儿童抗CV2自身免疫性脑炎1例报告并文献复习

Anti-CV2 autoimmune encephalitis in children: a case report and literature review

目的 探讨儿童抗CV2自身免疫性脑炎(AE)患者的临床表现、实验室检查、影像学特点、治疗及预后。方法 回顾性分析2020年9月在贵阳市妇幼保健院儿童神经内科确诊的1例抗CV2 AE患儿的病例资料,并复习既往发表的抗CV2 AE的相关文献。结果 本例患儿主要表现为精神行为异常、意识障碍、语言障碍、癫痫发作及舞蹈样运动等,头颅MRI示双侧丘脑及脑干异常信号,脑脊液(CSF)和血清抗CV2抗体阳性,未合并肿瘤,免疫治疗效果良好。既往文献共报道57例抗CV2 AE,均为成人患者,主要表现为精神行为异常、癫痫发作、睡眠障碍、记忆缺失、舞蹈样运动及帕金森样症状等;头颅MRI多表现为边缘叶及纹状体如壳核、尾状核异常,常合并肿瘤,免疫治疗效果差。结论 儿童抗CV2 AE鲜见,本例为国内外首例,与成人病例症状类似,但头颅MRI以双侧丘脑及脑干异常信号为特点,未合并肿瘤,免疫治疗效果较好。

Objective To investigate the clinical manifestations, laboratory examination, imaging characteristics, treatment and prognosis of anti-CV2 autoimmune encephalitis(anti-CV2 AE) in children. Methods The data of a child with anti-CV2 AE diagnosed in the Department of Pediatric Neurology, Guiyang Maternal and Child Health Care Hospital in September 2020 was retrospective analyzed. The previous literatures on anti-CV2 AE were reviewed. Results The main manifestations of this child were abnormal mental behavior, disturbance of consciousness, language disorder, epileptic seizure, dance-like movements, etc. Cranial MRI showed abnormal signals in bilateral thalamus and brain stem. Anti-CV2 antibody was positive in cerebrospinal fluid and serum. No tumor was found. Immunotherapy effect was good. There were 57 patients reported in the previous literatures. All of them were adult patients, mainly manifested as psychobehavioral abnormalities, seizures, sleep disorders, memory loss, dance-like movements and Parkinson like symptoms.Cranial MRIs usually exhibit abnormalities in marginal lobe and striatum, such as putamen and caudate nucleus, often associated with tumors. Additionally, the effect of immunotherapy is poor. Conclusion Anti-CV2 AE is rare in children. This case is the first one at home and abroad, and its symptoms are similar to those of adult patients. However, cranial MRI of this child is characterized by abnormal signals in bilateral thalamus and brainstem, and there is no tumor. Immunotherapy is effective.