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颅内动脉瘤与颈内动脉夹层:神经并发症与遗传相关性。

Cerebral aneurysms and cervical artery dissection: Neurological complications and genetic associations.

机构信息

Departments of Neurology and Public Health Sciences, University of Virginia, Charlottesville, VA, United States.

Departments of Neurology and Public Health Sciences, University of Virginia, Charlottesville, VA, United States.

出版信息

Handb Clin Neurol. 2021;177:241-251. doi: 10.1016/B978-0-12-819814-8.00033-0.

Abstract

Dissections and aneurysms are two of the more common nonatherosclerotic arteriopathies of the cerebrovascular system and a significant contributor to neurovascular complications, particularly in the young. Specifically, ruptured intracranial aneurysms (IA) account for nearly 500,000 cases of subarachnoid hemorrhage annually with a 30-day mortality approaching 40% and survivors suffering often permanent neurologic deficits and disability. Unruptured IAs require dedicated assessment of risk and often warrant serial radiologic monitoring. Cervical artery dissection, affecting the carotid and vertebral arteries, accounts for nearly 20% of strokes in young and middle-aged adults. While approximately 70% of cervical artery dissection (CeAD) cases present with stroke or TIA, additional neurologic complications include severe headache and neck pain, oculosympathetic defect (i.e., partial Horner's syndrome), acute vestibular syndrome, and rarely lower cranial nerve palsies. Both aneurysms and dissections of the cerebrovascular system may occur frequently in patients with syndromic connective tissue disorders; however, the majority of cases are spontaneously occurring or mildly heritable with both polygenic and environmental associations. Fibromuscular dysplasia, in particular, is commonly associated with both risk of CeAD and IA formation. Further research is needed to better understand the pathophysiology of both IA and CeAD to better understand risk, improve treatments, and prevent devastating neurologic complications.

摘要

夹层和动脉瘤是脑血管系统中两种较常见的非动脉粥样硬化性血管病,是神经血管并发症的重要原因,尤其是在年轻人中。具体而言,每年约有近 500,000 例颅内破裂动脉瘤(IA)导致蛛网膜下腔出血,30 天死亡率接近 40%,幸存者常伴有永久性神经功能缺损和残疾。未破裂的 IA 需要专门评估风险,通常需要进行连续影像学监测。颈内动脉夹层(CeAD)累及颈总动脉和颈内动脉,约占年轻和中年成年人中风的 20%。虽然约 70%的颈内动脉夹层(CeAD)病例表现为中风或 TIA,但其他神经并发症包括严重头痛和颈部疼痛、交感神经眼缺陷(即部分霍纳氏综合征)、急性前庭综合征,极少数情况下还会出现颅神经麻痹。脑血管系统的动脉瘤和夹层在伴有综合征性结缔组织疾病的患者中可能经常发生;然而,大多数病例是自发发生的,或具有轻度遗传性,具有多基因和环境关联。纤维肌性发育不良,特别是 CeAD 和 IA 形成的风险增加。需要进一步研究以更好地了解 IA 和 CeAD 的病理生理学,从而更好地了解风险、改善治疗并预防灾难性的神经并发症。

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