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。
Abstract
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.
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
Key words
cerebral amyloid angiopathy
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]. 神经损伤与功能重建. 2023, 18(6): 334-338
Analysis of Clinical Heterogeneity of Cerebral Amyloid Angiopathy Related Inflammation[J]. Neural Injury and Functional Reconstruction. 2023, 18(6): 334-338
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