INVESTIGATION OF CENTRAL NERVOUS SYSTEM VASCULAR DISORDERS

中枢神经系统血管疾病的调查

• 批准号: 3922620
• 负责人: E H OLDFIELD
• 金额: --
• 依托单位: NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE
• 依托单位国家: 美国
• 资助国家: 美国
• 起止时间: 至
• 项目状态: 未结题
• 来源: https://reporter.nih.gov/project-details/3922620
• 关键词: blood flow measurement; blood pressure; cardiovascular disorder diagnosis; cerebral hemorrhage; cerebrovascular disorders; cerebrovascular imaging /visualization; congenital arteriovenous shunt; hemodynamics; heterodyning; human subject; magnetic resonance imaging; myelinopathy; spinal cord disorders; spinal cord imaging /visualization

The clinical features, arteriographic findings, and treatment of 81 patients with spinal arteriovenous malformations demonstrated that there are distinguishing clinical features in patients with arteriovenous malformations of the spinal cord compared to those of patients with arteriovenous fistulas of the spinal dura. The findings indicate that spinal arteriovenous fistulas are acquired lesions, and not congenital, as was previously thought, and support arteriovenous malformations of the spinal cord as congenital lesions. The findings also indicate that AVMs of the spinal cord produce myelopathy as a result of high blood flow, but that patients with spinal dural AV fistulas develop myelopathy as a result of increased venous pressure in the spinal cord. Magnetic resonance imaging permits demonstration of the presence and site of arteriovenous malformations of the spinal cord and therefore is a valuable and safe, non-invasive technique to investigate patients suspected of having spinal AVMs. Foix-Alajounine syndrome was demonstrated to be due to venous congestion, and not venous thombosis, and therefore, amenable to reversal by treatment of the spinal AVMs. Spinal AVM were shown to recanalize consistently after embolic occlusion. Patients with Von Hippel-Lindau disease were investigated and the following were shown: 1) investigation of the molecular biology of the hemangioblastomas of the central nervous system revealed a homozygous deletion of a portion of the short arm of the third chromosome, demonstrating that these tumors are probably caused by the absence of a tumor suppressing gene, as are familial retinoblastomas. MRI with gadolinium EDTA contrast enhancement was shown to be a sensitive technique of detection for small hemangioblastomas of the central nervous system. Excision of the tumors alone was shown to result in resolution of syringomyelia associated with spinal cord hemangioblastomas. Therefore the tumor-associated syrinx does not need separate treatment.

动脉粥样硬化的临床特征、动脉造影表现和治疗 81例脊髓动静脉畸形患者证实 有区别的临床特征， 脊髓动静脉畸形与 硬脊膜动静脉瘘的患者。 的 研究结果表明，脊髓动静脉瘘是后天性的， 病变，而不是先天性的，因为以前认为，和支持 先天性脊髓动静脉畸形 病变 研究结果还表明，脊髓AVM 由于高血流量而产生脊髓病，但是， 患有硬脊膜动静脉瘘的患者发展为脊髓病， 脊髓静脉压升高的结果。 磁共振成像可以证明 和脊髓动静脉畸形的部位， 因此是一种有价值的、安全的、非侵入性的技术， 调查疑似患有脊柱AVM的患者。 Foix-Alajounine综合征被证明是由于静脉 充血，而不是静脉血栓形成，因此， 通过治疗脊柱动静脉畸形来逆转。 显示脊髓AVM 在栓塞闭塞后持续再通。 对Von Hippel-Lindau病患者进行了调查， 主要内容如下：1）对黑曲霉的分子生物学研究 中枢神经系统的血管母细胞瘤显示出 第三基因的短臂的一部分的纯合缺失 染色体，表明这些肿瘤可能是由 缺乏肿瘤抑制基因，如家族性的 视网膜母细胞瘤 MRI增强扫描， 这是一种灵敏检测技术， 中枢神经系统的血管母细胞瘤 切除 单独的肿瘤可导致脊髓灰质炎的消退 与脊髓血管母细胞瘤有关。 因此 肿瘤相关的空洞不需要单独治疗。