Objective: This study aimed to identify risk factors and develop a predictive scoring system for autoimmune-associated epilepsy in subjects with autoimmune encephalitis presenting with new onset refractory status epilepticus (NORSE). Methods: This retrospective, multicenter, cohort study included subjects who presented with NORSE at the onset of autoimmune encephalitis and had at least 24 months of follow-up after immunotherapy. The outcome was the development of autoimmune-associated epilepsy, defined as persistent seizures despite adequate immunotherapy and absence of active inflammation. Factors independently associated with the outcome were identified through a backward stepwise selection. Adjusted regression coefficients of each independent predictor were transformed to produce a points-based risk-scoring system. Results: Seventy participants were included (median age = 24.2 years, 38.6% male). During a median follow-up of 53 months, 54.3% of subjects developed autoimmune-associated epilepsy. Status epilepticus duration ≥ 10 days (odds ratio [OR] = 31.14, 95% confidence interval [CI] = 2.12–456.87, p =.012), positivity for antibodies against surface antigens (OR =.12, 95% CI =.02–.85, p =.034), bitemporal magnetic resonance imaging (MRI) abnormalities suggestive of autoimmune encephalitis during acute stage (OR = 49.80, 95% CI = 2.95–841.77, p =.007), and interictal epileptiform discharges during electroencephalographic (EEG) follow-up (OR = 71.32, 95% CI = 6.48–785.32, p <.001) were independently associated with the study outcome. The duration–antibodies–MRI–EEG (DAME) score was developed as an integer-based scoring system predictive of autoimmune-associated epilepsy. With an optimal cutoff of ≥3 points, it yielded a sensitivity of 86.8%, a specificity of 87.5%, and an overall accuracy of 87.1%. Significance: The DAME score could serve as a user-friendly score to predict the risk of autoimmune-associated epilepsy in patients with NORSE due to autoimmune encephalitis.
Predicting epilepsy after new onset refractory status epilepticus due to autoimmune encephalitis: The DAME score / Lattanzi, S.; Matricardi, S.; Vogrig, A.; Pauletto, G.; Nosadini, M.; Sartori, S.; Massa, F.; Benedetti, L.; Meletti, S.; Bisulli, F.; Freri, E.; Operto, F. F.; Bozzetti, S.; Mariotto, S.; Beretta, S.; Rosati, E.; Cesaroni, E.; Marini, C.; Granata, T.; Villani, F.; Bernabei, R.; Bova, S. M.; Cappanera, S.; Caputi, L.; Cesnik, E.; Deleo, F.; Di Vito, L.; D'Orsi, G.; Falcicchio, G.; Fallica, E.; Ferlazzo, E.; Fusco, L.; Gastaldi, M.; Giovannini, G.; La Neve, A.; Liguori, C.; Mattioli, P.; Muccioli, L.; Mutti, C.; Pascarella, A.; Pasini, E.; Passarelli, D.; Ragona, F.; Rizzi, R.; Tartara, E.; Tontini, V.; Verrotti, A.; Zibordi, F.; Zinno, L.; Zito, A.. - In: EPILEPSIA. - ISSN 0013-9580. - 67:4(2026), pp. 1792-1801. [10.1002/epi.70081]
Predicting epilepsy after new onset refractory status epilepticus due to autoimmune encephalitis: The DAME score
Massa F.;Benedetti L.;Meletti S.;Marini C.;D'Orsi G.;Fusco L.;Liguori C.;Mattioli P.;Muccioli L.;Mutti C.;Pascarella A.;Rizzi R.;Tontini V.;Zinno L.;
2026-01-01
Abstract
Objective: This study aimed to identify risk factors and develop a predictive scoring system for autoimmune-associated epilepsy in subjects with autoimmune encephalitis presenting with new onset refractory status epilepticus (NORSE). Methods: This retrospective, multicenter, cohort study included subjects who presented with NORSE at the onset of autoimmune encephalitis and had at least 24 months of follow-up after immunotherapy. The outcome was the development of autoimmune-associated epilepsy, defined as persistent seizures despite adequate immunotherapy and absence of active inflammation. Factors independently associated with the outcome were identified through a backward stepwise selection. Adjusted regression coefficients of each independent predictor were transformed to produce a points-based risk-scoring system. Results: Seventy participants were included (median age = 24.2 years, 38.6% male). During a median follow-up of 53 months, 54.3% of subjects developed autoimmune-associated epilepsy. Status epilepticus duration ≥ 10 days (odds ratio [OR] = 31.14, 95% confidence interval [CI] = 2.12–456.87, p =.012), positivity for antibodies against surface antigens (OR =.12, 95% CI =.02–.85, p =.034), bitemporal magnetic resonance imaging (MRI) abnormalities suggestive of autoimmune encephalitis during acute stage (OR = 49.80, 95% CI = 2.95–841.77, p =.007), and interictal epileptiform discharges during electroencephalographic (EEG) follow-up (OR = 71.32, 95% CI = 6.48–785.32, p <.001) were independently associated with the study outcome. The duration–antibodies–MRI–EEG (DAME) score was developed as an integer-based scoring system predictive of autoimmune-associated epilepsy. With an optimal cutoff of ≥3 points, it yielded a sensitivity of 86.8%, a specificity of 87.5%, and an overall accuracy of 87.1%. Significance: The DAME score could serve as a user-friendly score to predict the risk of autoimmune-associated epilepsy in patients with NORSE due to autoimmune encephalitis.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
