Progressive Limb Weakness in A Young Man: A Case Report of POEMS Syndrome

Bangladesh Journal of Neuroscience Pub Date : 2019-01-31 DOI:10.3329/bjn.v35i1.57626

A. Chowdhury, Sujan Saha, Md Abdullah Al Muzahid, Uttam Roy, Md Suman Kabir, A. Agarwalla, Samim Reza, Imran Sarker, Md Rafiqul Islam

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Abstract

Polyneuropathy is an initial presentation and essential feature of POEMS ( polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome. Neuropathy is typically distal, symmetric and slowly progressive with demyelinating changes. After a gradual proximal spread, it usually results in severe muscle weakness and functional disabilities. In the present report, we describe a 40-year-old diabetic male presented with gradually progressive weakness of both lower limbs for 1 year followed by the involvement of both upper limbs for the last 3 months. On examination hyperpigmentation, lymphadenopathy, gynecomastia, anasarca, hepatomegaly, bilateral optic disc swelling, sensory-motor polyneuropathy was found. Laboratory findings showed IgG lambda monoclonal gammopathy, raised VEGF, sensory-motor demyelinating and axonal polyneuropathy. All findings were consistent with POEMS syndrome. The patient was treated with lenalidomide and dexamethasone cyclical therapy with some clinical improvement. Bangladesh Journal of Neuroscience 2019; Vol. 35 (1): 51-56

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青年男子进行性肢体无力:POEMS综合征一例报告

多神经病变是POEMS(多神经病变、器官肿大、内分泌病变、m蛋白和皮肤变化)综合征的初始表现和基本特征。神经病变是典型的远端，对称和缓慢进展与脱髓鞘改变。在逐渐近端扩散后，通常会导致严重的肌肉无力和功能障碍。在本报告中，我们描述了一位40岁的男性糖尿病患者，其表现为两下肢逐渐进行性无力，持续1年，最后3个月累及两上肢。检查发现色素沉着、淋巴结肿大、男性乳房发育、腹膜肿大、肝肿大、双侧视盘肿胀、感觉-运动多神经病变。实验室结果显示IgG单克隆γ -病变，VEGF升高，感觉-运动脱髓鞘和轴突多发性神经病。所有结果均符合POEMS综合征。患者给予来那度胺和地塞米松循环治疗，临床有一定改善。2019年孟加拉国神经科学杂志;Vol. 35 (1): 51-56

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Bangladesh Journal of Neuroscience

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