36例烟雾病的临床特点及DSA分析

Clinical characteristics of moyamoya disease and its DSA analysis:a study of 36 patients

目的探讨烟雾病的临床特点及DSA的特征。方法对36例经DSA确诊的烟雾病患者,总结其临床特点,同时分析其造影资料。结果①临床特点:烟雾病的发病年龄有两个高峰:5～10岁和30～35岁左右。脑缺血表现27例(75%)、脑出血表现7例(19%)、症状性癫痫2例(6%)。儿童患者(≤15岁)15例(42%),成人患者(>15)21例(58%)。其发病类型均以脑缺血为主,而在临床表现上尤以单侧肢体无力或偏瘫最为常见。②DSA特征:双侧颈内动脉床突上段狭窄或闭塞者22例(61%)、单侧受累者14例(39%);单/双侧大脑前、中、后动脉均存在不同程度的狭窄或闭塞性病变,分别占18例(50%)、20例(56%)及6例(17%);所有患者均存在不同程度的脑底异常血管网形成;本组病例侧支循环丰富,颅底烟雾血管代偿36例(100%)、皮质侧支开放26例(72%)、眼动脉分支代偿3例(8%)、颈外动脉系统代偿19例(53%)。结论烟雾病发病高峰年龄为5～10岁和30～35岁;本病临床表现多样,但以脑缺血最为常见;DSA能对烟雾病作出明确诊断,对临床疑似病例应及早行DSA检查。

Objective To study the clinical characteristics of moyamoya disease and the features of digital subtraction angiography （DSA）. Methods ①The clinical characteristics of 36 patients who were diagnosed as having moyamoya disease by DSA were summarize, and their data of angiography were analyzed simultaneously. Results Clinical characteristics： the age of onset of moyamoya disease had two peaks （around 5 - 10 years old and 30 - 35 years old）. Twenty-seven patients （75%） manifested as cerebral ischemia, 7 （ 19% ） as cerebral hemorrhage, and 2 （6%） as symptomatic epilepsy; 15 children （ ≤ 15 years old, 42% ） , and 21 adults （ 〉 15 year old, 58% ）. Their types of diseases were mainly cerebral ischemia, and their most common clinical manifestations were unilateral limb weakness or hemiplegia. ②The DSA features in 22 patients （61% ） were bilateral internal carotid arteries stenosis or occlusions on the distal segments, and in 14 patients （39%） were affected on one side. Unilateral or bilateral anterior cerebral artery, middle cerebral artery and posterior cerebral artery existed various degrees of stenosis or occlusive lesions, accounting for 50% （ 18 patients）, 56% （20 patients） and 17% （6 patients） , respectively; All patients existed various degrees of abnormal vascular networks at the base of the brain. In addition, the patients in this group had abundant collateral circulation： such as moyamoya vessel compensation at the base of the brain in 36 patients （ 100% ）, opened collateral （ cortical ） branches in 26 patients （72%）, ophthalmic artery branches compensation in 3 patients （8%）, and external carotid artery system compensation in 19 patients （ 53% ）. Conclusions The age of onset of moyamoya disease has two peaks. The clinical manifestations of the disease are various, but cerebral ischemia is the most common feature. Because moyamoya disease can be diagnosed definitely by DSA, so in patients clinically suspected of having this disease DSA should be performed as early as possible.