首页|抗接触蛋白相关蛋白2抗体相关脑炎临床特征分析

抗接触蛋白相关蛋白2抗体相关脑炎临床特征分析

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目的 探讨抗接触蛋白相关蛋白2(CASPR2)抗体相关脑炎的临床特征。方法 回顾性分析2018年1月至2022年12月于郑州大学第一附属医院确诊的24例抗CASPR2抗体相关脑炎患者的临床资料。依据发病年龄分为早发型10例(发病年龄<45岁)和晚发型14例(发病年龄≥45岁),比较两组间患者临床表现、辅助检查及治疗反应的差异。结果 24例患者表现为癫痫、认知障碍及精神异常者各13例,伴自主神经症状14例,周围神经过度兴奋症状8例,莫旺综合征5例,步态不稳5例,睡眠障碍8例。10例早发型患者中女性7例,表现为癫痫者8例(8/10),癫痫的发生率高于晚发型患者(5/14,P=0。047)。14例晚发型患者中,女性6例,表现为认知障碍者9例,精神异常者8例,头颅磁共振成像(MRI)异常者6例;晚发型患者的脑脊液蛋白水平[0。37(0。29,0。58)g/L]高于早发型[0。22(0。16,0。30)g/L,Z=-2。667,P=0。008]。晚发型患者治疗前后的改良Rankin量表(mRS)评分分别为(3。29±0。83)分和1。50(0。75,2。25)分,均高于早发型患者[治疗前后mRS评分为(2。10±0。99)分和 0(0,1。00)分;t=-3。188,P=0。004;Z=-2。335,P=0。020]。结论 抗CASPR2抗体相关脑炎症状多样。早发型以女性多见,癫痫发生率更高。晚发型以男性多见,认知障碍及精神异常表现突出,对日常生活能力损害更重,头颅MRI异常多见,脑脊液蛋白更高。抗CASPR2抗体对老年患者神经系统的免疫损伤可能更为严重。
Analysis of clinical characteristics of autoimmune encephalitis with antibodies against contactin-associated protein-like 2
Objective To explore the clinical characteristics of patients with antibodies against contactin-associated protein-like 2(CASPR2).Methods The clinical data of 24 patients with anti-CASPR2 encephalitis diagnosed at the First Affiliated Hospital of Zhengzhou University from January 2018 to December 2022 were retrospectively analyzed.According to the age of first onset,the patients were divided into early onset group(10 cases,onset age<45 years)and late onset group(14 cases,onset age≥45 years).The clinical data including clinical manifestations,auxiliary examinations,and treatment response between these 2 groups were compared.Results Among the 24 patients,there were 13 cases with epilepsy,13 cases with cognitive decline,13 cases with mental disorders,14 cases with autonomic dysfunction,8 cases with peripheral nerve hyperexcitability,5 cases with Morvan syndrome,5 cases with unstable walking,and 8 cases with sleep disorders.Among the 10 cases of the early onset group,7 cases are females,and 8 cases showed epilepsy.The incidence rate of epilepsy in the early onset group was higher than that in the late onset group(5/14,Fisher exact probability,P=0.047).Among the 14 cases of the late onset group,6 cases are females,9 cases showed cognitive impairment and 8 cases presented with mental disorders.There were 6 cases with abnormal brain magnetic resonance imaging(MRI).The cerebrospinal fluid protein of the late onset group[0.37(0.29,0.58)g/L]was higher than that in the early onset group[0.22(0.16,0.30)g/L;Z=-2.667,P=0.008].The modified Rankin Scale(mRS)scores before and after treatment were 3.29+0.83 and 1.50(0.75,2.25),which were higher than those in the early onset group[mRS scores before and after treatment were 2.10+0.99 and 0(0,1.00),t=-3.188,P=0.004;Z=-2.335,P=0.020J.Conclusions There are various symptoms in patients with anti-CASPR2 encephalitis.The early onset patients are common in women,with a higher incidence of epilepsy.The late onset patients are common in males,with prominent manifestations of cognitive impairment and mental disorders,which have a greater impact on daily living abilities.And abnormal MRI findings are common,and the cerebrospinal fluid protein is higher in late onset patients.Anti-CASPR2 antibody may cause more severe immune damage to the nervous system in elderly patients.

EncephalitisAutoantibodiesAge of onsetEpilepsyPeripheral nerves

祝清勇、陈培、梁东晓、张瑞、滕军放

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郑州大学第一附属医院神经内科,郑州 450052

脑炎 自身抗体 发病年龄 癫痫 周围神经

2024

中华神经科杂志
中华医学会

中华神经科杂志

CSTPCD北大核心
影响因子:1.329
ISSN:1006-7876
年,卷(期):2024.57(1)
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