烟雾病临床特点分析

Clinical characteristics of moyamoya disease

目的分析经颅多普勒超声（TCD）问世后，烟雾病的临床特点。方法总结了北京协和医院自TCD应用于临床后（1992-2004年）的54例烟雾病的特点。35例（65％）通过数字减影血管造影（DSA）确诊，19例（35％）通过磁共振血管成像（MRA）确诊。结果（1）起病年龄有两个高峰，10岁左右和40岁左右。≤25岁年龄组19例（35％，中位数9岁），〉25岁年龄组35例（65％，中位数39岁）。（2）最常见的首发症状包括脑梗死和短暂性脑缺血发作（TIA）在内的缺血性血管病，共39例（72％）。以缺血为首发症状的发生频率在不同年龄组分别为：〈16岁年龄组87％；16—30岁年龄组55％和〉30岁年龄组71％。脑出血8例（15％），发病年龄17—49岁。其余7例首发症状表现为非典型血管病临床表现。确诊时为脑出血的10例患者均直接经DSA检查诊断，而非出血的44例患者中的37例（84％）先经TCD筛查而后行DSA或MRA确诊为烟雾病。结论TCD能够发现更多缺血性和表现为非典型血管病临床症状的烟雾病患者，我们的资料显示，成年发病的烟雾病患者较儿童更多。此外，虽然脑出血好发于成年人，但无论儿童期还是成年期，烟雾病的临床表现都以缺血症状为主，尤其是TIA。

Objective To analyze the clinical manifestations of （ moyamoya disease, MMD） since transcranial Doppler （TCD） is available to be a screen method for diagnosing the intracranial artery stenosis in Chinese. Methods Totally 54 MMD patients in Peking Union Medical College Hospital during 1992 to 2004 were enrolled in this study. Of them, 35 （65%） patients were diagnosed by using DSA and 19 （35%） using MRA. Results There were two age peaks, one around 10 years old and the other around 40 years old. 19 （35%） of patients were less than 25 years old, with a median of 9 years old and 35 （65%） of patients were more than 25 years old, with a median of 39 years old. Among 54 MMD patients, cerebral ischemia occurred in 39 （72%） and was shown as the commonest initial clinical manifestation. The frequency of cerebral ischemia were 87% in the age group of less than 16 years old （ 13/15 patients） ; 55% in 16-30 years old group （6/11 patients） and 71% in the more than 30 years old group （20/28 patients）. 8 （ 15% ） patients had cerebral hemorrhage. The remaining 7 （ 13% ） patients had symptoms other than cerebral ischemia or hemorrhagic stroke. All 10 patients with hemorrhagic symptom were diagnosed by DSA directly. Among 44 patients with non-hemorrhagic symptoms were screened by using TCD first and then diagnosed by using DSA or MRA. Conclusion Our data suggest that TCD should be able to identify a wilder spectrum of MMD with mild or atypical symptoms, lschemic stroke is the major clinical manifestation both in children and adult MMD patients.