甲状腺功能亢进患者的烟雾综合征：临床特征和手术治疗

Moyamoya syndrome in patients with hyperthyroidism：clinical features and surgical treatment

目的：初步探讨甲状腺功能亢进患者烟雾综合征的临床特征和外科手术疗效。方法回顾性分析2002年12月至2013年4月期间解放军第三〇七医院神经外科收治的41例以甲状腺功能亢进为基础疾病的烟雾综合征患者，收集包括性别、发病年龄、首发症状、进展症状、影像学特征、铃木分期和手术疗效在内的临床资料，并与同时期收治的烟雾病患者的临床资料进行比较。结果伴甲状腺功能亢进的烟雾综合征患者男女之比约为1∶4，发病高峰年龄为25~34岁。与同期收治的烟雾病患者比较，烟雾综合征组首发症状为脑梗死的比例更高（39.0％对24.2％；χ2=4.796，P =0.029），症状进展患者更多（46.3％对25.4％；χ2=9.207，P =0.002），而脑出血比例较低（2.4％对14.6％；χ2=4.829，P =0.028）。对37例接受 EDAS 手术治疗的患者随访（43±19）个月，结果显示31例患者临床症状有不同程度的改善。结论伴甲状腺功能亢进的烟雾综合征多见于女性，脑梗死风险较高，更容易出现病情进展。 EDAS 对于控制甲状腺功能亢进患者烟雾综合征的病情进展的疗效较好。

Objective To preliminarily investigate the clinical features and efficacy of surgical treatment of moyamoya syndrome in patients w ith hyperthyroidism. Methods From December 2002 to April 2013, 41 patients w ith moyamoya syndrome based on the disease of hyperthyroidism admitted to the Department of Neurosurgery, the 307th Hospital of PLA w ere analyzed retrospectively. The clinical data w ere colected, including sex, age of onset, initial symptoms, progress symptoms, imaging features, Suzuki staging, and surgical efficacy, and they w ere compared w ith the clinical data of the patients w ith moyamoya disease treated at the same time. Results The ratio of male to female w as about 1∶4 in moyamoya syndrome patients w ith hyperthyroidism. The peak age of onset w as 25 to 34 years old. Compared w ith the patients w ith moyamoya disease at the same period, the proportion of patients w ith cerebral infarction as initial symptom w as higher in the moyamoya syndrome group （ 39.0% vs.24.2%; χ2 = 4.796, P =0.029）, more patients had symptomatic progression （46.3% vs.25.4%; χ2 =9.207, P =0.002）, and the proportion of of patients w ith cerebral hemorrhage as initial symptom w as low er （ 2.4% vs.14.6%; χ2 = 4.829, P =0.028）. Thirty-seven patients w ho received encephalo -duro-arterio-synangiosis （EDAS） w ere folow ed up for 43 ± 19 months. The results show ed that the clinical symptoms of 31 patients had different degrees of improvement. Conclusions Moyamoya syndrome of hyperthyroidism is more common in w omen. The risk of cerebral infarction is higher and more prone to have disease progression. The efficacy of EDAS is better for controling the progression of moyamoya syndrome in patients w ith hyperthyroidism.