Neurology:炎性脑淀粉样血管病1例

2019-07-28 杨中华 脑血管病及重症文献导读

60岁,女性,既往反应迟钝。

60岁,女性,既往反应迟钝。

记忆力丧失1个月,头痛2周。

头 MRI 见下图。A 图,T2相可见融合的脑白质病,延伸到皮层下白质,伴占位效应。B 图,10天后 DWI 发现脑梗死。C 图,GRE 显示微出血:



脑DSA,脑脊液水痘-带状疱疹病毒 PCR和免疫球蛋白 G,B/C 肝炎,CSF 隐球菌抗原,HIV,莱姆病血清学检查和梅毒筛查皆未见异常。

最初的鉴别诊断包括 CNS 血管炎和脑内淋巴瘤

基于临床放射学标准(敏感性为82%,特异性为97%)(JAMA Neurol 2016;73:197–202),更正诊断为炎性脑淀粉样血管病(inflammatory cerebral amyloid angiopathy),新发脑梗死提示β淀粉相关血管炎亚型。

给予激素治疗,几天后患者完全清醒(fully alert),5个月后复查 MRI 显示脑白质病改善(下图)。图 A 和 D 为首次MRI,图 B 和 E 为激素治疗3周时 MRI,图 C 和 F 为激素治疗5个月后 MRI 显示脑白质病明显改善:





原始出处:Kim DD1, Gofton T1, Young GB2. Teaching NeuroImages: Steroid-responsive leukoencephalopathy in inflammatory cerebral amyloid angiopathy. Neurology. 2019 Jun 4;

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    2020-07-03 yinhl1978
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