Wang Yao, Kong Huimin, Wang Yue, Jin Peina, Ding Juan, Li Hongwei, Wang Huaili, Zhuo Zhihong
Department of Pediatrics, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
PICU, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
Pediatr Neurol. 2023 Jan;138:17-24. doi: 10.1016/j.pediatrneurol.2022.08.007. Epub 2022 Aug 29.
Childhood moyamoya disease (MMD) can lead to progressive and irreversible neurological impairment. Early age at onset is likely associated with a worst prognosis of the disease. The study aims to summarize the clinical characteristics of childhood MMD for supporting the diagnosis and treatment of early MMD.
A retrospective study was conducted on children aged zero to 16 years who were diagnosed with MMD in the Department of Neurology and neurosurgery of our hospital from October 2016 to April 2020. The clinical characteristics of children with MMD were summarized for analysis, and the distribution of sex and initial attack type among different age groups was determined by data comparison.
The study surveyed 114 children (male to female sex ratio of 1:1.07) with MMD, and 6.1% of them had family history. The mean age of onset was 7.15 ± 3.30 years, and the peak age of onset was five to eight years. The most common initial attack type was transient ischemic attack (TIA) (62 cases, 54.4%) with limb weakness. The incidence of the initial attack type in the three age groups was varied (P < 0.05). The result of overall prognosis was good in 86 cases (89.6%).
In this study, MMD cases were mainly ischemic type and TIA was the most common initial attack type. Infant group was more prone to have cerebral infarction, whereas preschool and school-age groups tended to have TIA. The treatments and prognosis of the studied MMD cases were achieved with good outcomes.
儿童烟雾病（MMD）可导致进行性且不可逆的神经功能损害。发病年龄早可能与该病的不良预后相关。本研究旨在总结儿童烟雾病的临床特征，以支持早期烟雾病的诊断和治疗。
对2016年10月至2020年4月在我院神经内科和神经外科确诊为烟雾病的0至16岁儿童进行回顾性研究。总结分析烟雾病患儿的临床特征，并通过数据比较确定不同年龄组的性别分布和首发类型。
本研究共调查了114例烟雾病患儿（男女比例为1:1.07），其中6.1%有家族史。平均发病年龄为7.15±3.30岁，发病高峰年龄为5至8岁。最常见的首发类型是短暂性脑缺血发作（TIA）（62例，54.4%），伴有肢体无力。三个年龄组首发类型的发生率各不相同（P<0.05）。86例（89.6%）患儿的总体预后良好。
在本研究中，烟雾病病例主要为缺血型，TIA是最常见的首发类型。婴儿组更容易发生脑梗死，而学龄前和学龄组则倾向于发生TIA。本研究中烟雾病病例的治疗和预后效果良好。
