TY - JOUR T1 -成人烟雾病患者白质病变的特征及临床意义JF -神经学JO -神经学DO - 10.1212/WNL.0000000000207130首页Sp - 10.1212/ wnl.0000000000207130AU - Wookjin Yang AU - Keun-Hwa Jung AU - Dong-Wan Kang AU - Eung-Joon Lee AU - hanyeong Jeong AU - Matthew Chung AU - Youngjoon Kim AU - Jiyeon Ha AU - Jeong- min Kim AU - seung hoon Lee Y1 - 2023/03/06 UR - http://n.首页neurology.org/content/early/2023/03/06/WNL.0000000000207130.abstract N2 -背景和目的白质高强度(WMH)据报道在烟雾病(MMD)中增加;然而，由于其分布的病理生理异质性，其临床重要性尚不明确。本研究旨在评估WMH的负担和模式及其在烟雾病轨迹中的临床意义。方法无明显结构病变的烟雾病患者在性别和血管危险因素方面与健康对照1:1倾向评分匹配。全部、心室周围和皮质下的WMH体积被完全自动分割和量化。WMH体积随年龄变化呈去趋势，并在两组之间进行比较。基于铃木分期和未来缺血事件的烟雾病严重程度与WMH体积的关系被评估。结果对161对烟雾病患者和对照组进行了分析。MMD与WMH总体积增加显著相关(B[标准误差]，0.126 [0.030]; p<0.001), periventricular WMH volume (0.114 [0.027]; p<0.001), and periventricular-to-subcortical ratio (0.090 [0.034]; p=0.009). In the MMD subgroup (n=187), advanced MMD had an independent positive association with the total WMH volume (0.120 [0.035]; p<0.001), periventricular WMH volume (0.110 [0.031]; p<0.001), and periventricular-to-subcortical ratio (0.139 [0.038]; p<0.001). Periventricular WMH volume (adjusted hazard ratio [95% confidence interval], 5.12 [1.26–20.79]) and periventricular-to-subcortical ratio (3.80 [1.51–9.56]) were associated with future ischemic events in medically followed-up MMD patients. However, no demonstrable association was found between subcortical WMH volume and MMD, MMD severity, or future ischemic events.DiscussionPeriventricular WMH, but not subcortical WMH, may represent the main pathophysiology of MMD. Periventricular WMH may be used as a marker for ischemic vulnerability in patients with MMD. ER -