Moyamoya is a chronic brain vasculopathy involving the distal intracranial internal carotid artery (ICA) or proximal middle cerebral artery (MCA). Moyamoya patients can be divided into those with primary moyamoya disease (MMD) and those with moyamoya secondary to other known causes such as intracranial atherosclerosis (moymoya syndrome [MMS]). Our aim was to compare the characteristics of MMD patients to those of MMS patients in a sample of Israeli patients seen over the course of 20 years at a tertiary referral center. Included patients were diagnosed with either MMD or MMS based on typical imaging findings and the presence or absence of known concomitant vascular risk factors or associated disorders and vascular disease. Patients with MMS were compared to those with MMD. Demographics, symptoms, signs, and radiological data were compared between the groups. Treatment options and long-term rates of recurrent stroke and functional outcome were also studied. Overall, 64 patients were included (25 MMD, 39 MMS). Patients with MMD were significantly younger (median IQR 20 (7-32) vs. 40 (19-52); p=0.035). Patients with MMS more often had vascular risk factors but there were no significant differences in clinical presentations or long-term disability rates between the groups and a similar proportion of patients underwent surgical interventions to restore hemispheric perfusion in both groups (48% vs. 44% MMS vs. MMD; p=0.7). Almost one in four patient had a recurrent stroke after the initial diagnosis in both groups. Most recurrences occurred in the pre-surgery period in the MMS group and in the post-surgery period in the MMD group. There were no statistically significant differences in clinical or radiological presentations between the MMS and MMD patients. The course is not benign with recurrent stroke occurring in as many as 25%. More data is needed in order to identify those at high risk for stroke occurrence and recurrence.

中文翻译：

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以色列烟雾病与烟雾病综合征的比较

烟雾病是一种慢性脑血管病变，累及颅内颈内动脉远端（ICA）或大脑中动脉近端（MCA）。烟雾病患者可分为原发性烟雾病（MMD）患者和继发于其他已知原因（如颅内动脉粥样硬化）的烟雾病患者（烟雾综合征[MMS]）。我们的目的是比较 20 年来在三级转诊中心就诊的以色列患者样本中 MMD 患者与 MMS 患者的特征。根据典型的影像学表现以及是否存在已知的伴随血管危险因素或相关疾病和血管疾病，纳入的患者被诊断为 MMD 或 MMS。将患有 MMS 的患者与患有 MMD 的患者进行比较。比较各组之间的人口统计学、症状、体征和放射学数据。还研究了治疗方案以及卒中复发率和功能结果的长期发生率。总体而言，纳入了 64 名患者（25 名 MMD，39 名 MMS）。MMD 患者明显更年轻（中位 IQR 20 (7-32) vs. 40 (19-52)；p=0.035）。MMS 患者更常有血管危险因素，但两组之间的临床表现或长期残疾率没有显着差异，并且两组中接受手术干预以恢复半球灌注的患者比例相似（48% 对比 44%） MMS 与 MMD；p=0.7）。两组患者在初次诊断后几乎有四分之一的患者再次出现中风。MMS 组的大多数复发发生在手术前，MMD 组的大多数复发发生在手术后。MMS 和 MMD 患者之间的临床或影像学表现没有统计学上的显着差异。该病程并非良性，复发性中风的发生率高达 25%。需要更多数据来识别中风发生和复发的高风险人群。