A retrospective study on clinical features of childhood moyamoya disease



      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 0-16 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 attacks type among different age groups were determined by data comparison.


      The study surveyed 114 children (the 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 old and the peak age of onset was 5 to 8 years old. The most commonly initial attacks type was transient ischemic attack (TIA) (62 cases, 54.4%) with limb weakness. The incidence of the initial attacks 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 commonly initial attacks type. Infant group was more prone to have cerebral infarction, while preschool and school-age groups tended to have TIA. The treatments and prognosis of the studied MMD cases were achieved with good outcomes.


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