78例儿童烟雾病和烟雾综合征的临床特征及影像分析

目的分析儿童烟雾病及烟雾综合征临床特点及影像资料,根据年龄发育阶段研究首发症状及临床特征,探讨脑梗死发生与颈内动脉病变的关系。方法对2002年1月~2009年3月连续住院的78例儿童烟雾病及烟雾综合征患者的病历资料进行回顾性分析。分析患儿的年龄及性别分布特点,首发症状在不同年龄段、不同类型(出血型和缺血型)中的特点。将脑血管造影(digital subtraction angiography,DSA)图像清晰的缺血型烟雾病分为短暂性脑缺血发作(transient ischemic attack,TIA)组和脑梗死(cerebral infarction,CI)组,比较两组的Suzuki分期差异。结果 78例患儿中男36例(46.2%),女42例(53.8%)。起病年龄18个月~17岁,平均(8.55±3.80)岁,5~10岁患儿47例(60.3%)。幼儿期首发症状仅见肢体无力和抽搐,发作性头痛从学龄前期开始出现,学龄期开始出现视力障碍和不自主运动,记忆力下降等。脑缺血型烟雾病72例(92.3%)。缺血型首发症状多见TIA 31例(43.1%)、肢体瘫痪15例(20.8%)、发作性头痛22例(30.6%)、肢体麻木11例(15.3%)、抽搐8例(11.1%)、言语障碍6例(8.3%)等。出血型首发症状为头痛伴意识障碍(50%)、伴肢体无力(50%)、伴言语障碍(16.7%)。CI部位多在额颞顶叶,脑血管病变主要累及颈内动脉系统。出血部位多在脑室系统(50%),其次为基底节(33.4%),丘脑和额颞叶各占16.7%。TIA组(27例)和CI组(27例)脑血管造影Suzuki分期无显著性差异(χ2=1.034,P=0.596)。结论 5~10岁为儿童烟雾病的发病高峰期,临床以缺血型为主,出血型少见。不同年龄发育阶段临床首发症状有所差异。以TIA和CI为表现的缺血型烟雾病患者DSA的Suzuki分期无显著差异。

Clinical Characteristics and Imaging Analysis of Moyamoya Disease and MoyamoyaSyndrome in Children

Objective To analyze initial symptoms, clinical characteristics and radiological findings of
moyamoya disease (MMD) in children and the association between cerebral infarction and stenoocclusive
lesions of advanced Suzuki stage.
Methods The clinical and imaging data of 78 children with MMD who were successively
hospitalized in Beijing Tiantan Hospital from Jan. 2002 to Mar. 2009 were analyzed retrospectively.
The ischemic MMD was divided into transient ischemic attack (TIA) group and cerebral infarction
(CI) group. Evaluate two groups' Suzuki stages in digital subtraction angiography (DSA).
Results There were 36 boys and 42 girls; mean onset age of MMD was (8.55±3.80) years (range
from 1 year 6 months to 17 years). Ages at onset between 5 years and 10 years in children were 47
cases (60.3%). There were 72 of cerebral ischemic and 6 of cerebral hemorrhage. The most frequent
initial symptoms of ischemic MMD were weakness of limbs (46, 63.9%), TIA (31, 43.1%) and
hemiplegia (15, 20.8%), headache (22, 30.6%), sensory impairment (11, 15.3%), seizure (8, 11.1%),
speech disturbance (6, 8.3%). The initial symptoms of hemorrhagic MMD were frequent headache,
disturbance of consciousness. The bleeding parts were mainly located at the ventricular system. The
frontal/temporal parietal lobe infarctions were involved and the internal carotid artery system was
mainly impaired in MMD. There were no statistically significant differences between TIA group and CI group in Suzuki stages (χ 2=1.034, P =0.596).
Conclusion The peak onset of MMD in children is 5~10 years old. There were different early
clinical characteristics in different developmental stages. It seems that the occurrence of CI in
MMD does not associate with the occlusion severity of the internal carotid artery.