Ling Yifeng, De Guio François, Duering Marco, Jouvent Eric, Hervé Dominique, Godin Ophélia, Dichgans Martin, Chabriat Hugues
From the Department of Neurology, GH Saint-Louis-Lariboisière, Assistance Publique des Hoôpitaux de Paris (APHP), Université Paris Denis Diderot and DHU NeuroVasc Sorbonne Paris-Citeé, Paris, France (F.D.G., E.J., D.H., O.G., H.C.); INSERM UMR 1161, Paris, France (Y.L., F.D.G., E.J., D.H., H.C.); Department of Neurology, Huashan Hospital, Fudan University, Shanghai, China (Y.L.); Institute for Stroke and Dementia Research, Klinikum der Universität München, Ludwig-Maximilians-University LMU, Munich, Germany (M. Duering, M. Dichgans); Munich Cluster for Systems Neurology (SyNergy), Munich, Germany (M. Dichgans); and German Center for Neurodegenerative Diseases (DZNE), Munich (M. Dichgans).
Stroke. 2017 Feb;48(2):283-289. doi: 10.1161/STROKEAHA.116.015750. Epub 2016 Dec 29.
Previous studies in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy showed that accumulation of lacunes strongly relate to clinical severity. However, the potential predictors of incident lacunes and their clinical consequences over a short time frame have not been investigated. This study aimed to determine the predictors and clinical impact of such lesions in a large cohort of patients.
Two hundred and six NOTCH3 mutation carriers (mean age, 49.5±10.6 years) were followed up over 3 years. Incident lacunes were identified using difference imaging from 3-dimensional T1 images. Clinical events and change in different clinical scores such as the Mattis Dementia Rating Scale, Modified Rankin Scale, Barthel index, and time to complete part A and part B of Trail Making Test were recorded. Associations were analyzed with multivariable logistic regression analysis and ANCOVA.
Over a mean period of 3.4±0.7 years, incident lacunes occurred in 51 of 206 patients. Both the number of lacunes (P<0.0001) and systolic blood pressure at baseline (P<0.01) were independent predictors of incident lacunes during follow-up. The results were still significant after excluding patients with systolic blood pressure >140 mm Hg. Incident lacunes were also associated with incident stroke and with change in time to complete Trail Making Test part B, initiation/perseveration subscale of the Mattis Dementia Rating Scale and Barthel Index over the study period.
Systolic blood pressure and the number of prevalent lacunes are independent predictors of incident lacunes in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy. These lesions mainly impact executive performances and functional independence over 3 years.
先前关于伴有皮质下梗死和白质脑病的大脑常染色体显性动脉病的研究表明，腔隙灶的累积与临床严重程度密切相关。然而，在短时间内，腔隙灶发生的潜在预测因素及其临床后果尚未得到研究。本研究旨在确定一大群患者中此类病变的预测因素及其临床影响。
对206名NOTCH3突变携带者（平均年龄49.5±10.6岁）进行了3年的随访。利用三维T1图像的差异成像识别新出现的腔隙灶。记录临床事件以及不同临床评分的变化，如马蒂斯痴呆评定量表、改良Rankin量表、Barthel指数以及完成连线测验A部分和B部分的时间。采用多变量逻辑回归分析和协方差分析进行相关性分析。
在平均3.4±0.7年的时间里，206名患者中有51人出现了新的腔隙灶。腔隙灶数量（P<0.0001）和基线收缩压（P<0.01）均为随访期间新出现腔隙灶的独立预测因素。排除收缩压>140 mmHg的患者后，结果仍然显著。在研究期间，新出现的腔隙灶还与新发中风以及完成连线测验B部分的时间变化、马蒂斯痴呆评定量表的起始/持续性子量表和Barthel指数相关。
收缩压和现存腔隙灶数量是伴有皮质下梗死和白质脑病的大脑常染色体显性动脉病中新发腔隙灶的独立预测因素。这些病变在3年内主要影响执行能力和功能独立性。
