儿童烟雾病的临床表现及神经影像学特征分析

Clinical manifestations and neuroimaging characteristics of children with moyamoya disease

摘要:

目的：研究儿童烟雾病(MMD)的临床及影像学特征。方法：回顾性分析l7例儿童MMD的临床资料，对其临床表现及神经影像学特征、治疗进行分析。 结果：发病年龄在3~14岁，临床表现为反复发作一侧肢体无力、偏瘫、感觉障碍、头痛等。头颅CT和磁共振（MRI）主要表现为脑梗死，磁共振血管成像(MRA)及数字减影血管造影(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,脑基底部异常血管网形成。结论：儿童MMD发病类型以脑缺血常见，表现为肢体无力或偏瘫、感觉障碍和头痛。DSA检查是确诊烟雾病的主要方法，如具备手术条件的，可及早行血管重建术。［中国当代儿科杂志，2010，12（8）：637-640］

Abstract:

OBJECTIVE: To study the clinical manifestations and neuroimaging characteristics of pediatric moyamoya disease. METHODS: The clinical data of 17 children with moyamoya disease were retrospectively studied. RESULTS: The onset age was between 3 and 14 years. The main clinical manifestations included motor weakness of extremities or hemiplegia, sensory disturbance and headache. Cranial CT or/and MRI examinations predominately showed cerebral infarct. Magnetic resonance angiography (MRA) and digital subtraction angiography (DSA) showed stenosis or occlusion at the terminus of the siphon portions of internal carotid arteries and proximal portions of anterior or middle cerebral arteries, and abnormal vascular networks at the base of brain. CONCLUSIONS: Cerebral ischemia is main clinical manifestations in children with moyamoya disease, presenting motor weakness of extremities or hemiplegia, sensory disturbance and headache. DSA is essential to the diagnosis of the disease.［Chin J Contemp Pediatr, 2010, 12 (8):637-640］