30例烟雾病的临床及影像学特征分析

Study on clinical manifestations and imaging characteristics of 30 Moyamoya disease patients

目的研究烟雾病的临床特点、影像表现及其两者之间关系。方法对30例经磁共振血管成像(MRA)和数字减影全脑血管造影(DSA)确诊的烟雾病患者,分析其临床和影像学特征。结果(1)烟雾病发病年龄在35～45岁左右,发病以脑缺血为主,临床表现以单侧肢体无力或偏瘫最为常见。(2)CT和MRI提示,脑出血11例(36.7%),脑梗死10例(33.3%),脑出血合并梗死灶5例。MRA和DSA均发现大脑前、中、后动脉狭窄或闭塞性病变,双侧病变均多于单侧病变,烟雾状异常血管网分别为5例(45.5%)和29例(100.0%);DSA发现动脉瘤3例(10.3%);后交通动脉增粗13例、前交通动脉增粗7例、眼动脉增粗6例及软脑膜吻合支增多4例。4例大面积脑梗死侧枝循环差。结论烟雾病发病高峰为35～45岁;本病临床表现多样,但以脑缺血常见;MRI和MRA为无创伤性,为本病筛选、诊断、随访的重要方法。对临床疑似病例应及早行DSA检查。

Objective Purposes of this paper was to study the clinical manifestations and imaging characteristics and the relation ship between the patients with moyamoya disease. Methods The clinical characteristics of 30 patients who were diagnosed as moyamoya disease by magnetic resonance angiography （MRA） and digital subtraction angiography （DSA） were summarized, and their data of angiography were analyzed simultaneously. Results （1） The age of onset of moyamoya disease had one peak in adults （around 35～45 years old ）. The cerebral ischemia was main type of disease.The main clinical manifestations were unilateral limb weakness or hemiplegia. （2） On CT and MRI,there were cerebral hemorrhage in 7 patients（36.7%） and cerebral infarction in 10 patients（33.3%）,cerebral hemorrhage and cerebral infarction in 5 patients. On MRA and DSA,unilateral or bilateral anterior cerebral artery, middle cerebral artery and posterior cerebral artery existed stenosis or occlusive lesions, bilateral arterial stenosis or occlusion was more than unilateral arterial. moyamoya abnormal vessels were showed in the basal ganglia in 5 patients（45.5%）and 29 patients（100%）especially. On DSA,in all patients,posterior communicating artery was dilated in 13 patients,and anterior communicating artery was dilated in 7 patients,ophthalmic artery（OPHA） was dilated in 6 patients,and leptomeningeal collateral vessels were dilated in 4 patients. The collateral circulation was very bad in 4 patients with excessive cerebral infarction in our study. Conclusion The age of onset of moyamoya disease has one peak in the adults. The clinical manifestations of the disease are various, but cerebral ischemia is the most common feature. MRI and MRA are no vasion and the preferred method for screening test,diagnosis and follow-up survey in this disease. DSA should be performed as early as possible in patients clinically suspected of having moyamoya disease.