Muscle & nerve · 2025
Neurological Peculiarities of POEMS Syndrome: Experience From a Brazilian University Center.
СтатьяНазвание и аннотация: Перевод в очереди
Обработка статьи · этапы, ошибки и управление
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Rosenstein Renan Fabri, Soares Baima Jose Pedro, Meneguin Pereira Thales Dalessandro, Martinez Gracia Aparecida, Heise Carlos Otto, Lino Angelina Maria Martins
Аннотация
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.
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.