Long-term Outcomes of Conservative Management in Patients with Moyamoya Disease and Their First-degree Relatives
试验速览
- 阶段
- 不适用
- 状态
- 招募中
- 发起方
- 入组人数
- 400
- 试验地点
- 1
- 主要终点
- Cerebrovascular events
研究概览
简要总结
The purpose of this study is to investigate the long-term outcomes of conservative management in patients with moyamoya disease and their first-degree relatives, and provide potential pathogenesis of moyamoya disease.
详细描述
Moyamoya disease (MMD) is a chronic occlusive-stenosis cerebrovascular disease that characterized by the stenosis of internal carotid artery termination and the formation of net-like vessel. It is a multifactorial disease caused by genetic, inflammatory, immunological and other environmental factors. The specific pathogenesis of MMD is still unclear. The treatment modalities of revascularization and conservative management have been used in patients with MMD. However, the long-term outcomes of MMD with conservative management remain unknown. Also, some first-degree relatives who are carriers of genetic variants occasionally manifest with intracranial arterial stenosis. Therefore, it is significant to detect the long-term outcomes of conservative treatment in MMD patients and their first-degree relatives, and thus provide potential pathogenesis of MMD.
研究设计
- 研究类型
- Observational
- 观察模型
- Cohort
- 时间视角
- Prospective
入排标准
- 年龄范围
- 2 Years 至 60 Years（Child, Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Patients with age between 2-60 years;
- •A clinical diagnosis of moyamoya disease, including unilateral and bilateral disease.
- •Patients are medically treated with antiplatelets, antiepileptics, antihypertensives and vasodilators depending on the presentation.
- •Capable of understanding the purpose and risk of the study and has signed the informed consent. If the participant is not capable of this at the time of enrollment, a legally authorized representative will provide written informed consent in accordance with all regulations.
- •Ability to comply with study follow-up.
排除标准
- •Concomitant other diseases, including systemic vasculitis, neurofibroma, meningitis, sickle cell disease, down's syndrome, and previous basilar radiotherapy.
- •Patients with cardiogenic embolism, including a history of atrial fibrillation, valvular disease or cardiac valve replacement.
- •Patients are allergic to the contrast agents.
- •Patients are treated with direct, indirect, or combined revascularization depending on the presentation.
- •Physical or subjective failure to cooperate with the examination or serious comorbid diseases.
- •Patients are unable or unlikely to return for follow-up visits.
- •Any other reasons that, in the opinion of the investigators, make the participant unsuitable for enrollment.
结局指标
主要结局
Cerebrovascular events
时间窗: 5 years
次要结局
- Change in immunological, inflammatory, angiogenesis biomarkers of peripheral blood(Baseline, 1 year, 2 years, and 5 years during follow-up)
- Identification of RNF213 variants(Baseline)
- Unfavorable neurological outcome (mRS>2)(6 months, 1 year, 2 years, and 5 years during follow-up)
- Change in radiological characteristics as assessed by HR-MRI(Baseline, 1 year, 2 years, and 5 years during follow-up)
- Change in angiographic characteristics as assessed by MRA(Baseline, 1 year, 2 years, and 5 years during follow-up)
- Change in cerebral perfusion status as assessed by CTP(Baseline, 6 months, 1 year, 2 years, and 5 years during follow-up)
- Change in angiographic features as assessed by CTA(Baseline, 6 months, 1 year, 2 years, and 5 years during follow-up)