Wang Xiao-Peng, Li Jing-Jie, Wang Qian-Nan, Gao Gan, Yu Dan, Zhang Qian, Liu Si-Meng, Wang Min-Jie, Bao Xiang-Yang, Duan Lian
Senior Department of Neurosurgery, The First Medical Center of Chinese PLA General Hospital, Beijing, China.
Medical School of Chinese PLA, Beijing, China.
CNS Neurosci Ther. 2024 Dec;30(12):e70165. doi: 10.1111/cns.70165.
Moyamoya disease (MMD) and moyamoya syndrome (MMS) are rare cerebrovascular conditions with unclear distinctions in clinical presentation and prognosis.
This study assessed potential differences between MMD and MMS patients using real-world data on clinical manifestations, surgical outcomes, and stroke risk factors.
This multicenter, retrospective cohort study examined patients with MMD or MMS treated at three tertiary academic hospitals in China, with a mean follow-up of 11.2 ± 3.1 years. Clinical differences were compared between MMD and MMS, and postoperative cerebrovascular events were compared between patients who underwent surgery and those with conservative management. Primary outcomes were postoperative ischemic and hemorrhagic strokes. Risk factors were evaluated via multivariate Cox regression analysis.
Of the 2565 patients, 2349 had MMD and 216 had MMS. After 1:1 propensity-score matching, no significant differences were observed between these two cohorts. Surgical patients had fewer cerebrovascular events than those who received conservative treatment (HR, 0.487; 95% CI, 0.334-0.711; p < 0.001). Preadmission modified Rankin scale scores > 2 (HR, 3.139; 95% CI, 1.254-7.857; p = 0.015) and periprocedural complications (HR, 8.666; 95% CI, 3.476-21.604; p < 0.001) were independent stroke risk factors in patients with MMD. Periprocedural complications (HR, 31.807; 95% CI, 10.916-92.684; p < 0.001) increased stroke risk in patients with MMS.
This real-world study revealed substantial clinical overlap between MMD and MMS. Both groups derived significant benefits from surgical revascularization, suggesting distinction may not be necessary to guide surgical management decisions. Optimizing preoperative status and preventing periprocedural complications may improve outcomes in these rare cerebrovascular conditions.
This study has been registered in the Chinese Clinical trial registry (registration number: ChiCTR2200064160).
烟雾病（MMD）和烟雾综合征（MMS）是罕见的脑血管疾病，临床表现和预后的区别尚不清楚。
本研究利用关于临床表现、手术结果和卒中危险因素的真实世界数据，评估MMD和MMS患者之间的潜在差异。
这项多中心、回顾性队列研究对在中国三家三级学术医院接受治疗的MMD或MMS患者进行了检查，平均随访时间为11.2±3.1年。比较了MMD和MMS之间的临床差异，以及接受手术治疗的患者和接受保守治疗的患者术后脑血管事件的情况。主要结局是术后缺血性和出血性卒中。通过多变量Cox回归分析评估危险因素。
在2565例患者中，2349例患有MMD，216例患有MMS。经过1:1倾向评分匹配后，这两个队列之间未观察到显著差异。手术患者的脑血管事件比接受保守治疗的患者少（风险比，0.487；95%置信区间，0.334 - 0.711；p < 0.001）。入院前改良Rankin量表评分>2（风险比，3.139；95%置信区间，1.254 - 7.857；p = 0.015）和围手术期并发症（风险比，8.666；95%置信区间，3.476 - 21.604；p < 0.001）是MMD患者独立的卒中危险因素。围手术期并发症（风险比，31.8
