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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 and prognostic features of adult moyamoya disease and to compare the correlation between non-genetic factors and prognosis. Methods The clinical data of 109 adult patients with moyamoya disease in our hospital were retrospectively analyzed. According to the results of CT, the patients were divided into cerebral hemorrhage group and cerebral ischemia group and followed up. Results The most common clinical symptom of adult moyamoya headache was headache (38.5%, 42/109). Most of the angiography grading was stage 3 (65.1%) in Suzuki. The proportion of patients with adult moyamoya disease was higher (67.0%) than those with cerebral ischemia (33.0%) and 38 patients (34.9%) had multiple lesions on CT or MRI. Of the 73 patients with ICH followed up, 34 (46.6%) had recurrent episodes of ICH. Cerebral hemorrhage was positively correlated with death toll, aneurysm, recurrent cerebral hemorrhage and triglyceride (P <0.05). Cerebral hemorrhage, the number of recurrent cerebrovascular events, hypertension and mortality were significantly correlated (P <0.05). The overall survival at 2, 4, 6, 8 and 10 years was 98.1%, 89.4%, 79.1%, 77.8% and 66.6%, respectively. Conclusion The incidence of cerebral hemorrhage and headache in adult patients with moyamoya disease is higher. Hypertriglyceridemia and hypertension may play a role in the development of the disease, especially in patients with cerebral hemorrhage.