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接触蛋白相关蛋白2抗体阳性自身免疫性脑炎的临床特点分析

Clinical characteristics of contactin-associated protenin-like 2 antibody positive autoimmune encephalitis
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摘要 目的探讨接触蛋白相关蛋白2(Caspr2)抗体阳性自身免疫性脑炎的临床表现、影像学改变、实验室检查、治疗方案及预后。方法报道郑州大学第一附属医院2017年6月神经内科收治的1例Caspr2抗体阳性自身免疫性脑炎患者,同时对文献中报道的9例Caspr2抗体阳性自身免疫性脑炎病例进行总结。结果10例Caspr2抗体阳性自身免疫性脑炎患者中,男6例,女4例,中位发病年龄53岁(22~75岁)。10例患者中,8例血清Caspr2抗体阳性[其中2例同时脑脊液Caspr2抗体阳性,3例同时血清富亮氨酸胶质瘤失活蛋白1(LGI1)抗体阳性],2例仅脑脊液Caspr2抗体阳性。7例头颅MRI异常,主要表现为颞叶及海马T2相高信号。10例患者均亚急性起病,临床表现包括认知障碍、精神行为异常、癫痫、睡眠障碍、自主神经症状、疼痛等,且均叠加有一种以上症状。8例行激素和(或)丙种球蛋白治疗后症状好转,2例对症治疗后好转。结论Caspr2抗体阳性自身免疫性脑炎患者临床表现多样,可累及中枢和周围神经肌肉系统及自主神经系统等,免疫抑制治疗有效。 Objective To investigate the clinical manifestation,imaging changes,laboratory examination,treatment plan and prognosis of Caspr2 antibody positive autoimmune encephalitis.Methods This paper reported 1 case of Caspr2 antibody positive autoimmune encephalitis admitted to Neurology Department in the First Affiliated Hospital of Zhengzhou University in June 2017,and summarized 9 cases of Caspr2 antibody positive autoimmune encephalitis reported in the literature.Results There were 6 males and 4 females in 10 patients with autoimmune encephalitis with positive Caspr2 antibody.The median age of onset was 53 years(22-75 years).Among the 10 patients,8 cases were positive for Caspr2 antibody(2 cases also positive for Caspr2 antibody in cerebrospinal fluid,3 cases also positive for LGI1 antibody)and 2 cases were only positive for Caspr2 antibody in cerebrospinal fluid.Seven cases of abnormal cranial MRI manifested mainly temporal lobe and hippocampal T2 phase hyperintensity.All the 10 patients had subacute onset,of them the clinical manifestations included cognitive impairment,psychobehavioral abnormality,epilepsy,sleep disorder,autonomic neurological symptoms,pain and so on.All of the 10 patients had more than one symptom superimposed.Symptoms were improved in 8 cases after hormone and/or gamma globulin treatment,and 2 cases were on the mend after symptomatic treatment.Conclusions Caspr2 antibody positive autoimmune encephalitis patients have a variety of clinical manifestations,and this sisease can implicate the central and peripheral neuromuscular system and the independent nervous system.Immunosuppressive therapy is effective on it.
作者 闫现芳 刘宇 李伟新 郭晓玥 Yan Xianfang;Liu Yu;Li Weixin;Guo Xiaoyue(Department of Neurology,the First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China)
出处 《中国实用医刊》 2019年第4期14-18,共5页 Chinese Journal of Practical Medicine
关键词 接触蛋白相关蛋白-2 自身免疫性脑炎 免疫抑制治疗 Contactin-associated protein-like 2 Autoimmune encephalitis Immunotherapy
作者简介 通信作者:刘宇,Email:martinsums@126.com.
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