抗二肽基肽酶样蛋白 6 脑炎伴单纯小脑性共济失调：病例报告。
Anti-dipeptidyl-peptidase-like protein 6 encephalitis with pure cerebellar ataxia: a case report.
机构信息
Department of Neurology, Dong'hu District, The First Affiliated Hospital of Nanchang University, No.17, Yongwaizheng Road, Nanchang, 330000, China.
出版信息
BMC Neurol. 2022 Jul 1;22(1):242. doi: 10.1186/s12883-022-02769-0.
BACKGROUND
Anti-dipeptidyl-peptidase-like protein 6 (DPPX) encephalitis is a rare autoimmune encephalitis. The clinical symptoms of anti-DPPX encephalitis are often severe, manifested as diarrhea/weight loss, central nervous system hyperexcitability and cognitive dysfunction.
CASE PRESENTATION
An 18-year-old boy was admitted for 1-week-long cerebellar symptoms including dizziness, unsteady gait and frequent vomiting. Magnetic resonance imaging (MRI) displayed no abnormal findings. However, autoimmune encephalitis panel revealed anti-DPPX antibody was positive in the serum. This patient completely recovered after immunoglobulin and corticoids therapy. In addition, repeat serum antibody test for DPPX was negative within one month.
CONCLUSION
In addition to the classic triad, anti-DPPX encephalitis may manifest as mild and rare symptoms due to lower antibody titers. Fast identification of rare symptoms can help to quickly diagnosis and effective treatment.
背景
抗二肽基肽酶样蛋白 6（DPPX）脑炎是一种罕见的自身免疫性脑炎。抗 DPPX 脑炎的临床症状通常较为严重，表现为腹泻/体重减轻、中枢神经系统兴奋和认知功能障碍。
病例介绍
一名 18 岁男性，因小脑症状（包括头晕、步态不稳和频繁呕吐）入院，病程 1 周。磁共振成像（MRI）未见异常。然而，自身免疫性脑炎抗体谱检测显示血清中存在抗 DPPX 抗体。该患者经免疫球蛋白和皮质激素治疗后完全康复。此外，一个月内重复检测血清 DPPX 抗体为阴性。
结论
除了经典三联征外，由于抗体滴度较低，抗 DPPX 脑炎也可能表现为轻度和罕见的症状。快速识别罕见症状有助于快速诊断和有效治疗。
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