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A Retrospective Study on Clinical Features of Childhood Moyamoya Disease
Yao Wang1, Huimin Kong1, Yue Wang1
1Department of Pediatrics, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
Insights
Childhood moyamoya disease (MMD) primarily presents as ischemic stroke, with transient ischemic attacks (TIAs) being the most common initial symptom. Early diagnosis and treatment of MMD in children lead to good prognoses.
Area of Science:
- Neurology
- Pediatrics
- Vascular Neurology
Background:
- Childhood moyamoya disease (MMD) can cause progressive, irreversible neurological damage.
- Early onset of MMD is linked to poorer disease prognosis.
- Understanding MMD's clinical features is crucial for early diagnosis and management in children.
Purpose of the Study:
- To summarize the clinical characteristics of childhood moyamoya disease.
- To aid in the diagnosis and treatment of early-stage MMD in pediatric patients.
Main Methods:
- Retrospective study of 114 children (0-16 years) diagnosed with MMD.
- Analysis of clinical characteristics, sex distribution, and initial attack types.
- Comparison of attack types across different age groups.
Main Results:
- The study included 114 children with MMD (1:1.07 male to female ratio); 6.1% had a family history.
- Mean age of onset was 7.15 ± 3.30 years, peaking between five and eight years.
- Transient ischemic attack (TIA) was the most frequent initial presentation (54.4%), often with limb weakness; attack types varied by age group (P < 0.05).
- Overall prognosis was favorable in 89.6% of cases.
Conclusions:
- Childhood MMD is predominantly ischemic, with TIA as the most common initial symptom.
- Infants with MMD are more susceptible to cerebral infarction, while older children tend to experience TIAs.
- Effective treatments resulted in good outcomes for the studied MMD cases.
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.
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