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ORIGINAL RESEARCH article

Front. Neurosci.
Sec. Translational Neuroscience
Volume 18 - 2024 | doi: 10.3389/fnins.2024.1379933
This article is part of the Research Topic Neurological Autoimmunity: etiology, infectious complications, treatment strategies and outcomes in antibody-associated syndromes and beyond View all 11 articles

Expanding the clinical spectrum of anti-DPPX encephalitis: a multicenter retrospective study

Provisionally accepted
Yining Gao Yining Gao 1Zhang Yizongheng Zhang Yizongheng 1Hangxing Chunyu Hangxing Chunyu 1Yongfeng Xu Yongfeng Xu 2Ying Wang Ying Wang 3Su Zhi Liu Su Zhi Liu 3Jie Chang Jie Chang 4Bo Tang Bo Tang 5Congying Xu Congying Xu 6Yi Lu Yi Lu 7Jian Zhou Jian Zhou 8Xiangyong Kong Xiangyong Kong 9Xiaoying Zhu Xiaoying Zhu 10Qinming Zhou Qinming Zhou 1*Huanyu Meng Huanyu Meng 1*
  • 1 Ruijin Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China
  • 2 Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, Zhejiang Province, China
  • 3 Zhejiang Taizhou Hospital, Taizhou, Zhejiang Province, China
  • 4 Huadong Hospital, Fudan University, Shanghai, Shanghai Municipality, China
  • 5 Hangzhou First People's Hospital, Hangzhou, Zhejiang Province, China
  • 6 Second Hospital of Jiaxing City, Jiaxing, Zhejiang Province, China
  • 7 The First Affiliated Hospital of Bengbu Medical College, Handan, Anhui Province, China
  • 8 Yongkang First People's Hospital, Yongkang, China
  • 9 Yongkang Hospital of Traditional Chinese Medicine, Jinhua, Zhejiang Province, China
  • 10 Shanghai First People's Hospital, Shanghai, Shanghai Municipality, China

The final, formatted version of the article will be published soon.

    Objective: Anti-dipeptidyl-peptidase-like protein-6 (DPPX) encephalitis is a rare autoimmune encephalitis, and clinical and experimental information regarding this disease is limited. We conducted this study to comprehensively describe the clinical characteristics, ancillary test results, neuroimaging results, and treatment response in a group of Chinese patients with anti-DPPX encephalitis for better understanding this disease.We recruited 14 patients who tested positive for anti-DPPX antibodies in the serum and/or cerebrospinal fluid from 11 medical centers between March 2021 and June 2023. This retrospective study evaluated data on symptoms, autoantibody test, auxiliary examinations, treatments, and outcomes.The average age at diagnosis was 45.93±4.62 years (range: 11-72 years), and 9 of the 14 patients were males. The main symptoms included cognitive impairment (50.0%, 7/14), central nervous system hyperexcitability (42.9%, 6/14), gastrointestinal dysfunction (35.7%, 5/14), and psychiatric disorders (35.7%, 5/14). Notably, we discovered specific findings on 18 Ffluorodeoxyglucose positron-emission tomography (PET)/magnetic resonance imaging in two patients. Co-existing autoantibodies were identified in two patients. Parainfection was identified in four patients. One patient had other autoimmune diseases, and one had tumor. Eleven patients received immunotherapy and most patients improved at discharge. Surprisingly, three male patients but no female patients relapsed during the 6 months of follow-up.The development and outcome of anti-DPPX encephalitis are variable. Male patients were predominant in our cohort. The most common symptoms were the classical triad of prodromal gastrointestinal dysfunction, cognitive and mental disorders, and central nervous system hyperexcitability. Infections, immune dysregulation, and tumors may be important etiologies. Longterm monitoring of disease development should be done in male patients. Overall, our results highlight novel clinical characteristics of anti-DPPX encephalitis.

    Keywords: anti-DPPX encephalitis, Anti-DPPX antibody, multicenter, clinical characteristic, Immunotherapy

    Received: 31 Jan 2024; Accepted: 15 Apr 2024.

    Copyright: © 2024 Gao, Yizongheng, Chunyu, Xu, Wang, Liu, Chang, Tang, Xu, Lu, Zhou, Kong, Zhu, Zhou and Meng. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

    * Correspondence:
    Qinming Zhou, Ruijin Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China
    Huanyu Meng, Ruijin Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China

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