Cerebral large artery stenosis and occlusion in POEMS syndrome.

Cerebral large artery stenosis and occlusion in POEMS syndrome.
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POEMS综合征患者大脑大动脉狭窄闭塞。

DOI:
10.1186/s12883-021-02260-2
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发表时间:
2021-06-24
期刊:
影响因子:
2.6
通讯作者:
Kuwabara S
Kuwabara S
中科院分区:
医学4区
文献类型:
--
作者:
Sugiyama A;Yokota H;Misawa S;Mukai H;Sekiguchi Y;Koide K;Suichi T;Matsushima J;Kishimoto T;Tanei ZI;Saito Y;Ito S;Kuwabara S

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本研究旨在探讨多发性神经病、脏器肿大、内分泌疾病、M蛋白和皮肤变化综合征(POEMS)患者脑动脉狭窄和闭塞的发生率及其危险因素。我们回顾了2010至2017年间61例POEMS综合征患者的磁共振血管成像(MRA)或计算机断层血管成像(CTA)结果。在最初的MRA/CTA中评估狭窄或闭塞。采用多因素分析确定动脉狭窄/闭塞的危险因素。在一例尸检病例中,对闭塞的大脑中动脉进行了病理检查。29例(47.5%)患者在首次 检查中发现大脑主要动脉狭窄或闭塞(> 50 %)。受累部位以颈内动脉最多(32.8 %),其次为大脑前动脉(21.3%)和中动脉(16.4 %)。基底动脉(1.3 %)和椎动脉(3.6 %)受累较少。发生脑梗塞8例(13.1 %)。血清血管内皮生长因子水平是狭窄/闭塞的独立预测因子(优势比,1.228;95 %可信区间,1.042-1.447;P = 0.014)。尸检显示大脑中动脉闭塞的原因是内弹力板断裂,内膜纤维性和粘液性增厚。23例患者随访时狭窄改善、恶化和不变分别为20.7 %、8.7 %和69.6 %。脑部大血管狭窄或闭塞常见于约一半的POEMS综合征患者。血管病变与血清血管内皮生长因子水平有关,从而与疾病活动性有关。建议对这些患者进行脑血管评估,以改善治疗。网上版载有补充材料,可在10.1186/s12883-021-02260-2查阅。
This study aimed to investigate the frequency and risk factors for cerebral artery stenosis and occlusion in patients with polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome. We reviewed results of magnetic resonance angiography (MRA) or computed tomography angiography (CTA) in 61 patients with POEMS syndrome seen between 2010 and 2017. Stenosis or occlusion was assessed in the initial MRA/CTA. Multivariate analysis was used to identify risk factors for artery stenosis/occlusion. In an autopsy case, pathologic examination was conducted of the occluded middle cerebral arteries. Stenosis (> 50 %) or occlusion of the major cerebral arteries was found in 29 (47.5 %) patients on the initial MRA/CTA. The internal carotid artery was involved most frequently (32.8 %), followed by the anterior (21.3 %) and middle (16.4 %) cerebral arteries. The basilar (1.3 %) and vertebral (3.6 %) arteries were rarely affected. Cerebral infarction developed in eight (13.1 %) patients. The serum vascular endothelial growth factor (VEGF) level was an independent predictor for stenosis/occlusion (odds ratio, 1.228; 95 % confidence interval, 1.042–1.447; P = 0.014). An autopsy study showed occluded middle cerebral arteries by fibrous and myxomatous thickening of intima with splitting of the internal elastic lamina. Follow-up MRA in 23 patients showed improved, worsened, and unchanged stenosis in 20.7 %, 8.7 %, and 69.6 %, respectively. Cerebral large-vessel stenosis or occlusion is frequently seen in approximately half of patients with POEMS syndrome. Vasculopathy was related to serum VEGF levels and thereby disease activity. Assessment of cerebral vessels is recommended in these patients to improve management. The online version contains supplementary material available at 10.1186/s12883-021-02260-2.
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影响因子: 4.6
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