosis. METHODS: We retrospectively reviewed medical record data of adult patients diagnosed with POEMS syndrome between 2007 and December 2023 fulfilling the 2014 International Myeloma Working Group criteria for POEMS syndrome in a single tertiary care Hospital in Brazil. Clinical, laboratory and electrophysiological data were analyzed. RESULTS: Thirty-three patients were included, with median time from symptom onset to diagnosis of 13 (11-35) months, and 30% of patients were nonambulatory at the diagnosis. Neuropathy was present in 100% of patients, and early proximal muscle weakness was present in 57%. Nerve conductions studies (NCS) disclosed demyelinating polyneuropathy with axonal damage in 96% and conduction block in 33%. Serum monoclonal paraprotein heavy chain was IgG subtype in 73% and lambda light chain in 91%. Castleman disease was diagnosed in 6% of patients. Organomegaly, bone lesions and endocrinological profiles were similar to previously reported cohorts. DISCUSSION: Patients in our cohort had similar clinical profiles to previously reported data. However, unlike other large cohorts, our study showed a high proportion of IgG class paraprotein, non-length-dependent findings and conduction block on electrophysiology, and a lower number of cases associated with Castleman's disease. Our findings highlight the importance of pursuing a POEMS syndrome diagnosis even in the presence of atypical electrophysiological features.

Source: Muscle & Nerve

DOI: 10.1002/mus.70114

Authors: Renan Fabri Rosenstein, Jose Pedro Soares Baima, Thales Dalessandro Meneguin Pereira, Gracia Aparecida Martinez, Carlos Otto Heise, Angelina Maria Martins Lino

Institutions: Universidade de São Paulo, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, Universidade Federal do Ceará

Language: English

Topic: Peripheral Neuropathies and Disorders

Subfield: Neurology

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: hybrid

Estimated APC paid: $4,610