The effect of double filtration plasmapheresis and corticosteroids on patients with anti-dipeptidyl-peptidase-like protein 6 encephalitis.

IF 1.5 4区医学 Q3 HEMATOLOGY

Therapeutic Apheresis and Dialysis Pub Date : 2024-02-01 Epub Date: 2023-07-17 DOI:10.1111/1744-9987.14039

Wenbin Wan, Yuanmei Pan, Ying Chen, Shuwei Bai, Xiaoying Yao, Yan Lin, Jun Wu, Liping Ni, Yufang Mei, Huiying Qiu, Yan Zhou, Yong Hao, Yangtai Guan

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引用次数: 0

Abstract

Introduction: Anti-dipeptidyl-peptidase-like protein 6 (DPPX) encephalitis is a rare condition with varied symptoms including gastrointestinal issues, weight loss, cognitive and mental dysfunction, and hyperexcitability of the central nervous system.

Methods: We studied five patients with anti-DPPX encephalitis who received immunotherapy, specifically DFPP, at our hospital. We analyzed their clinical symptoms, lab results, electrophysiological and imaging findings, and outcomes with immunotherapy.

Results: Patients presented with cognitive dysfunction, tremor, seizures, psychiatric disturbances, and cerebellar and brainstem dysfunction. Magnetic resonance imaging (MRI) showed brain abnormalities in one patient and elevated cerebrospinal fluid (CSF) protein levels in two patients. Antibodies against DPPX were detected in all patients and in CSF in two patients. One patient had antibodies against anti-CV2/contactin response mediator protein 5 (CRMP5). All patients responded well to DFPP and corticosteroids.

Conclusion: DFPP may be an effective treatment for anti-DPPX encephalitis. Further research is needed to understand disease progression and evaluate immunotherapy efficacy.

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抗二肽基肽酶样蛋白 6 脑炎患者接受双滤过性浆细胞清除术和皮质类固醇的效果。

导言：抗二肽基肽酶样蛋白 6（DPPX）脑炎是一种罕见病，症状多样，包括胃肠道问题、体重减轻、认知和精神功能障碍以及中枢神经系统过度兴奋：我们研究了在本医院接受免疫疗法（特别是 DFPP）的五名抗DPPX脑炎患者。我们分析了他们的临床症状、实验室结果、电生理和影像学检查结果以及免疫治疗的效果：结果：患者出现认知功能障碍、震颤、癫痫发作、精神障碍以及小脑和脑干功能障碍。磁共振成像（MRI）显示一名患者脑部异常，两名患者脑脊液（CSF）蛋白水平升高。所有患者均检测到 DPPX 抗体，两名患者的脑脊液中检测到 DPPX 抗体。一名患者体内有抗CV2/接触素反应介质蛋白5（CRMP5）抗体。所有患者对 DFPP 和皮质类固醇反应良好：结论：DFPP可能是治疗抗DPPX脑炎的有效方法。结论：DFPP可能是治疗抗DPPX脑炎的有效方法，但还需要进一步研究，以了解疾病进展情况并评估免疫疗法的疗效。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Therapeutic Apheresis and Dialysis 医学-泌尿学与肾脏学

CiteScore

3.00

自引率

10.50%

发文量

166

审稿时长

6-12 weeks

期刊介绍： Therapeutic Apheresis and Dialysis is the official peer-reviewed journal of the International Society for Apheresis, the Japanese Society for Apheresis and the Japanese Society for Dialysis Therapy. The Journal publishes original articles, editorial comments, review articles, case reports, meeting abstracts and Communications information on apheresis and dialysis technologies and treatments.