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 investigate the clinical features, pathogenesis and treatment effects of patients with moyamoya syndrome associated with hyperthyroidism. Methods The clinical data of 10 patients with moyamoya syndrome associated with hyperthyroidism from department of neurosurgery were analyzed retrospectively. These patients were diagnosed MMS by magnetic resonance angiography (MRA) or digital subtract angiography (DSA). All the patients met the diagnostic criteria of hyperthyroidism. After controlling hyperthyroidism, 9 patients underwent encephalo-duro-arterio-synangiosis, the other 1 was only given drugs to control hyperthyroidism. Results 10 patients (7 females) showed thyroid dysfunction. 2 patients showed high level of immunoglobulin. 4 patients showed high level of antinuclear antibody spectrum. 10 patients showed cerebral infarction by CT or MR and all patients had bilateral lesions. After controlling hyperthyroidism, 9 patients underwent encephalo-duro-arterio-synangiosis, the other 1 was only given drugs to control hyperthyroidism. 10 patients were followed-up for 5～48 months. The symptoms of 9 patients were relieved or disappeared after the treatment. 8 of them conducted postoperative DSA (12～24 month) and all showed a great deal of compensatory blood supply from extracranial to intracranial arteries. Conclusion Moyamoya syndrome associated with hyperthyroidism is mostly occurred in female patients and their clinical symptoms are mainly cerebral infarction. The pathogenesis of moyamoya syndrome associated with hyperthyroidism may be associated with a variety of genetic and immune factors. After controlling hyperthyroidism, encephalo-duro-arterio-synangiosis is thought to be an appropriate management for prevention further ischemic stroke.