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。
Objective:By analyzing the clinical data of patients with cerebral amyloid angiopathy related in-flammation(CAA-ri),the heterogeneity in clinical and imaging features,prognosis or other aspects was summa-rized,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 di-versity 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 behav-ior abnormality are frequently seen.There were common features of asymmetrical white matter lesions,microhe-morrhage 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 diag-nosis.
作者
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
YANG Yi-shu;YI Li;ZHAO Yuan;LI Ji-mei;XU Chun-ling;WANG Shu-hui;ZHAO Ying-ying;SUN Jin-mei;TUO Hou-zhen(Department of Neurology,Beijing Friendship Hospital,Capital Medical University,Beijing 100050,China)
出处
《神经损伤与功能重建》
2023年第6期334-338,共5页
Neural Injury and Functional Reconstruction
基金
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)。
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
cerebral amyloid angiopathy
cerebral amyloid angiopathy related inflammation
β-amyloid pro-tein
clinical heterogeneity
