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Peer-reviewedCC BY-NCBMJ neurology open

Clinical features and long-term outcomes of anti-DPPX autoimmune encephalitis: a multicentre retrospective study in a Chinese cohort.

5 affiliations
  1. 1Department of Neurology, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China.
  2. 2School of Medicine, Shanghai Jiao Tong University, Shanghai, China.
  3. 3Department of Neurology, General Hospital of Ningxia Medical University, Yinchuan, China.
  4. 4Department of Neurology, Guangdong Sanjiu Brain Hospital, Guangzhou, China.
  5. 5Department of Pediatric Pulmonology, Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine, Shanghai, China.

Abstract

Objective

To explore the clinical characteristics and long-term prognostic factors of anti-Dipeptidyl-peptidase-like protein 6 (DPPX) antibody encephalitis in Chinese patients.Method

This study retrospectively included anti-DPPX antibody encephalitis patients diagnosed at three medical centres from January 2019 to May 2024. modified Rankin Scale (mRS) and Clinical Assessment Scale in Autoimmune Encephalitis (CASE) were used to evaluate baseline, short-term (3 months) and long-term (12 months) outcomes. Furthermore, we analysed the relationship between clinical characteristics, immune markers and prognosis.Results

15 patients were included (median age 45, 60% female). Predominant symptoms were cognitive impairment (66.7%, 10/15), seizures (53.3%, 8/15) and psychiatric symptoms (53.3%, 8/15), with a low incidence of preceding weight loss (6.7%, 1/15); 60.0% (9/15) of patients had abnormal brain MRI findings. Both mRS and CASE scores significantly improved at 3 and 12 months of follow-up (all p<0.05), but 46.2% (5/11) of patients still had memory impairment after 12 months of follow-up. Short-term disease severity (3M-CASE) was significantly positively correlated with hospital stay (β=0.22, 95% CI 0.02 to 0.42, p=0.03), psychiatric disorders (β=2.71, 95% CI 0.26 to 5.17, p=0.03), intensive care unit admission (β=4.11, 95% CI 2.07 to 6.15, p=0.001) and MRI abnormalities (β=3.13, 95% CI 0.73 to 5.52, p=0.02). Long-term disease severity (12M-CASE) was significantly correlated with sleep disorders (β=2.72, 95% CI 0.36 to 5.08, p=0.03). No specific immunological biomarkers remained significantly correlated with CASE scores after adjustment for multiple comparisons. No clinical relapse was observed in the 11 patients with available long-term follow-up data.Conclusion

This study delineates the clinical phenotype of anti-DPPX encephalitis in China. Immunotherapy was significantly associated with improvements in short-term and long-term outcomes. Prognostic factors and relevant immune biomarkers differ between disease stages, underscoring the need for long-term management and stage-specific immunointervention.

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BibTeX
@article{chen2026clinical,
  title = {{Clinical features and long-term outcomes of anti-DPPX autoimmune encephalitis: a multicentre retrospective study in a Chinese cohort.}},
  author = {Fuping Chen and Wendi Yu and Jing Peng and Tao Xue and Tingting Ma and Hua Li and Chong Xie and Haipei Liu and Kan Wang and Yong Hao},
  journal = {BMJ neurology open},
  year = {2026},
  month = oct,
  doi = {10.1136/bmjno-2026-001645},
  url = {https://europepmc.org/article/MED/42968772},
  note = {CC BY-NC},
}
RIS
TY  - JOUR
TI  - Clinical features and long-term outcomes of anti-DPPX autoimmune encephalitis: a multicentre retrospective study in a Chinese cohort.
AU  - Fuping Chen
AU  - Wendi Yu
AU  - Jing Peng
AU  - Tao Xue
AU  - Tingting Ma
AU  - Hua Li
AU  - Chong Xie
AU  - Haipei Liu
AU  - Kan Wang
AU  - Yong Hao
PY  - 2026
DA  - 2026/10/29
JO  - BMJ neurology open
DO  - 10.1136/bmjno-2026-001645
UR  - https://europepmc.org/article/MED/42968772
AB  - Objective  To explore the clinical characteristics and long-term prognostic factors of anti-Dipeptidyl-peptidase-like protein 6 (DPPX) antibody encephalitis in Chinese patients.Method  This study retrospectively included anti-DPPX antibody encephalitis patients diagnosed at three medical centres from January 2019 to May 2024. modified Rankin Scale (mRS) and Clinical Assessment Scale in Autoimmune Encephalitis (CASE) were used to evaluate baseline, short-term (3 months) and long-term (12 months) outcomes. Furthermore, we analysed the relationship between clinical characteristics, immune markers and prognosis.Results  15 patients were included (median age 45, 60% female). Predominant symptoms were cognitive impairment (66.7%, 10/15), seizures (53.3%, 8/15) and psychiatric symptoms (53.3%, 8/15), with a low incidence of preceding weight loss (6.7%, 1/15); 60.0% (9/15) of patients had abnormal brain MRI findings. Both mRS and CASE scores significantly improved at 3 and 12 months of follow-up (all p<0.05), but 46.2% (5/11) of patients still had memory impairment after 12 months of follow-up. Short-term disease severity (3M-CASE) was significantly positively correlated with hospital stay (β=0.22, 95% CI 0.02 to 0.42, p=0.03), psychiatric disorders (β=2.71, 95% CI 0.26 to 5.17, p=0.03), intensive care unit admission (β=4.11, 95% CI 2.07 to 6.15, p=0.001) and MRI abnormalities (β=3.13, 95% CI 0.73 to 5.52, p=0.02). Long-term disease severity (12M-CASE) was significantly correlated with sleep disorders (β=2.72, 95% CI 0.36 to 5.08, p=0.03). No specific immunological biomarkers remained significantly correlated with CASE scores after adjustment for multiple comparisons. No clinical relapse was observed in the 11 patients with available long-term follow-up data.Conclusion  This study delineates the clinical phenotype of anti-DPPX encephalitis in China. Immunotherapy was significantly associated with improvements in short-term and long-term outcomes. Prognostic factors and relevant immune biomarkers differ between disease stages, underscoring the need for long-term management and stage-specific immunointervention.
SN  - 2632-6140
ER  -