Analysis on clinical characteristics of anti-DPPX encephalitis

doi:10.3969/j.issn.1672-6731.2020.10.004

Abstract

Abstract:

Objective To summarize the clinical features of anti-dipeptidyl-peptidase-like protein-6 (DPPX) encephalitis. Methods and Results We presented a 36-year-old male patient with anti-DPPX encephilitis who developed with myoclonus, progressive memory loss, dyspnea and diarrhea. Antibody against DPPX was positive in his serum. ¹⁸F-fluoro-2-deoxy-D-glucose (¹⁸F-FDG) PET imaging indicated the hypometabolism in the bilateral temporal lobes. Then we reviewed the literatures about anti-DPPX encephalitis. A total of 7 articles with 36 cases were enrolled. We found that anti-DPPX encephalitis was common in male middle-aged patients with a chronic disease course. The clinical features of anti-DPPX encephalitis were myoclonus, tremor, cognitive dysfunction, automatic dysfunction, injury of brain stem or cerebellum and body weight loss. A small group of patients had underlying B cell neoplasms. DPPX antibody could be detected in the cerebrospinal fluid and serum of anti-DPPX encephalitis patients. The neuro-imaging often showed non-specific changes. The immunotherapy was effective in treating anti-DPPX encephalitis. It had a good prognosis. Conclusions Anti-DPPX encephalitis is rare, and it has complicated clinical manifestations. There are difficulties in the early and differential diagnosis. But it is usually responsive to the immunotherapy.

Key words: Encephalitis, Autoimmune diseases, Potassium channels,voltage-gated, Serology, Cerebrospinal fluid, Positron-emission tomography

摘要：

目的总结抗二肽基肽酶样蛋白-6（DPPX）抗体脑炎之临床特征。方法与结果 36岁男性抗DPPX抗体脑炎患者临床表现为肌阵挛、进行性记忆力减退、呼吸困难和腹泻，实验室检查血清DPPX抗体阳性，¹⁸F-FDG PET提示双侧颞叶代谢减低。为进一步了解疾病特征，文献检索获得7篇国外文献计36例病例，经分析显示抗DPPX抗体脑炎以男性、中年好发，慢性病程；肌阵挛、震颤、认知功能障碍、自主神经功能失调、小脑或脑干损害及体重下降等为其主要症状与体征，可合并B细胞淋巴瘤；脑脊液或血清DPPX抗体呈阳性；无特异性影像学表现；治疗原则以免疫调节治疗为主，预后良好。结论抗DPPX抗体脑炎临床罕见，症状复杂多样，早期诊断与鉴别诊断困难，早期予免疫调节治疗预后良好。

关键词: 脑炎, 自身免疫疾病, 钾通道, 电压门控, 血清学, 脑脊髓液, 正电子发射断层显像术

ZHOU Qin-ming, CAI Yong, NI You, MENG Huan-yu, SHI Hong-qin, CHEN Sheng-di, CHEN Sheng. Analysis on clinical characteristics of anti-DPPX encephalitis[J]. Chinese Journal of Contemporary Neurology and Neurosurgery, 2020, 20(10): 862-867.

周勤明, 蔡勇, 倪优, 孟环宇, 石红琴, 陈生弟, 陈晟. 抗二肽基肽酶样蛋白-6抗体脑炎[J]. 中国现代神经疾病杂志, 2020, 20(10): 862-867.

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URL: https://journal11.magtechjournal.com/cjcnn/EN/10.3969/j.issn.1672-6731.2020.10.004

https://journal11.magtechjournal.com/cjcnn/EN/Y2020/V20/I10/862

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