Clinical spectrum and long-term outcomes of antibody-negative severe autoimmune encephalitis: a retrospective study
ObjectiveThe aims of the study were to characterize the clinical manifestations and outcomes of patients with antibody-negative severe autoimmune encephalitis (AE).MethodsThis retrospective, monocentric study recruited patients from the Neurology Department of Henan Provincial People’s Hospital betw...
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Frontiers Media S.A.
2025-05-01
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| Series: | Frontiers in Immunology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fimmu.2025.1591771/full |
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| author | Fangfang Li Yu He Yu He Xiaoqian Chen Ali Yang Jiewen Zhang Weizhou Zang |
| author_facet | Fangfang Li Yu He Yu He Xiaoqian Chen Ali Yang Jiewen Zhang Weizhou Zang |
| author_sort | Fangfang Li |
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| description | ObjectiveThe aims of the study were to characterize the clinical manifestations and outcomes of patients with antibody-negative severe autoimmune encephalitis (AE).MethodsThis retrospective, monocentric study recruited patients from the Neurology Department of Henan Provincial People’s Hospital between April 2017 and December 2023. All patients underwent neural antibody testing in both blood and cerebrospinal fluid (CSF) and met the diagnostic criteria for autoantibody-negative but probable severe AE, with available 1-year follow-up data.ResultsIn total, 124 patients with autoantibody-negative severe AE were analyzed. Among them, 27.4% achieved good functional outcomes at discharge. Older age (OR 1.034, 95% confidence interval [CI] 1.010-1.058, p = 0.004) and the presence of dyskinesia/dystonia (OR 8.463, 95% CI 3.282-21.820, p < 0.001) were predictive of poor short-term outcomes. At the 1-year follow-up, 54.8% experienced favorable long-term outcomes. Independent predictors of unfavorable long-term outcomes included older age (OR 1.076, 95% CI 1.018-1.136, p = 0.009), longer hospital stays (OR 1.264, 95% CI 1.105-1.446, p = 0.001), the presence of refractory status epilepticus (OR 14.765, 95% CI 1.759-123.935, p = 0.013) and higher CASE scores at discharge (OR 2.079, 95% CI 1.450-2.980, p < 0.001). Additionally, 30.6% of patients had relapsed, with refractory status epilepticus being an independent risk factor for relapse.ConclusionAlthough patients with antibody-negative severe AE experience significant disability in the early stages of their disease, the majority eventually regain independent functioning. Older age at disease onset, longer hospital stays, the presence of refractory status epilepticus and higher CASE scores at discharge may predict a poor long-term prognosis. |
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| institution | OA Journals |
| issn | 1664-3224 |
| language | English |
| publishDate | 2025-05-01 |
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| series | Frontiers in Immunology |
| spelling | doaj-art-080f3deb66654a22bdc53b43cff162312025-08-20T01:50:00ZengFrontiers Media S.A.Frontiers in Immunology1664-32242025-05-011610.3389/fimmu.2025.15917711591771Clinical spectrum and long-term outcomes of antibody-negative severe autoimmune encephalitis: a retrospective studyFangfang Li0Yu He1Yu He2Xiaoqian Chen3Ali Yang4Jiewen Zhang5Weizhou Zang6Department of Neurology, Henan Provincial People’s Hospital, Zhengzhou University People’s Hospital, Zhengzhou, Henan, ChinaDepartment of Neurology, Henan Provincial People’s Hospital, Zhengzhou University People’s Hospital, Zhengzhou, Henan, ChinaDepartment of Neurology, Henan University People’s Hospital, Zhengzhou, ChinaThe Second Affiliated Hospital of Luohe Medical College, Luohe, Henan, ChinaDepartment of Neurology, Henan Provincial People’s Hospital, Zhengzhou University People’s Hospital, Zhengzhou, Henan, ChinaDepartment of Neurology, Henan Provincial People’s Hospital, Zhengzhou University People’s Hospital, Zhengzhou, Henan, ChinaDepartment of Neurology, Henan Provincial People’s Hospital, Zhengzhou University People’s Hospital, Zhengzhou, Henan, ChinaObjectiveThe aims of the study were to characterize the clinical manifestations and outcomes of patients with antibody-negative severe autoimmune encephalitis (AE).MethodsThis retrospective, monocentric study recruited patients from the Neurology Department of Henan Provincial People’s Hospital between April 2017 and December 2023. All patients underwent neural antibody testing in both blood and cerebrospinal fluid (CSF) and met the diagnostic criteria for autoantibody-negative but probable severe AE, with available 1-year follow-up data.ResultsIn total, 124 patients with autoantibody-negative severe AE were analyzed. Among them, 27.4% achieved good functional outcomes at discharge. Older age (OR 1.034, 95% confidence interval [CI] 1.010-1.058, p = 0.004) and the presence of dyskinesia/dystonia (OR 8.463, 95% CI 3.282-21.820, p < 0.001) were predictive of poor short-term outcomes. At the 1-year follow-up, 54.8% experienced favorable long-term outcomes. Independent predictors of unfavorable long-term outcomes included older age (OR 1.076, 95% CI 1.018-1.136, p = 0.009), longer hospital stays (OR 1.264, 95% CI 1.105-1.446, p = 0.001), the presence of refractory status epilepticus (OR 14.765, 95% CI 1.759-123.935, p = 0.013) and higher CASE scores at discharge (OR 2.079, 95% CI 1.450-2.980, p < 0.001). Additionally, 30.6% of patients had relapsed, with refractory status epilepticus being an independent risk factor for relapse.ConclusionAlthough patients with antibody-negative severe AE experience significant disability in the early stages of their disease, the majority eventually regain independent functioning. Older age at disease onset, longer hospital stays, the presence of refractory status epilepticus and higher CASE scores at discharge may predict a poor long-term prognosis.https://www.frontiersin.org/articles/10.3389/fimmu.2025.1591771/fullantibody-negativesevereautoimmune encephalitisclinical characteristicsprognosis |
| spellingShingle | Fangfang Li Yu He Yu He Xiaoqian Chen Ali Yang Jiewen Zhang Weizhou Zang Clinical spectrum and long-term outcomes of antibody-negative severe autoimmune encephalitis: a retrospective study Frontiers in Immunology antibody-negative severe autoimmune encephalitis clinical characteristics prognosis |
| title | Clinical spectrum and long-term outcomes of antibody-negative severe autoimmune encephalitis: a retrospective study |
| title_full | Clinical spectrum and long-term outcomes of antibody-negative severe autoimmune encephalitis: a retrospective study |
| title_fullStr | Clinical spectrum and long-term outcomes of antibody-negative severe autoimmune encephalitis: a retrospective study |
| title_full_unstemmed | Clinical spectrum and long-term outcomes of antibody-negative severe autoimmune encephalitis: a retrospective study |
| title_short | Clinical spectrum and long-term outcomes of antibody-negative severe autoimmune encephalitis: a retrospective study |
| title_sort | clinical spectrum and long term outcomes of antibody negative severe autoimmune encephalitis a retrospective study |
| topic | antibody-negative severe autoimmune encephalitis clinical characteristics prognosis |
| url | https://www.frontiersin.org/articles/10.3389/fimmu.2025.1591771/full |
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