Objective We analyzed clinical characteristics of dipeptidyl-peptidase-like protein 6(DPPX)antibody-associated encephalitis and evaluated the effect of immunotherapy on anti-DPPX encephalitis.Methods A total of 9 patients with anti-DPPX encephalitis were retrospectively analyzed,including clinical manifestations,laboratory examinations,imaging features,treatment and prognosis.Results Nine patients were identified(medium age 45 years old,5 were men).Neurologic disorders were multifocal:cognitive dysfunction(3 cases),psychosis(anxiety,irritability,auditory hallucination,abnormal behavior)(3 cases),CNS hyperexcitability(6 cases),brainstem(5 cases),cerebellar dysfunction(4 cases),sleep disorders(3 cases),dysautonomia(1 case),allodynia(1 case).In additional,there were 4 patients complicated with weight loss and/or gastrointestinal symptoms.CSF data showed that white cell counts were elevated in 2 patients and protein levels were elevated in 4 patients.EEG revealed epileptiform discharges in 3 patients and delta wave in 1 patient.Abnormalities were found in the brain MRIs of 4 patients including T2/FLAIR lesions in the hippocampus,insula.Seven patients received corticosteroids and six patients received plasma exchange.Seven patients subsequently treated with Mycophenolate Mofetil.Among 7 patients with adequate follow-up data,all patients had recovered to some degree and 3 of them completely remitted after 4 months to 2 years of follow-up.Conclusion The clinical symptoms of anti-DPPX encephalitis were diverse,the classic triad of clinical symptoms were weight loss and/or gastrointestinal disorders,CNS hyperexcitability and cognitive disorders.Long-term oral Mycophenolate Mofetil may be helpful in improving prognosis and reducing recurrence.

Acute Disease ; Allografts ; Humans ; Kidney ; Kidney Transplantation ; Nephritis ; Pyelonephritis ; Transplantation, Homologous