脑淀粉样血管病相关炎症两例分析

Analysis on two cases of cerebral amyloid angiopathy-related inflammation

目的总结脑淀粉样血管病相关炎症的临床表现、辅助检查、诊断与治疗及预后特点。方法与结果选择2017年2月至2018年2月共2例老年女性脑淀粉样血管病相关炎症患者,均表现为认知功能障碍、步态异常、日常生活活动能力下降;腰椎穿刺脑脊液压力和白细胞计数大致正常,蛋白定量均升高(988和975 mg/L),1例β-淀粉样蛋白42和40(Aβ_(42)和Aβ_(40))下降、1例Aβ42下降;1例为ApoE基因型ε3/ε3型、1例为ε3/ε4型;MRI均可见片状融合的长T_1、长T_2异常信号影,磁敏感加权成像(SWI)可见多发皮质和皮质下微出血灶;均诊断为脑淀粉样血管病相关炎症;予甲泼尼龙冲击序贯治疗,症状明显改善。结论除认知功能障碍外,步态异常也是脑淀粉样血管病相关炎症的主要临床表现,典型影像学改变是T_2WI和FLAIR成像融合的白质异常高信号影,伴SWI皮质和皮质下多发微出血灶。具有典型临床表现和影像学改变时,明确诊断脑淀粉样血管病相关炎症无须行脑组织活检术,大多数患者免疫调节治疗有效。

Objective To study the clinical features, auxiliary examination, diagnosis andtreatment, and prognosis of cerebral amyloid angiopathy-related inflammation(CAA-ri). Methods and Results Two elderly female cases with CAA-ri during February 2017 to February 2018 in our hospitalwere reported. Both of them manifested with cognitive impairment, gait abnormality and decreased dailylife ability. Lumbar puncture of 2 cases showed the pressure of cerebrospinal fluid(CSF) was normal, whiteblood cell(WBC) count was generally normal and protein quantification was increased(988 and 975 mg/L).One case presented decreased CSF amyloid-β protein 40(Aβ40) and 42(Aβ42), and the other one haddecreased Aβ42. One case was apolipoprotein E(ApoE) ε3/ε3, and the other was ε3/ε4. Brain MRI showedlong T1 and long T2 abnormal signals with flaky fusion, and susceptibility-weighted imaging(SWI) showedmultiple cortical and subcortical microhemorrhage. They were diagnosed as CAA-ri, and the symptomswere obviously improved after methylprednisolone implosive sequential therapy. Conclusions In additionto cognitive impairment, gait abnormality can also be the main clinical manifestation of CAA-ri. Typicalimaging changes are white matter hyperintensity on T2 WI and FLAIR fusion sequences, with multiplecortical and subcortical hemorrhagic lesions on SWI. Once the typical clinical and imaging changes meetthe diagnostic criteria, the diagnosis of CAA-ri can be made without biopsy. Immunotherapy is effective formost patients.