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目的分析和探讨甲状腺功能亢进型烟雾病患者临床特征、发病机制及治疗效果。方法回顾性分析军事医学科学院附属医院神经外科经MRA或DSA确诊为烟雾病的患者10例,所有患者经内分泌科会诊符合甲亢诊断标准。9例患者经抗甲亢治疗,病情稳定后根据病人情况选择单侧或双侧(一般为单侧)脑硬模颞浅动脉血管融通术,1例患者单纯抗甲亢药物治疗。结果 10例患者中,7例为女性,3例为男性,甲状腺功能均异常。2例患者免疫球蛋白升高,4例患者抗核抗体谱有异常。对10例患者进行头颅部CT/MR检查,全部表现为脑梗死,均为双侧。9例患者待甲亢控制平稳后进行脑硬模颞浅动脉血管融通术。有1例患者在明确诊断后给予抗甲状腺治疗及保守治疗。对10例患者的随访时间为5~48个月。9例患者经甲亢和手术(EDAS)治疗后均恢复良好,复查MRI未出现新的梗死灶。8例术后(12~24个月)复查DSA提示,颅外已经向颅内大量代偿供血,语言不清、肢体麻木、无力等症状均减轻或消失。结论甲亢型烟雾综合征患者女性发病率高于男性,临床症状主要表现为脑梗死,二者合并发病可能与免疫和遗传等多种因素有关。积极抗甲亢治疗是手术治疗烟雾病的基础,待甲亢控制平稳后进行脑硬模颞浅动脉血管融通术是预防缺血性卒中的有效方法之一。
Objective To analyze and explore the clinical features, pathogenesis and therapeutic effect of hyperthyroidism moyamoya disease. Methods A retrospective analysis of 10 cases of moyamoya disease confirmed by MRA or DSA in neurosurgical department of Affiliated Hospital of Academy of Military Medical Sciences was made. All patients were diagnosed as hyperthyroidism by endocrine department. Nine patients were treated with anti-hyperthyroidism. After stable condition, unilateral or bilateral (usually unilateral) cerebral superficial temporal artery vascular surgery was selected according to the patient’s condition. One patient was treated with anti-hyperthyroidism alone. Results Of the 10 patients, 7 were female and 3 were male, with abnormal thyroid function. Two patients had elevated immunoglobulin, and four patients had abnormal antinuclear antibody. Ten patients underwent cranial CT / MR examination, all showed cerebral infarction, are bilateral. Nine patients were treated with hypertensive intracranial superficial temporal artery after ventricular hypertrophy control. One patient was given anti-thyroid and conservative treatment after a definite diagnosis. Ten patients were followed up for 5 to 48 months. All 9 patients recovered well after hyperthyroidism and surgery (EDAS), and no new infarction was found on MRI. 8 cases of postoperative (12 to 24 months) review of DSA tips, extracranial cranium has been a large number of compensatory blood supply, language is unclear, limb numbness, weakness and other symptoms are reduced or disappeared. Conclusions The incidence of Hyperthyroidism patients with smoke syndrome is higher than that of men, and the clinical symptoms mainly include cerebral infarction. The combination of the two may be related to many factors such as immunity and heredity. Active treatment of anti-hyperthyroidism is the basis of surgical treatment of moyamoya disease to be stable after hyperthyroidism for the treatment of cerebral superficial temporal artery shunt is one of the effective ways to prevent ischemic stroke.