| Contributors: |
Lattanzi, S; Matricardi, S; Vogrig, A; Pauletto, G; Nosadini, M; Sartori, S; Massa, F; Benedetti, L; Meletti, S; Bisulli, F; Freri, E; Operto, Ff; Bozzetti, S; Mariotto, S; Beretta, S; Rosati, E; Cesaroni, E; Marini, C; Granata, T; Villani, F; Immune Epilepsies Study Group Of The Italian League Against Epilepsy., Ruggero Bernabei; Stefania Maria, Bova; Silva, Cappanera; Luigi, Caputi; Edward, Cesnik; Francesco, Deleo; Lidia Di, Vito; Giuseppe, D'Orsi; Giovanni, Falcicchio; Elisa, Fallica; Edoardo, Ferlazzo; Lucia, Fusco; Matteo, Gastaldi; Giada, Giovannini; Angela La, Neve; Claudio, Liguori; Pietro, Mattioli; Lorenzo, Muccioli; Carlotta, Mutti; Angelo, Pascarella; Elena, Pasini; Daniela, Passarelli; Francesca, Ragona; Romana, Rizzi; Elena, Tartara; Valentina, Tontini; Alberto, Verrotti; Federica, Zibordi; Lucia, Zinno; Antonio, Zito |
| Description: |
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%. |