Objective：To analyze the clinical characteristics of patients diagnosed with dipeptidyl-peptidase-like protein 6（DPPX） antibody -associated encephalitis. Methods：A retrospective analysis was conducted on five patients with DPPX antibody -associated encephalitis admitted to the Nanjing Brain Hospital Affiliated to Nanjing Medical University between January 2016 and February 2025. Clinical characteristics，electroencephalography（EEG），magnetic resonance imaging（MRI）findings，and outcomes were reviewed. Results：All five patients were male，aged from 14 to 56 years. All patients tested positive for DPPX antibody in serum，with titers ranging from 1∶100 to 1∶10. One patient also tested positive for serum contactin-associated protein-like 2（CASPR2）antibody. Cerebrospinal fluid（CSF）DPPX antibody were negative in four patients，and positive in one patients with the titer 1∶1. Two patients presented with behavioral abnormalities，one with seizures，and one with memory impairment. The patient with concurrent anti-DPPX and anti-CASPR2 antibodies presented with multifocal migratory myoclonus. Brain MRI was normal in four patients，while one patient’s brain MRI indicated an abnormal signal in the bilateral temporal lobe. EEG of the patient with seizure onset showed heavy background abnormalities，accompanied by frequent sharp waves and sharp - slow waves in the left anterior temporal region. Conclusion：DPPX antibody -associated encephalitis is characterized by heterogeneous clinical presentations，which may complicate early diagnosis and differential diagnosis. Although immunotherapy demonstrates therapeutic efficacy，the disease remains susceptible to relapse.