19例儿童烟雾病的诊断及治疗

The diagnosis and treatment of childhood Moyamoya disease in 19 cases

目的 探讨儿童烟雾病的临床特征、影像学诊断价值及预后。方法 对 19例烟雾病患儿的临床特征和辅助检查结果进行分析 ,并对治疗后的 13例患儿进行随访。结果 本组男 13例 ,女6例 ,发病年龄 2岁～ 13岁 2个月 ,5岁以上 12例 ( 63 %)。患儿临床表现以肢体无力或偏瘫 ( 17例 )、运动性失语 ( 11例 )及头痛 ( 10例 )为主。脑磁共振血管造影 (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 18例 ,手术治疗 1例 ,对其中 13例进行 9个月至 5年 4个月 (平均 3年 3个月 )的随访 ,10例遗留神经系统异常症状或体征 ,3例无任何神经系统后遗症。结论 肢体无力或偏瘫、运动性失语、头痛是烟雾病的突出症状 ;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 Moyamoya disease (MMD) is a chronic occlusive cerebrovascular disease of unknown etiology, which occurs predominantly in childhood. Patients under 15 years of age at onset account for 60%-80% of the total cases. Since the cases of childhood MMD have not been widely reported in our country, this study aimed at exploring the clinical characteristics and prognosis of the disease and diagnostic values of neuroradiological findings to help improve our understanding of MMD. Methods The clinical data of 19 inpatients with MMD admitted to our hospital between December 1989 and January 2000 were analyzed. Patients in this study consisted of 13 males and 6 females, aged from 2 years to 13 years and 2 months,of whom 12 cases were beyond 5 years of age(63%). And follow-up period ranged from 9 months to 5 years and 4 months (mean 3 years and 3 months) in 13 cases in terms of residual neurological symptoms, daily activities, etc. Results The main clinical symptoms were motor weakness of extremities or hemiplegia (17 cases), motor-aphasia (11cases), and headache (10 cases). Stenosis or occlusion at the terminus of the siphon portions of internal carotid arteries (ICA) and proximal portions of anterior or middle cerebral arteries (ACA or MCA), and abnormal vascular networks at the base of brain were noted by the digital subtraction angiography (DSA) or magnetic resonance angiography (MRA). The medical therapy was followed by 18 cases, while 1 case underwent a surgical treatment. The follow-up of 12 cases with medical therapy showed neurological impairment in 10 cases, of which motor impairment in 6, transient ischemic attack (TIA) or headaches in 2, epilepsy in 2, motor-aphasia in 1 and intellectual deterioration in 5. The other 2 cases had a relatively favorable outcome without neurological sequelae. Of the 12 cases, 9 could live on themselves. The surgically treated patient remained absent of any neurological sequelae or intellectual impairment for a 2-year and 5-month follow-up period. Conclusions Motor weakness of extremities or hemiplegia, motor-aphasia and headache are the predominant clinical features of MMD .The findings of MRA or DSA are essential to the early diagnosis of the disease. Early diagnosis and timely treatment contribute to the improvement of the prognosis of MMD.