Clinical features and outcomes of anti-NMDAR autoimmune-related central nervous system involvement in patients with teratoma: a retrospective cohort study

Teratoma-associated anti-NMDAR encephalitis represents a rare but clinically severe subset of autoimmune encephalitis. Evidence regarding predictors of disease severity and 12-month functional outcomes remains limited. In this single-center retrospective cohort study, 84 patients who met the inclusion criteria for teratoma-associated anti-NMDAR encephalitis were included for analysis. Clinical manifestations, MRI and EEG findings, treatment characteristics, and tumor-related markers (Ki-67, AFP, LDH) were systematically collected. Severe disease was defined as requiring ICU care with organ support, mechanical ventilation, or documented central hypoventilation. Functional outcomes were evaluated using the modified Rankin Scale (mRS) at 6 and 12 months. Univariable and multivariable logistic regression models were used to identify independent predictors of poor 12-month outcomes (mRS > 2). ROC analyses assessed discriminatory performance. Central hypoventilation (57.14% vs. 10.71%), multifocal MRI lesions (25.00% vs. 7.14%), extreme delta-brush on EEG (42.86% vs. 12.50%), and higher symptom burden were significantly associated with severe disease (all P < 0.05). At 12 months, 23.81% of patients had poor functional outcomes. Independent predictors of poor outcome included central hypoventilation (adjusted OR 5.86, 95% CI 2.01–17.10), ≥ 4 symptom categories (adjusted OR 3.42, 95% CI 1.15–10.20), multifocal MRI involvement (adjusted OR 3.71, 95% CI 1.02–13.40), extreme delta-brush (adjusted OR 4.28, 95% CI 1.31–13.90), and tumor resection > 21 days after onset (adjusted OR 3.16, 95% CI 1.05–9.53). The multivariable model achieved strong predictive performance (AUC 0.89). Markers of diffuse neuroinvolvement—including central hypoventilation, higher symptom burden, multifocal MRI lesions, and extreme delta-brush—are key indicators of severe disease and poor long-term outcomes in pediatric teratoma-associated anti-NMDAR encephalitis.

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Journal
BMC Neurology
Published
2026-02-27
DOI
https://doi.org/10.1186/s12883-026-04717-8
Primary Topic
Autoimmune Neurological Disorders and Treatments
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article
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article

Clinical features and outcomes of anti-NMDAR autoimmune-related central nervous system involvement in patients with teratoma: a retrospective cohort study

Yue Huang, Zhan Huang, Mingfang Zhu, Leilei Tan et al.
BMC Neurology
Autoimmune Neurological Disorders and Treatments
article

Clinical features and outcomes of anti-NMDAR autoimmune-related central nervous system involvement in patients with teratoma: a retrospective cohort study

Yue Huang, Zhan Huang, Mingfang Zhu, Leilei Tan, Zhengyu Sun
article en

Abstract

Teratoma-associated anti-NMDAR encephalitis represents a rare but clinically severe subset of autoimmune encephalitis. Evidence regarding predictors of disease severity and 12-month functional outcomes remains limited. In this single-center retrospective cohort study, 84 patients who met the inclusion criteria for teratoma-associated anti-NMDAR encephalitis were included for analysis. Clinical manifestations, MRI and EEG findings, treatment characteristics, and tumor-related markers (Ki-67, AFP, LDH) were systematically collected. Severe disease was defined as requiring ICU care with organ support, mechanical ventilation, or documented central hypoventilation. Functional outcomes were evaluated using the modified Rankin Scale (mRS) at 6 and 12 months. Univariable and multivariable logistic regression models were used to identify independent predictors of poor 12-month outcomes (mRS > 2). ROC analyses assessed discriminatory performance. Central hypoventilation (57.14% vs. 10.71%), multifocal MRI lesions (25.00% vs. 7.14%), extreme delta-brush on EEG (42.86% vs. 12.50%), and higher symptom burden were significantly associated with severe disease (all P < 0.05). At 12 months, 23.81% of patients had poor functional outcomes. Independent predictors of poor outcome included central hypoventilation (adjusted OR 5.86, 95% CI 2.01–17.10), ≥ 4 symptom categories (adjusted OR 3.42, 95% CI 1.15–10.20), multifocal MRI involvement (adjusted OR 3.71, 95% CI 1.02–13.40), extreme delta-brush (adjusted OR 4.28, 95% CI 1.31–13.90), and tumor resection > 21 days after onset (adjusted OR 3.16, 95% CI 1.05–9.53). The multivariable model achieved strong predictive performance (AUC 0.89). Markers of diffuse neuroinvolvement—including central hypoventilation, higher symptom burden, multifocal MRI lesions, and extreme delta-brush—are key indicators of severe disease and poor long-term outcomes in pediatric teratoma-associated anti-NMDAR encephalitis.

BMC Neurology
Zhengzhou University (CN), Henan Provincial People's Hospital (CN)
Good health and well-being
Openalex Percentile: Top 46%
Autoimmune Neurological Disorders and Treatments
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