抗二肽基肽酶样蛋白6抗体脑炎临床特征分析

Analysis of clinical data of anti-dipeptidyl peptidase-like protein 6 encephalitis

目的研究抗二肽基肽酶样蛋白6(dipeptidyl-peptidase-like protein 6,DPPX)抗体脑炎的临床症状及免疫治疗的效果。方法回顾性分析2020年1月至2023年3月在我科确诊为抗DPPX抗体脑炎患者的临床症状、实验室检查、影像学特征、治疗和预后。结果共纳入患者9例,年龄中位数(上下四分位数)为45(24,61)岁,其中男5例。神经系统症状中认知功能障碍3例,精神症状(焦虑、易激惹、幻听、行为怪异等)3例,中枢神经系统过度兴奋症状6例,小脑共济失调表现4例,脑干症状5例,睡眠障碍3例,自主神经功能紊乱1例,躯干肢体游走性疼痛1例。另外,有4例患者合并有体重减轻和(或)胃肠道症状。2例患者脑脊液细胞数轻度升高,4例患者脑脊液蛋白升高。4例患者脑电图出现异常,多表现为痫样放电。4例患者脑MRI报告异常,病灶主要位于海马、岛叶,表现为FLAIR高信号。7例患者接受糖皮质激素治疗,6例患者接受血浆置换治疗,7例患者接受长期口服吗替麦考酚酯治疗。7例患者门诊随访,随访时间为4个月至2年不等,所有患者的症状得到不同程度改善,其中3例症状基本完全缓解。结论抗DPPX抗体脑炎的临床症状具有多样性,最常见的症状为胃肠道症状和/或体重减轻、认知功能障碍和中枢神经系统过度兴奋症状,长期口服吗替麦考酚酯可能对改善预后和预防复发有帮助。

Objective We analyzed clinical characteristics of dipeptidyl-peptidase-like protein 6(DPPX)antibody-associated encephalitis and evaluated the effect of immunotherapy on anti-DPPX encephalitis.Methods A total of 9 patients with anti-DPPX encephalitis were retrospectively analyzed,including clinical manifestations,laboratory examinations,imaging features,treatment and prognosis.Results Nine patients were identified(medium age 45 years old,5 were men).Neurologic disorders were multifocal:cognitive dysfunction(3 cases),psychosis(anxiety,irritability,auditory hallucination,abnormal behavior)(3 cases),CNS hyperexcitability(6 cases),brainstem(5 cases),cerebellar dysfunction(4cases),sleep disorders(3 cases),dysautonomia(1 case),allodynia(1 case).In additional,there were 4 patients complicated with weight loss and/or gastrointestinal symptoms.CSF data showed that white cell counts were elevated in 2 patients and protein levels were elevated in 4 patients.EEG revealed epileptiform discharges in 3 patients and delta wave in 1 patient.Abnormalities were found in the brain MRIs of 4 patients including T_(2)/FLAIR lesions in the hippocampus,insula.Seven patients received corticosteroids and six patients received plasma exchange.Seven patients subsequently treated with Mycophenolate Mofetil.Among 7 patients with adequate follow-up data,all patients had recovered to some degree and 3 of them completely remitted after 4 months to 2 years of follow-up.Conclusion The clinical symptoms of anti-DPPX encephalitis were diverse,the classic triad of clinical symptoms were weight loss and/or gastrointestinal disorders,CNS hyperexcitability and cognitive disorders.Long-term oral Mycophenolate Mofetil may be helpful in improving prognosis and reducing recurrence.