Takeuchi Yosuke, Murahashi Shuei, Hara Yasuyuki, Nakajima Makoto, Ueda Mitsuharu
Department of Neurology, Kumamoto Rosai Hospital.
Department of Neurology, Graduate School of Medical Sciences, Kumamoto University.
Rinsho Shinkeigaku. 2021 Mar 25;61(3):188-193. doi: 10.5692/clinicalneurol.cn-001534. Epub 2021 Feb 23.
A 76-year-old woman with a 7-year history of dementia presented to our hospital with generalized convulsive seizure for the first time. Contrast-enhanced brain magnetic resonance imaging revealed leptomeningeal enhancement mainly in the right occipital lobe and multiple lobar microbleeds in the bilateral cerebral and cerebellar subcortex. No white matter lesions were observed. A brain biopsy of the right parieto-occipital lobe revealed cerebral amyloid angiopathy (CAA). White matter lesions appeared in the right parieto-occipital lobe three days after the biopsy, and we considered inflammatory CAA. Three courses of methylprednisolone pulse followed by oral prednisolone therapy gradually reduced leptomeningeal and white matter lesions. An apolipoprotein E genotype investigation identified the ε2/ε3 genotype. In patients with inflammatory CAA, a risk of exacerbation should be considered after brain biopsy, in which the ε2 allele might play a role.
一名患有7年痴呆病史的76岁女性首次因全身性惊厥发作前来我院就诊。对比增强脑磁共振成像显示软脑膜强化主要位于右侧枕叶,双侧大脑和小脑皮质下有多个脑叶微出血。未观察到白质病变。右侧顶枕叶脑活检显示为脑淀粉样血管病(CAA)。活检后三天右侧顶枕叶出现白质病变,我们考虑为炎症性CAA。三个疗程的甲泼尼龙冲击治疗后口服泼尼松龙治疗逐渐减轻了软脑膜和白质病变。载脂蛋白E基因型检测确定为ε2/ε3基因型。在炎症性CAA患者中,脑活检后应考虑病情加重的风险,其中ε2等位基因可能起作用。