以抽动障碍为首发症状的抗DPPX抗体相关性自身免疫性脑炎1例报告

doi:10.12372/jcp.2025.24e0231

Abstract

Abstract:

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.

Key words: tic disorders, DPPX antibody, abnormal mental behavior, frequent urination, autoimmune encephalitis

CLC Number:

GUAN Qiuyue, XIE Yi, CHEN Liqing, LIU Yan. Anti-DPPX antibody-associated autoimmune encephalitis with tic disorder as the first symptom: a case report[J].Journal of Clinical Pediatrics, 2025, 43(8): 621-627.

Figures/Tables 4

Figure 1

Figure 2

Figure 3

Table 1

Baseline characteristics, treatment, and outcomes of 7 anti-DPPX antibody encephalitis patients (age≤18 years)"

患者	性别	发病年龄/岁	主要临床表现	头颅影像	EEG	抗体	治疗及预后
1^[24]	男	15	认知障碍、共济失调、肌强直、眼震颤、过度惊跳、反射亢进	头颅MRI、FDG-PET显像无异常	无异常	脑脊液抗DPPX抗体1∶320；血清抗DPPX抗体1∶10000	甲基泼尼松龙、免疫球蛋白、血浆置换未改善病情或短暂减轻症状，症状复发后42个月开始使用利妥昔单抗，效果良好
2^[6]	女	13	恶心等消化道症状、发作性共济失调、复视、眼肌麻痹、睡眠障碍	未报道	未报道	血清抗DPPX抗体阳性，滴度未报道	甲基泼尼松龙、血浆置换、利妥昔单抗治疗后症状部分缓解，3周后有复发
3^[6]	男	18	呕吐、腹泻、体重减轻等消化道症状、认知障碍、头晕、肌阵挛、眼震颤、复视、尿潴留、癫痫发作	未报道	未报道	血清抗DPPX抗体阳性,滴度未报道	甲基泼尼松龙、硫唑嘌呤，治疗后症状完全改善，随访4年未复发
4^[21]	男	14	认知障碍、精神障碍	头颅MRI无异常	无异常	脑脊液抗DPPX抗体阴性；血清抗DPPX抗体1∶100，抗TPO>600 IU/ml,抗TG 345.03 IU/ml	甲基泼尼松龙、免疫球蛋白治疗后，随访26个月未复发，改良 Rankin 量表评分有改善
5^[21]	男	17	头痛、迁移性肌阵挛	头颅MRI无异常	无异常	脑脊液抗DPPX抗体阴性；血清抗DPPX抗体 1∶100，抗CASPR2 1∶10	甲基泼尼松龙、免疫球蛋白、氯硝西泮和丙戊酸治疗后，患者仍有肌阵挛，进行第二轮免疫球蛋白治疗后，随访2个月未复发，改良 Rankin 量表评分有改善
6^[9]	男	14	便秘、胃轻瘫等消化道症状、认知障碍、反射亢进、肢体麻痹、呼吸衰竭、发热	头颅MRI：脑干、基底神经节和双侧白质中出现广泛的异常信号	未报道	脑脊液抗DPPX抗体阴性；血清抗DPPX抗体1∶320	甲基泼尼松龙、免疫球蛋白治疗后症状改善不完全
7^[27]	男	18	呕吐等消化道症状、头晕、共济失调、眼震颤、发热	头颅MRI无异常	无异常	脑脊液抗DPPX抗体阴性；血清抗DPPX抗体1∶10	甲基泼尼松龙、免疫球蛋白治疗后症状完全改善，1个月内复查血清和脑脊液抗DPPX抗体均为阴性，随访10个月未复发
本例	女	7	头痛、发热、精神行为异常、性格改变、尿频、抽动障碍	头颅MRI：右侧海马较对侧小，右侧侧脑室颞角较对侧大	背景节律慢化，清醒安静闭目状态下双侧枕区见7~ 8 Hz左右θ、α波节律	脑脊液抗DPPX抗体滴度1∶1；血清抗DPPX抗体滴度1∶32	甲基泼尼松龙、免疫球蛋白，治疗后症状完全改善，随访15个月未复发，病程1年复查血清抗DPPX抗体滴度 1:32，病程15个月复查血清抗DPPX抗体滴度1:10

Table 1

References 27

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Anti-DPPX antibody-associated autoimmune encephalitis with tic disorder as the first symptom: a case report

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