Severe Abdominal Pain as a Prominent Clinical Manifestation of Anti-DPPX Autoimmune Encephalitis: A Case Report and Systematic Review.

BACKGROUND

Anti-dipeptidyl-peptidase-like protein 6 encephalitis (DPPXE) is an exceptionally rare form of autoimmune encephalitis characterized by a highly heterogeneous clinical phenotype.

METHODS

In this study, we report a Chinese patient presenting with severe abdominal pain as a prominent symptom; furthermore, we conducted a systematic review and analysis of 125 cases of DPPXE (including the present case).

RESULTS

This rare case presented with severe abdominal pain as a prominent symptom. We comprehensively summarized the clinical presentations, patterns of recurrence, and therapeutic responses of DPPXE. Notably, by comparing the clinical characteristics of DPPXE cases reported in China with those from other regions, we found some differences in disease presentation, suggesting potential racial or geographic variability.

CONCLUSION

Our findings may provide up-to-date and comprehensive insights into the pathophysiology and clinical course of this rare disorder from multiple perspectives.

© 2026 The Author(s). Immunity, Inflammation and Disease published by John Wiley & Sons Ltd.