文章摘要
杨伊姝,易立,赵媛,李继梅,许春伶,王淑辉,赵莹莹,孙金梅,脱厚珍.脑淀粉样血管病相关炎症临床异质性分析[J].神经损伤功能重建,2023,(6):334-338
脑淀粉样血管病相关炎症临床异质性分析
Analysis of Clinical Heterogeneity of Cerebral Amyloid Angiopathy Related Inflammation
  
DOI:
中文关键词: 脑淀粉样血管病  脑淀粉样血管病相关炎症  β-淀粉样蛋白  临床异质性
英文关键词: cerebral amyloid angiopathy  cerebral amyloid angiopathy related inflammation  β-amyloid protein  clinical heterogeneity
基金项目:国 家 自 然 科 学 基 金-面上项目(No. 82071257); 吴阶平医学基金会 临床科研专项资助 基金(No. 320.6750. 2020-06-18)
作者单位
杨伊姝,易立,赵媛,李继梅,许春伶,王淑辉,赵莹莹,孙金梅,脱厚珍 首都医科大学附属 北京友谊医院神经 内科 
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中文摘要:
      目的:分析脑淀粉样血管病相关炎症(CAA-ri)患者的临床资料,总结其临床、影像学特征及治疗预后 等方面的异质性。方法:选择脑淀粉样血管病相关炎症患者6例,均行头颅核磁检查,其中3例行脑脊液相 关检查,2例行APOE基因型检测,2例行病理活检。分析患者的临床资料。结果:该病好发于老年男性,临 床异质性包括病程多样性以及症状体征多样性,可为急性、亚急性或慢性病程,亦可为少见的发作性病程; 认知障碍、肢体无力、言语障碍和精神行为异常等症状临床出现频次较高。影像学具有共性的不对称白质 病变及微出血、含铁血黄素沉积等特征,但病灶范围、程度差异巨大。治疗及转归亦呈高度异质性,激素等 免疫相关治疗对重症患者有效,轻症患者可呈自限性病程,但易复发。 结论:CAA-ri具有高度临床异质性, 但其影像学表现的共性特征可高度提示诊断,激素等治疗有效协助进一步临床确诊。
英文摘要:
      By analyzing the clinical data of patients with cerebral amyloid angiopathy related inflammation (CAA-ri), the heterogeneity in clinical and imaging features, prognosis or other aspects was summarized, so as to guide the diagnosis and treatment of the disease. Methods: Clinical data of 6 CAA-ri inpatients or outpatients in the Department of Neurology in our hospital from 2019 to 2021 were enrolled. All the patients underwent cranial MRI scans, of which 3 underwent cerebrospinal fluid related examination, 2 underwent APOE genotype detection, and 2 underwent pathological biopsy. Results: The clinical heterogeneity includes the diversity of disease course, symptoms and signs. CAA-ri may be acute, subacute or chronic disease, or a rare course of attack. Symptoms such as cognitive impairment, limb weakness, speech impairment and mental behavior abnormality are frequently seen. There were common features of asymmetrical white matter lesions, microhemorrhage and hemosiderin deposition in MRI, but the range and degree of lesions varied greatly. Hormone and other immune therapies are effective in severe cases. Mild patients may have a self-limited course, but are prone to recurrence. Conclusion: CAA-ri has a high degree of clinical heterogeneity, but the common features of its imaging findings can highly suggest diagnosis, and hormone therapy can effectively assist further clinical diagnosis.
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