Background

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.

Methods

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.

Results

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%).

Conclusions

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.

中文翻译：

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儿童烟雾病临床特点的回顾性研究

背景

儿童烟雾病 (MMD) 可导致进行性和不可逆的神经损伤。发病年龄较早可能与疾病的最差预后有关。本研究旨在总结儿童MMD的临床特点，以支持早期MMD的诊断和治疗。

方法

回顾性研究2016年10月至2020年4月在我院神经内科和神经外科确诊为MMD的0～16岁患儿，总结MMD患儿的临床特征进行分析，并分析其分布情况。通过数据比较确定不同年龄组的性别和初发类型。

结果

该研究调查了114名患有MMD的儿童（男女比例为1:1.07），其中6.1%的儿童有家族史。平均发病年龄为 7.15 ± 3.30 岁，发病高峰年龄为 5 至 8 岁。最常见的初始发作类型为短暂性脑缺血发作（TIA）（62例，54.4%）伴肢体无力。三个年龄组的首发类型发生率存在差异（P  < 0.05）。86例（89.6%）总体预后良好。

结论

本研究中MMD病例以缺血型为主，TIA是最常见的初发类型。婴儿组更容易发生脑梗塞，而学龄前和学龄组更容易发生TIA。所研究的MMD病例的治疗和预后均取得了良好的效果。