The Role of Cerebral Hemodynamics in Moya Moya Disease

描述（由申请人提供）：烟雾病是一种大脑底部大动脉闭塞性血管病变。在美国，它最常影响30岁和40岁的女性，导致缺血性中风。这种疾病的自然史尚未确定。血流动力学因素很可能在未来卒中风险中起重要作用。一些患者通过各种手术治疗，旨在改善大脑的侧支血流。这些手术对脑血流和随后降低中风风险的影响尚不清楚。本研究采用盲法，前瞻性研究探讨脑血流动力学因素在烟雾病患者卒中发病机制中的作用。我们将检验以下假设：在接受药物治疗的患者中，闭塞远端大脑半球氧摄取增加是同侧缺血性卒中后续风险的预测因子（具体目标1）。该项目的次要目的是（1）确定该人群中卒中的其他预测因素;（2）获得该人群中不同药物治疗方案效果的初步数据;（3）确定药物治疗患者血流动力学损害的时间变化;（4）确定手术对接受手术血运重建的患者亚组中血流动力学损害的影响;以及（5）获得有和无血流动力学损伤患者的手术并发症发生率的估计值。这些目标将通过使用正电子发射断层扫描（PET）来测量烟雾病患者的局部脑氧摄取分数（OEF）来实现。将在入组时获得有关临床、流行病学和血管造影风险因素的信息。随后每六个月通过电话对患者进行一次随访，以确定随后卒中的发生和预防卒中的中期治疗。PET研究的结果对患者、治疗医生和监测卒中发生的研究者保密。我们将确定局部OEF增加是否与同侧缺血性卒中风险增加相关。我们还将进行多变量分析，以确定该样本中卒中的独立预测因子。我们将对不同药物治疗方案的效果进行事后纵向分析。患者将每年返回进行随访PET研究。

DESCRIPTION (provided by applicant): Moyamoya disease is an obliterative vasculopathy of the large arteries at the base of the brain. In the United States, it most commonly affects women in their third and fourth decades of life, causing ischemic stroke. The natural history of this disorder is not defined. It is very likely that hemodynamic factors play an important role in the risk of future stroke. Some patients are treated surgically with a variety of procedures intended to improve collateral blood flow to the brain. The effect of these procedures on cerebral blood flow and on subsequent stroke risk reduction is unknown. This blinded, prospective study will investigate the role of cerebral hemodynamic factors in the pathogenesis of stroke in patients with moyamoya disease. We will test the hypothesis that increased oxygen extraction in the cerebral hemisphere distal to the occlusion is a predictor of the subsequent risk of ipsilateral ischemic stroke in medically treated patients (Specific Aim 1). The secondary objectives of this project are (1) to determine other predictive factors for stroke in this population; (2) to obtain preliminary data on the effects of different medical treatment regimens in this population; (3) to determine the temporal changes in hemodynamic impairment in medically treated patients; (4) to determine the effects of surgery on hemodynamic impairment in the subset of patients that undergo surgical revascularization; and (5) to obtain estimates of surgical complication rates for patients with and without hemodynamic impairment. These objectives will be accomplished by the use of positron emission tomography (PET) to measure regional cerebral oxygen extraction fraction (OEF) in patients with moyamoya disease. Information on clinical, epidemiologic and angiographic risk factors will be obtained on enrollment. Patients are subsequently followed every six months by telephone to determine the subsequent occurrence of stroke and the interim treatment for stroke prevention. Results of the PET study are kept confidential from the patient, treating physician and investigator monitoring the occurrence of stroke. We will determine if a regional increase in OEF is associated with an increased risk of ipsilateral ischemic stroke. We will also do a multivariate analysis to determine the independent predictors of stroke in this sample. We will perform a post-hoc longitudinal analysis of the effect of different medical treatment regimens. Patients will return annually for follow up PET studies.