成人烟雾病的临床特点及影像分析

目的　研究成人型烟雾病的临床特点、影像表现及其两者的关系。方法　 6 1例经常规血管造影 (包括 DSA)和 MRA确诊的成人型烟雾病患者 (>16岁 )。分析其临床表现及血管造影结果。结果　主要临床表现为突发剧烈头痛、意识障碍 ,伴呕吐、失语及一侧肢体瘫痪。在 CT和 MRI上 ,脑出血 4 1例 (6 7.2 % ) ,脑梗死 15例(2 4 .6 % ) ,脑萎缩 2例 ,无异常表现 3例。在 DSA和 MRA上 ,双侧病变者 32例 ,单侧病变 2 9例 ,5 1例可见到烟雾状异常血管网 ,尤其在出血型患者中。后交通动脉增粗 38支、眼动脉增粗 31支及软脑膜吻合支增多 4 4侧。 5例大面积脑梗死侧支循环很差或缺乏。结论　成人型烟雾病以脑出血多见 ,在发病率和血管造影上和儿童无明显差异。治疗应首选血管重建手术。 DSA为诊断金标准 ,为术前必要检查 ;MRA无创伤性 ,为本病筛选、诊断、随访的首选方法。

Moyamoya disease:analysis of clinical characteristics and angiographic findings

Objective Purposes of this paper was to study the clinical features and angiographic findings and the relationship between them in the patients with moyamoya disease in adult (>16 years). Methods Sixty one patients with adult onset of moyamoya disease had been diagnosed by means of conventional angiography (included digital subtraction angiograph,DSA)and magnetic resonance angiograpyh (MRA).Analyzing the clinical manifestations and angiographic findings of the patients with moyamoya in adults.Results The main clinical manifestations were abrupt strong headache,disturbance of consciousness,and accompanied by vomiting,aphasia and paralysis of hemilateral limb.On CT and MRI, there were cerebral hemorrhage in forty one patients (67.2%) and cerebral infarction in fifteen patients(24.6%),and cerebral atrophy in two patients and normal findings in three patients.On conventional angiography (included DSA) and MRA,bilateral arterial stenosis or occlusion was in thirty two patients and the unilateral in twenty patients.In fifty one patients,moyamoya abnormal vessels were showed in the basal ganglia, especiallyin patients with cerebral hemorrhage.In all patients,posterior communicating artery (ACOA) was dilated in thirty eight sides,and Ophthalmic artery (OPHA) was dilated in twenty nine sides on DSA and in two sides on MRA,and leptomeningeal collateral vessels were dilated in 44 sides.The collateral circulation was very bad in five patients with excessive cerebral infarction in our study.Conclusion The cerebral hemorrhage often occurres in patients with adult onset of moyamoya disease.The difference of the morbility of this disease and the angiographic findings was not significant between the adults and the children patients.The revascularization is the first choice for treatment of moyamoya disease.DSA is gold criterion for moyamoya disease and is necessary to preoperation. MRA is nonvasion and is the preferred method for screening test,diagnosis and follow up survey in this disease.