Cerebral infarction in POEMS syndrome Incidence, risk factors, and imaging characteristics
Cerebral infarction in POEMS syndrome Incidence, risk factors, and imaging characteristics
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DOI：
10.1212/wnl.0b013e3181bd136b
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发表时间：
2009-10-20
期刊：
影响因子：
9.9
通讯作者：
Brown, R. D., Jr.
中科院分区：
文献类型：
--
作者：
Dupont, S. A.;Dispenzieri, A.;Brown, R. D., Jr.
目的：确定与 POEMS（多发性神经病、器官肿大、内分泌病、单克隆丙种球蛋白病和皮肤变化）综合征相关的脑梗死的危险因素和发病率。方法：查询 Mayo Clinic 蛋白异常血症数据库，以识别编码诊断为 POEMS 综合征的患者。选择出现 POEMS 相关症状后发生的脑梗塞患者。采用回顾性观察研究设计来评估 POEMS 综合征患者中风的潜在预测因素。结果：总共确定了 9 名脑梗塞患者（10%；95% 置信区间 5.4-17.9）（2 名女性，22%）。传统的卒中危险因素在卒中和非卒中亚组之间没有显着差异，但血液学异常（例如血小板计数升高和骨髓浆细胞增多）在两组之间存在差异。未观察到成功治疗基础疾病后脑梗塞的发生。 CT 和 MRI 数据显示这些患者存在广泛的梗塞地形。常见的中风病因包括疑似血管结构异常导致血管夹层和狭窄，以及近端栓塞。结论：POEMS 综合征患者 5 年发生脑梗塞的风险为 13.4%。骨髓内浆细胞增殖和血清血小板计数升高的证据导致该人群脑梗死的风险增加。我们的结论是，应该积极管理已知的可改变的中风危险因素。无禁忌症的患者应考虑血小板增多症的治疗。治疗该综合征可能是降低这些患者脑梗死风险的最佳方法。神经病学（R）2009；73：1308-1312
Objectives: To determine the risk factors and incidence of cerebral infarction associated with POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome.Methods: The Mayo Clinic dysproteinemia database was queried to identify patients with coded diagnosis of POEMS syndrome. Patients with cerebral infarction, occurring after the onset of POEMS-related symptoms, were selected. A retrospective observational study design was used to evaluate potential predictors of stroke in patients with POEMS syndrome.Results: A total of 9 patients (10%; 95% confidence interval 5.4-17.9) with cerebral infarction were identified (2 women, 22%). Traditional stroke risk factors were not significantly different between the stroke and nonstroke subgroups, but hematologic abnormalities such as elevated platelet count and bone marrow plasmacytosis differed between the 2 groups. Cerebral infarction occurrence after successful treatment of the underlying condition was not observed. CT and MRI data demonstrated a wide spectrum of infarct topography in these patients. Common stroke etiologies comprised suspected vascular structural abnormalities leading to vessel dissection and stenosis, in addition to embolism from a proximal source.Conclusions: The 5-year risk of cerebral infarction in patients with POEMS syndrome is 13.4%. Evidence of plasma cell proliferation within the bone marrow and elevated serum platelet count led to increased risk of cerebral infarction in this population. We conclude that known modifiable stroke risk factors should be aggressively managed. Treatment of thrombocytosis should be considered in patients without a contraindication. Treatment of the syndrome may be the best approach to decreasing risk of cerebral infarction in these patients. Neurology (R) 2009;73:1308-1312