Chu Shuguang, Xu Feijia, Su Ya, Chen Hong, Cheng Xin
Department of Radiology, Huashan Hospital, Fudan University, Shanghai, China.
Department of Radiology, Shanghai Tenth People's Hospital, Tongji University, Shanghai, China.
J Alzheimers Dis. 2016;51(2):525-32. doi: 10.3233/JAD-151036.
Cerebral amyloid angiopathy-related inflammation (CAA-ri) is a relatively rare syndrome of reversible encephalopathy and could be divided into two subtypes of inflammatory CAA (ICAA) and amyloid-β-related angiitis (ABRA) according to histopathology. We present a case of pathologically proved ABRA with partial seizures and headache, and a focal lesion in the right temporal lobes on magnetic resonance imaging. Summarized from previous 139 ABRA and ICAA cases, ABRA is preferred when the lesion is enhanced on MRI and requires combination drug therapy, while ICAA is highly suspected with ApoE genotype of ɛ4/ɛ4. More clinical markers for diagnosis of CAA-ri warrant further researches.
脑淀粉样血管病相关炎症(CAA-ri)是一种相对罕见的可逆性脑病综合征,根据组织病理学可分为炎症性CAA(ICAA)和淀粉样β相关血管炎(ABRA)两种亚型。我们报告一例经病理证实的ABRA病例,患者有部分性癫痫发作和头痛症状,磁共振成像显示右侧颞叶有局灶性病变。综合此前139例ABRA和ICAA病例来看,当病变在MRI上有强化表现且需要联合药物治疗时,倾向于诊断为ABRA,而当载脂蛋白E基因型为ɛ4/ɛ4时,则高度怀疑为ICAA。更多用于诊断CAA-ri的临床标志物有待进一步研究。