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

畸胎瘤患者中抗NMDAR自身免疫相关中枢神经系统受累的临床特征和预后:一项回顾性队列研究

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Abstract

BACKGROUND: 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. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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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