Long term outcomes in patients with anti-DPPX autoimmunity

Abstract

DDPX antibody-associated encephalitis is characterized by cognitive dysfunction, neuropsychiatric symptoms, and CNS hyperexcitability, preceded by prodromal weight loss and diarrhea. Data regarding long-term outcomes is scarce. We retrospectively identified six anti-DPPX encephalitis patients across all three Mayo Clinic sites with inclusion criteria: 1) positive DPPX cell-based assay and mouse tissue-based immunofluorescence samples in both serum and CSF; 2) duration of follow up of at least 36 months from symptom onset to last follow up. Only one patient had a paraneoplastic process in the setting of chronic lymphocytic leukemia. At last follow up, all patients had resolution of GI symptoms. Residual cognitive impairment was seen in 4/6 (67%). Clinical stability was reached in 3/6 (50%) while on immunotherapy. Immunotherapy was discontinued in 2/6 (33%) and they remained stable without relapse at last follow up. One patient died of unclear etiology. Overall long-term outcomes are good in anti-DPPX encephalitis. Symptoms can improve on immunotherapy, but full resolution and return to premorbid baseline is unlikely.