针对CADASIL严重程度的新型分级系统:一个多中心的横截面研究
Bhrugun Anisetti1, Elena Greco1, Eldina Stojadinovic1
1Department of Neurology, Mayo Clinic, Jacksonville, FL, USA.
Cerebral circulation - cognition and behavior
|July 13, 2023
概括
对于带有皮下心脏病发作和白细胞大脑病变 (CADASIL) 的大脑自体主导性动脉病变的新分级系统是可靠和实用的临床使用. 这个系统有效地根据疾病严重程度对患者进行分类,并通过将更高的等级与增加的血管风险因素相关联,帮助研究.
科学领域:
- 神经学 神经学
- 遗传学 是一个遗传学.
- 血管医学 血管医学
背景情况:
- 大脑自体主导性动脉病变与皮下心脏病发作和白细胞脑病变 (CADASIL) 是一种渐进的,遗传性脑微血管病变.
- 卡达西尔在临床表现 (表型) 中表现出显著的变异性.
研究的目的:
- 评估CADASIL在美国多个中心的新分级系统的通用性和可靠性.
- 在现实临床环境中评估拟议的CADASIL分级系统.
主要方法:
- 在5个专门中心对138名已确认CADASIL的成年患者的电子病历 (EMR) 进行回顾性审查.
- 根据临床严重程度,患者被分为5个等级 (0-4). 评估了评价者之间的可靠性 (IRR).
- 人口统计,血管风险因素和神经成像数据被收集和分析.
主要成果:
- 在CADASIL分级系统证明了可接受的评级者之间的可靠性 (κ=0.855).
- 成绩的分布是这样的:0级 (10.9%),1级 (36.2%),2级 (44.2%),3级 (8.7%),以及4级 (0%).
- 较高的严重程度与评估时的年龄较小,高血压和糖尿病的患病率增加以及血管风险因素的较大负担有关.
结论:
- 拟议的CADASIL分级系统是用于表型CADASIL患者的务实和可靠工具.
- 该系统只需要标准的临床数据,使其在常规实践中很容易适用.
- 这种分级系统可以促进对CADASIL的观察和干预研究中的患者分类.
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