Determinants of disease severity and 12-month functional outcome in pediatric ovarian Teratoma-Associated Anti-NMDA receptor encephalitis.
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BACKGROUND: Pediatric ovarian teratoma-associated anti-N-methyl-D-aspartate receptor encephalitis (NMDARE) represents a clinically important but relatively uncommon subgroup, and evidence regarding factors associated with acute severity and long-term functional outcome in this population remains limited. METHODS: We conducted a single-center retrospective cohort study of pediatric patients with surgically resected, histopathologically confirmed ovarian teratoma-associated NMDARE. Baseline and acute-phase characteristics were summarized in the full cohort (N = 97). Acute severity was defined by ICU admission with organ support and/or mechanical ventilation. Twelve-month functional outcome was assessed using the modified Rankin Scale (mRS) and dichotomized as good (mRS≤2) versus poor (mRS>2); outcome analyses were performed in the complete-case cohort (N = 71). Univariable and multivariable logistic regression identified factors independently associated with poor outcome. Discriminative performance was evaluated using ROC curve analysis. RESULTS: Among 97 patients, median age at onset was 14.2 years (IQR 12.3-16.5) and 95.9% were female. Mature teratoma accounted for 80.4%. The median onset-to-resection interval was 18 days (IQR 11-29), and 34.0% underwent resection >21 days after onset. Severe disease occurred in 29 patients (29.9%) and was associated with higher symptom burden (median symptom categories 5.0 vs 3.0, P < 0.001), more frequent MRI abnormalities (62.1% vs 33.8%, P = 0.009) and multifocal MRI lesions (27.6% vs 7.4%, P = 0.013), and higher rates of EEG background slowing (89.7% vs 61.8%, P = 0.014) and extreme delta-brush (44.8% vs 13.2%, P = 0.001). In the 12-month outcome cohort (N = 71), 76.1% achieved mRS≤2 and 23.9% had mRS>2; relapse occurred in 8.5% and death in 2.8%. Multivariable analysis identified central hypoventilation (aOR 4.18, 95%CI 1.62-10.78), symptom categories ≥4 (aOR 2.94, 1.14-7.61), multifocal MRI lesions (aOR 3.12, 1.01-9.65), extreme delta-brush (aOR 3.37, 1.18-9.63), and onset-to-resection >21 days (aOR 2.81, 1.05-7.49) as independent correlates of poor functional outcome. The combined clinical plus imaging-EEG model showed the highest discrimination (AUC 0.86, 95%CI 0.76-0.95), with sensitivity 88.2% and specificity 74.1% at the optimal cutoff. CONCLUSIONS: In pediatric ovarian teratoma-associated NMDARE, higher acute-phase clinical burden, multifocal MRI abnormalities, extreme delta-brush, central hypoventilation, and delayed tumor resection were independently associated with poor 12-month functional outcome.