儿童与成人烟雾病临床表现及神经影像学特征分析

目的探讨儿童和成人烟雾病（MMD）的临床表现、神经影像学特征、预后等方面的区别。方法对19例儿童和55例成人MMD患者的临床资料进行回顾性分析，并对其l临床症状、神经影像学等进行对照研究。结果发病年龄高峰儿童为6～10岁，成人为36～40岁。以缺血性脑血管疾病为首发症状者，儿童组显著高于成人组（P〈0．05）；以出血性脑血管疾病为首发症状者，成人组显著高于儿童组（P〈0．01）。儿童患者头颅CT和磁共振（MRI）主要表现为脑梗死（73．7％），成人患者主要表现为颅内出血（58．2％）。数字减影血管造影（DSA）均可发现烟雾状异常血管网，儿童组主要表现为双侧颈内动脉、大脑中动脉和大脑前动脉狭窄，而成人组主要表现为双侧颈内动脉、大脑中动脉和大脑前动脉闭塞。结论儿童与成人MMD在起病形式、临床症状、神经影像学特征等方面存在一定的差异，在临床实践中应注意鉴别，将有助于MMD的个体化治疗。

Analysis of clinical manifestation and neuroimaging characteristics between child and adult patients with moyamoya disease

Objective To explore the differences in clinical manifestations, neuroimaging characteristics and prognosis between child and adult patients with moyamoya disease （MMD）. Methods A retrospective study was performed in 19 children and 55 adults with MMD. Their clinical manifestations and neuroimaging characteristics were comparatively analyzed. Results The peak of onset age of children and adults with MMD was 6 - 10 and 36 - 40 years ,respectively. The ratio of the children with ischemic cerebrovaseular disease as the first symptom was higher than that of the adults （ P 〈 0.05 ）, while the ratio of the adults with hemorrhagic cerebrovascular disease as the first symptom was higher than that of the children （P 〈 0.01 ）. Cranial CT and MRI examinations showed predominately cerebral infarct in child patients （73.7%） and intracranial hemorrhage in adult patients （58.2%）. Abnormal vascular networks were seen in all patients by digital subtraction angiography （DSA）. Furthermore, the child patients showed predominately stenosis of bilateral internal carotid artery （ICA）, middle cerebral artery （MCA） and anterior cerebral artery （ACA）, whereas the adult patients mainly presented occlusion of bilateral ICA, MCA and ACA. Conclusion There are differences of onset pattern, clinical symptoms, neuroimaging characteristics between child and adult patients with MMD and they should be paid close attention to identification in clinical practice,which is helpful to the individual treatment of patients with MMD.