回顾性分析1例以抽动表现起病，后出现明显的精神行为异常、尿频的抗二肽基肽酶样蛋白-6（DPPX）相关自身免疫性脑炎患儿的病历资料，并复习既往发表的抗DPPX相关自身免疫性脑炎的相关文献。探讨以抽动障碍为首发症状的抗DPPX相关性自身免疫性脑炎的临床特点及预后，提高儿科医师对该疾病的认识。患儿女，7岁，因明显的抽动表现入院，逐渐出现精神行为异常及尿频表现。头颅磁共振平扫基本正常；脑电图提示背景节律慢化；血清和脑脊液中抗DPPX抗体滴度升高（血清滴度1∶32，脑脊液滴度1∶1），肿瘤相关筛查未见异常。经免疫治疗后症状明显缓解，随访15个月未复发。检索并总结共37篇文献，总计报道了88例抗DPPX相关自身免疫性脑炎，以抽动障碍为首发症状的尚未报道。儿童抗DPPX相关性自身免疫性脑炎以抽动障碍为首发症状罕见，本例免疫治疗后效果好。临床医师须意识到该疾病的异质性，谨防漏诊。

A retrospective analysis was performed on the clinical data of a 7-year-old girl with anti-DPPX antibody-associated autoimmune encephalitis who initially presented with tic-like symptoms and subsequently developed significant behavioral abnormalities and frequent urination. This case was reviewed in conjunction with previously published literature on anti-DPPX-related autoimmune encephalitis to investigate the clinical features and prognosis of this condition when tic disorders are the initial symptom, hereby enhancing pediatricians' awareness of this rare presentation. The patient was admitted due to prominent tic-like symptoms and progressively exhibited behavioral abnormalities and frequent urination. Brain MRI showed no significant abnormalities, while EEG demonstrated a slowed background rhythm. Elevated titers of anti-DPPX antibodies were detected in both serum (1∶32) and cerebrospinal fluid (1∶1), and tumor-related screening revealed no abnormalities. Following immunotherapy, her symptoms significantly improved, and no recurrence was observed during the 15-month follow-up period. A total of 37 articles were reviewed, summarizing 88 cases of anti-DPPX-related autoimmune encephalitis. However, no cases presenting with tic disorders as the initial symptom have been reported. Anti-DPPX antibody-associated autoimmune encephalitis presenting with tic disorders as the initial symptom is exceedingly rare in children. In this case, immunotherapy yielded favorable results. Clinicists should be vigilant regarding the heterogeneity of this disease to avoid missed or delayed diagnoses.