鲁西南地区烟雾病临床特点及其外科治疗策略

Clinical characteristics of Moyamoya disease in the southwest of Shandong Province and its surgical treatment strategies

目的探讨鲁西南地区烟雾病临床特点和外科治疗经验。方法对自2011年3月至2013年12月济宁医学院附属医院神经外科收治的96例烟雾病患者临床资料进行回顾性分析。并将分析结果与日韩及国内相关报道分析比较。结果鲁西南地区烟雾病在发病年龄、性别比及临床表现方面具有特殊性，与日韩及国内相关报道不完全一致。具体表现为：女性多于男性（与日韩相似）；有0～10岁及3l～50岁2个发病高峰期（与日韩相似）；但发病年龄更集中（与国内报道相似1且儿童发病率（12．5％1明显低于日韩；临床表现以缺血型为主（80．2％），但短暂脑缺血发作（TIA）的发生率高f高于日韩及国内报道），癫痫和脑梗死的发生率低（低于日韩及国内报道），脑出血的发生率（19．8％1与日本及国内报道相似，明显低于韩国。经颅内外血管重建术治疗后大部分患者的临床症状得到改善．与日韩及国内相关报道的治疗效果无明显差异。结论鲁西南地区的烟雾病临床特点具有特殊性，其发病机制可能与日韩存在差异；颅内外血管重建术治疗烟雾病临床效果显著，是治疗烟雾病的有效方法。

Objective To explore the clinical characteristics of patients with Moyamoya disease in the southwest of Shandong province and evaluate the efficacy of cerebral revaseularization treatment in it. Methods Clinical data of 96 patients with Moyamoya disease, admitted to our hospital form March 2011 to December 2013, were analyzed retrospectively and compared with those in Japan, South Korea and Inland. Results The clinical characteristics of patients with Moyamoya disease in the southwest of Shandong province were similar with that in Japan, South Korea and Inland, but enjoyed their own particularity： the age distribution showed two peaks （aged 0-10 and aged 31-50） and the sex ratio （female〉male） was similar to those among Japanese and Korean patients, but the age distribution was more concentrated; the incidence of children （12.5%） was significantly lower than that of Japan and South Korea; the main type of Moyamoya disease was the ischemic one （80.2%）; the occurrence of transient ischemic attack was higher than that in Japan, South Korea and Inland; the incidences of epilepsy and cerebral infarction were lower than those in Japan, South Korea and Inland; the incidence of cerebral hemorrhage （19.8%） was similar to Japan and Inland, and significantly lower than that in Korea. The clinical symptoms of most Moyamoya disease patients were improved with the treatment of extracranial-intracranial revascularization; and the treatment outcomes showed no significant difference between Chinese, Japanese and Korean. Conclusions The clinical features and pathogenesis mechanism of Moyamoya disease in the southwest of Shandong province may be different form Japan and South Korea. Extracranial-intracranial revascularization is a effective treatment for Moyamoya disease.