神经肉芽肿病并发系统性血管炎:病例报告与文献综述

Alexander Carvajal-González, Octavio Arevalo-Espejo, Maria Carolina Beeter, Sarwat Umer, Oleg Chernyshev
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摘要

一名 62 岁女性患者,有高血压、糖尿病、冠心病、神经肉芽肿病病史,曾接受开颅手术(脑肿块切除术),两周来头痛、全身无力、呕吐和厌食症状不断加重。脑部核磁共振成像显示,已知的右侧海绵窦肿块有所恶化,但血管炎检查结果呈阴性。患者接受了静脉类固醇治疗;住院期间,她出现了晕厥,头部 CT 正常,心电图显示新的 T 波倒置,肌钙蛋白升高。她的精神状态恶化,左侧偏瘫;头部 CT 显示右侧 MCA 区急性低密度,CTA 显示双侧 M1 区段远端狭窄。由于不符合溶栓/血栓切除术的条件,她开始服用阿司匹林。超声心动图正常。她的右脚趾出现缺血症状,这促使她做了主动脉造影,显示RLE动脉阻塞,因此需要放置SFA支架和氯吡格雷。之后又追加了静脉注射环磷酰胺,但未出现其他血管并发症。该病例说明神经肉芽肿病并发全身性中大血管炎,使用糖皮质激素和细胞毒药物进行积极的免疫抑制后病情好转。
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Neurosarcoidosis complicated by systemic vasculitis.

A 62-year-old woman with medical history of hypertension, diabetes mellitus, coronaropathy, neurosarcoidosis, s/p craniotomy (brain mass resection) presented with worsening headaches, generalized weakness, vomiting, and hyporexia over two weeks. Brain MRI showed worsening of the known right cavernous sinus mass, vasculitis panel was negative. Patient received IV steroids; during hospitalization, she had a syncopal episode, CT Head was normal, EKG showed new T-wave inversion with troponin elevation. She experienced worsening mentation, left-sided hemiparesis; CT head showed acute hypodensity in the right MCA territory, CTA revealed bilateral distal M1 segment stenosis. Ineligible for thrombolysis/thrombectomy, she was started on aspirin. Echocardiograms were normal. Ischemic signs in her right toes prompted an aortogram showing arterial obstructions in the RLE, necessitating SFA stent placement, and clopidogrel. IV cyclophosphamide was added without additional vascular complications. This case illustrates neurosarcoidosis complicated by systemic vasculitis of medium-large vessels, responding to aggressive immunosuppression with glucocorticoids and cytotoxic agents.

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