烟雾病8例临床分析

Clinical analysis on moyamoya disease(8 cases report)

目的 探讨烟雾病的临床特征、影像学诊断价值及治疗。方法 回顾性分析8倒烟雾病患者的临床特征和辅助检查结果及治疗。结果 本组成人（5例）多于儿童（3例）；儿童临床表现以脑缺血症状为主；脑磁共振血管成像（MRA）或数字减影血管造影（DSA）检查显示颈内动脉虹吸部末端和大脑前或中动脉近端狭窄或闭塞，并有脑基底部异常血管网形成。内科保守治疗5倒。合并手术治疗3倒，进行0．5—5．0年随访，1例遗留轻度神经系统异常后遗症。结论烟雾病儿童主要表现为脑缺血症状；脑MRA或DSA的异常征象是早期诊断烟雾病的重要依据。早期诊断、及时治疗是改善烟雾病预后的有效方法。

Objective To investigate clinical features, neuroradiologieal diagnosis and therapy of moyamoya disease. Methods Restrospectire analyze of 8 cases of nnoyamoya＇s clinical features, neuroradiological diagnosis and therapy. Results In this group, there were more adults （ five cases） than children patients （ three cases） ;Children patients predominantly brain ischemia symptoms ; Stenosis or occlusion at the terminus of the siphon portions of internal carotid arteries （ICA） and preximalportions of anterior or middle cerebral arteries （ ACA or MCA）, and abnormal vascular networks at the base of brain were noted by the magnetic resonance angiography（MRA） and digital subtraction radiography（DSA）. All cases received neurological internal medical treatment and three cases of patients were conducted operation. The follow - up ranged from six months to five years and found only one patients have abnormal neurological sequelae. Conclusion Children patients of moyamoya disease presents predominantly brain ischemia symptoms, MRA or DSA of brain investigation are the main way to diagnose moyamoya disease in the early age. Early diagnosis and promptly medical intervention is the effect way to improve prognosis of moyamoya disease.