Analysis on clinical characteristics of anti⁃DPPX encephalitis

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.

DOI:10.3969/j.issn.1672⁃6731.2020.10.004