老年男性,四肢僵硬半年余—POEMS综合征  

An elderly man with stiff limbs for over half one year: POEMS syndrome

作　　者：李小双 李洵桦[1] 李晶晶[1] LI Xiaoshuang;LI Xunhua;LI Jingjing(Department of Neurology,The First Affiliated Hospital,Sun Yat-Sen University,Guangdong Provincial Key Laboratory of Diagnosis and Treatment of Major Neurological Diseases,National Key Clinical Department and Key Discipline of Neurology,Guangzhou 510080,China)

机构地区：[1]中山大学附属第一医院神经科,广东省重大神经疾病诊治研究重点实验室,国家临床重点专科和国家重点学科,广州510080

出　　处：《中国神经精神疾病杂志》2022年第10期636-640,共5页Chinese Journal of Nervous and Mental Diseases

摘　　要：报告1例老年男性患者,既往糖尿病史,主要症状为头面部和四肢关节僵硬、活动困难,主要体征为锥体束征阳性,检查结果提示周围神经损害及血钙明显升高。早期予扩容、利尿、血液透析降钙、改善肌张力及营养神经等治疗,血钙未下降,病情无明显好转。进一步行血尿本周蛋白、血管内皮生长因子(vascular endothelial growth factor,VEGF)、骨髓流式+活检检查、肌电图及正电子发射计算机断层显像(positron emission tomography with computer tomography,PET/CT)检查,明确诊断为POEMS(polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes,POEMS)综合征。转入血液科并予达雷妥尤单抗、硼替佐米及地塞米松方案治疗后,患者症状明显好转。本病例存在多个与经典POEMS综合征相比非典型的症状体征和辅助检查结果,增加了诊断的难度。提示系统性疾病导致的关节僵硬易与肌张力增高混淆,对于存在周围神经损害的系统性病变患者,应该注意浆细胞病如POEMS综合征的诊断,POEMS综合征患者也可能存在高钙血症,早期诊断及时治疗可改善患者预后。We reported an elderly man with a history of diabetes mellitus, whose main symptom was joint stiffness in limbs, trunk and head. He had positive pyramidal sign, peripheral neuropathy and hypercalcemia. Treatment with hydration enhancement, diuresis and haemodialysis failed to attenuate hypercalcemia. The patient’s symptoms still worsened after the treatment of neuroprotective and skeletal muscle relaxant agents. Further examinations of blood and urine Bence-Jones protein, vascular endothelial growth factor(VEGF), flow cytometry analysis, biopsy of bone marrow, and positron emission tomography with computer tomography(PET/CT) confirmed the diagnosis of polyneuropathy,organomegaly, endocrinopathy, M-protein, skin changes(POEMS) syndrome. The patient gradually improved with the treatment of daretuzumab, bortezomib and dexamethasone following transferred to the hematology department. The patient’s atypical symptoms and examination results increased the difficulty of diagnosis, suggesting that joint stiffness caused by systemic diseases can be easily misled to muscular hypertonia. Given that hypercalcemia can also be occurred in patients with POEMS syndrome, the plasma cell dyscrasia especially POEMS syndrome should not be ignored in patients with peripheral neuropathy and systemic disorders. Early diagnosis and treatment can improve the prognosis.

关 键 词：POEMS综合征 高钙血症 多发性周围神经病 恶性浆细胞病 M蛋白 甲状旁腺激素样蛋白 血管内皮生长因子 

分 类 号：R741[医药卫生—神经病学与精神病学]