TITLE:
Refractory Treatment of Chronic Inflammatory Demyelinating Polyneuropathy Revealing POEMS Syndrome
AUTHORS:
Loubna Chouaf, Yahya Naji, Mehdi Zouaoui, Nourdin Aqodad, Nawal Adali
KEYWORDS:
POEMS Syndrome, CIDP, Polyneuropathy, VEGF, Monoclonal Gammopathy
JOURNAL NAME:
Open Access Library Journal,
Vol.11 No.8,
August
28,
2024
ABSTRACT: Background: POEMS syndrome is a paraneoplastic syndrome resulting from abnormal plasma cell proliferation and associated polyneuropathy (P), organomegaly (O), endocrinopathy (E), monoclonal gammopathy (M), and skin changes (S). Case report: We report the case of a 43-year-old male patient who, since 2015, had presented with a motor deficit with paresthesia and intense pain in both lower limbs that had progressively set over a year. The electromyoneurography (EMNG) was consistent with demyelinating sensorimotor polyradiculoneuropathy predominating in the lower limbs, and a diagnosis of CIDP was made. The patient was administered Ig-IV for five days, followed by azathioprine and corticosteroids. Three years later, the evolution was marked by the appearance of abundant ascites and skin abnormalities such as hyperpigmentation. Abdominal and pelvic MRI revealed, in addition to the Budd Chiari, a sacral osteosclerotic lesion. Serum protein electrophoresis was in favor of monoclonal gammopathy with IGG Lambda light chain, the hormonal profile showed an elevated thyroid-stimulating hormone (TSH), and the vascular endothelial growth factor (VEGF) assay was elevated to 1740 pg/ml. The patient was scheduled for radiotherapy and stem cell transplantation; however, the clinical worsening and deterioration of the patient’s general condition were rapid and fatal. Conclusions: The early diagnosis of POEMS syndrome presents a challenge for clinicians. It is important to conduct detailed interviews and meticulous physical examinations to examine systemic involvement.