成人烟雾病的临床特点及预后与非遗传因素的相关性研究（英文）

Association of clinical features and prognosis with non-genetic factors in adult-onset moyamoya disease

目的描述成人烟雾病的临床及预后特点并比较非遗传因素与预后之间的相关性。方法回顾性分析该院109例成人烟雾病患者的临床资料,根据CT结果分为脑出血组和脑缺血组,并进行随访分析。结果成人烟雾病患者最常见的临床症状是头疼(38.5%,42/109),其大多数血管造影分级为铃木3期(65.1%)。成人烟雾病患者脑出血的比例(67.0%)高于脑缺血(33.0%),38例患者(34.9%)在CT或MRI上发现多发性病灶。在随访的73例脑出血患者中34例(46.6%)呈现反复的脑出血发作。脑出血与死亡人数、动脉瘤、复发脑出血事件及高三酰甘油呈明显的正相关(P<0.05)。脑出血、脑血管事件的复发次数、高血压与死亡之间有明显的相关性(P<0.05)。患者的2、4、6、8和10年的总生存率分别为98.1%、89.4%、79.1%、77.8%和66.6%。结论成人烟雾病患者发生脑出血及头疼症状的比例更高。高三酰甘油和高血压可能在疾病的发展过程中,特别在脑出血的患者中起到一定的作用。

[Objective] To describe the clinical characteristics and outcomes of patients with adulthood- onset moyamoya disease （A-MMD） and compare the correlations between non-genetic factors and the clinical outcome. [Methods] The clinical data of 109 consecutive adult moyamoya patients in our hospital were retro- spectively analyzed. These patients were divided into cerebral hemorrhage group and cerebral ischemia group based on the results of CT and were conservatively followed up. [Results] The most common cortical clinical manifestation was headache （38.5%, 42/109） and most of the hemispheres were in Suzuki and Takaku angio- graphic stage Ⅲ （65.1%）. There was a higher percentage of hemorrhage （67.0%） than ischemia （33.0%）, and 38 patients （34.9%） had multiple lesions on CT or MRI. There were 34 patients （46.6%） presented with re- peated episodes of cerebral hemorrhage in the 73 patients with hemorrhage. There were positive correlations between brain hemorrhage （at the first episode） and death toll, aneurysm, recurrent event type （hemorrhage） and high triglyceride （P 〈 0.05）. The features of CT/MRI at the first diagnosis （hemorrhage）, number of recur- rent stroke events, hypertension and recurrent event type （hemorrhage） were significantly associated with the risk of death （P〈0.05）. The overall survival in 2, 4, 6, 8 and 10 years were 98.1%, 89.4%, 79.1%, 77.8% and 66.6% respectively. [ Conclusions ] A-MMD patients have high percentages of hemorrhage and headache. High triglyceride and hypertension may play some roles in the development of A-MMD, especially in patients with cerebral hemorrhage.