脑淀粉样血管病相关炎症1例附文献分析

Cerebral amyloid angiopathy-related inflammation:a case report with literature analysis

目的探讨脑淀粉样血管病相关炎症的临床表现、血清及影像学检查、诊断、治疗及预后。方法选取2019年1月20日1例中年男性脑淀粉样血管病相关炎症患者,临床表现以认知行为异常、步态异常、人格改变等为主要特征。结果脑脊液生化:脑脊液总蛋白0.36 g/L,自身免疫系列、自身免疫性脑炎相关指标等检查未见异常。头颅MRI:T2WI及FLAIR上可见斑片状或融合的白质异常高信号影,非对称性并延伸至皮质下白质。磁敏感加权成像(SWI)提示≥1处皮质-皮质下微出血灶。诊断为很可能的脑淀粉样血管病相关炎症,给予甲泼尼龙冲击疗法,人免疫球蛋白序贯疗法,硫唑嘌呤后续疗法。结论甲泼尼龙冲击疗法后临床出现戏剧性效果;人免疫球蛋白静点治疗后近期效果不明显;除认知行为异常、步态异常等临床表现外,精神人格改变,如被迫害妄想、幻听幻视也是该病常见临床表现;出院后持续口服硫唑嘌呤及醋酸泼尼松,病情进一步好转中。

Objective To discuss the clinical manifestations,serology and imaging examinations,diagnosis,treatment and prognosis of cerebral amyloid angiopathy-related inflammation(CAA-ri).Methods A middle-aged male patient diagnosed with CAA-ri on January 20,2019 was selected.The clinical manifestations were mainly featured by abnormal cognitive behavior,abnormal gait,and personality changes.Results For cerebrospinal fluid biochemistry,with cerebrospinal fluid total protein of 0.36 g/L,autoimmune disease series,autoimmune encephalitis related indicators and other examinations showed no abnormalities.For brain MRI,patchy or fused white matter hyperintensities could be seen on T2WI and FLAIR which was asymmetric and extended to the subcortical white matter.Sensitivity-weighted imaging(SWI)revealed≥1 foci of cortical-subcortical microhemorrhage.The patient was diagnosed with probable CAA-ri,and was given methylprednisolone pulse therapy,sequential therapy of human immunoglobulin followed by azathioprine treatment.Conclusion Methylprednisolone pulse therapy shows dramatic clinical effects.The intravenous injection of human immunoglobulin shows no obvious short-term effect.In addition to the clinical manifestations of abnormal cognitive behavior and gait,changes in mental personality,such as persecutory delusions,auditory and visual hallucinations,are also common clinical manifestations of the disease.After discharge,with continuous oral administration of azathioprine and prednis one acetate,his condition is further improved.