【摘要】 脑淀粉样血管病相关炎症（cerebral amyloid angiopathy-related inflammation,CAA-ri）是由β淀粉样蛋白（amyloid β,Aβ）沉积于中枢神经系统血管壁而引起的一种罕见的中枢神经系统血管炎。本文报告1例患者，临床表现为发作性肢体无力、精神异常、头痛、认知功能减退、癫痫和尿潴留。MRI显示脑叶皮质及皮质下白质多发病灶伴出血，增强后部分病灶呈脑回样强化。脊髓MRI显示病变累及圆锥。该患者曾诊断为“短暂性脑缺血发作”及“病毒性脑膜脑炎”，经治疗后症状仍然加重。最终通过脑组织病理活检，确诊为CAA-ri。予激素治疗后，患者仍然死亡，总病程7个月。累及脊髓的CAA-ri临床罕见，通过对该病例分析及相关文献复习，以提高临床医生对该病的早期识别和诊断，从而帮助患者改善预后。更多还原

【Abstract】 Cerebral amyloid angiopathy-related inflammation(cerebral amyloid angiopathy–related inflammation,CAA-ri) is a rare central nervous system(CNS) vasculitis caused by the deposition of Abeta protein in the blood vessel walls of the CNS. This article reports a patient with episodic limb weakness, mental disorders,headache, cognitive decline,epilepsy, and urinary retention. MRI showed multiple lesions in the cerebral cortex and subcortical white matter with partial hemorrhage, some lesions showed gyri-like enhancement after MRI enhancement. MRI of the spinal cord showed lesions involving the conus. The patient had been misdiagnosed with "transient ischemic attack" and "viral meningoencephalitis", and his symptoms continued to worsen after treatment. The patient was eventually diagnosed with CAA-ri disease by pathological brain biopsy. Hormone therapy was given again later, but the patient still died. The total disease duration was7 months. CAA-ri involving the spinal cord is clinically rare. The analysis of this case and the review of related literature can help clinicians to improve the early identification and diagnosis of this disease, thereby helping these patients to improve their prognosis.更多还原