Mirjam Heldner，Urs Fischer，Marcel Arnold

　　瑞士伯尔尼大学小岛医院

　　Mirjam Heldner  瑞士伯尔尼大学小岛医院神经内科医学博士，研究领域为神经病学、卒中和多发性硬化。

　　Urs Fischer 瑞士伯尔尼大学小岛医院神经内科医学博士，研究领域包括神经病学的药物治疗、急诊医学等。

　　Marcel Arnold  瑞士伯尔尼大学小岛医院神经内科教授，擅长脑卒中、血管夹层的发病机理、影像学及诊断与治疗。

　　急性脊髓缺血综合征（ASCIS）、脊髓出血和脊髓血管畸形是脊髓血管病变最常见的3种类型。ASCIS罕见，常无前驱短暂脊髓缺血发作。临床表现各异。尚无循证治疗指南，治疗推荐来自缺血性卒中、动脉粥样硬化性血管疾病和急性脊髓损伤数据。多数中心使用阿司匹林。少数病例报告成功溶栓治疗。脊髓出血也较罕见，多数病因未明。患者常表现为急性背部或颈部剧烈刀割样、放射性疼痛。外科手术为治疗方法，保守治疗可用于部分病例。脊髓血管畸形有3种，分别是硬脊膜动静脉瘘（AVF）、脊髓动静脉畸形（AVM）和脊髓海绵状血管瘤。早期识别硬脊膜AVF并及时强化治疗可预防神经功能进一步下降。外科手术或血管内栓塞术封堵可稳定甚至改善临床情况。大多数症状或神经功能恶化的脊髓海绵状血管瘤患者可行外科手术治疗。对小部分脊髓AVM，放射治疗可能是治疗选择。但对脊髓海绵状血管瘤，放射治疗并非可接受的治疗选择。

　　对于脊髓血管病变，早期介入十分必要。患者常有长期剩余功能障碍，并可再次引起并发症，包括痉挛、疼痛，膀胱、肠道和性功能障碍，应予以治疗。专业康复可增加患者独立生活机会。

　　Acute spinal cord ischemia syndrome (ASCIS)， spinal cord hemorrhage and spinal vascular malformations are the three major groups of vascular lesions of the spinal cord.

　　Acute spinalcord ischemia syndrome (ASCIS)

　　ASCIS is rare， diagnostically challenging and mostly a single event. It accounts for up to 8% of all acute myelopathies and 2% of all vascular neurological pathologies. Interventions and pathologies affecting the aorta are main causes of ASCIS. Symptoms classically occur acute and often show a stepwise deterioration. Mostly， there is no preceding transient ischemic attack of the spinal cord. Clinical presentation is variable. Complete or incomplete myelopathy， para- or quadriparesis， loss of sensation， especially of pain and temperature， paresthesias and loss of bladder function and/or associated-often severe， radiating-back pain occur. Predictors of poor functional long-term outcome are severe initial neurological deficits， especially motor deficits， lack of improvement within 24 hours， affection of the cone， and higher age. The frequency of recovery to independent gait varies from 11% to 46%， 20%～57% of the patients remain wheelchair bound. Case fatality rates range from 9% to 20%. There are no evidence-based treatment guidelines. Treatment recommendations are derived from data of cerebral ischemic stroke， atherosclerotic vascular disease and acute spinal cord injury. Acetylsalicylic acid is used in most centers. Thrombolysis has been successfully applied in few case reports， though remains experimental. Rarely， steroids or antibiotics are administered in the rare case when underlying autoimmune vasculitis or infectious diseases are present. Pathologies affecting the aorta as well as underlying cerebrovascular conditions should be treated whenever possible. ASCIS may occur after aortic surgery， less often after thoracic endovascular aortic repair (TEVAR). Besides acute management， secondary prevention is important.

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