Objective To describe the clinical and paraclinical findings, treatment options and long-term outcomes in autoimmune encephalitis (AE), with a close look to epilepsy.
Methods In this retrospective observational cohort study, we enrolled patients with new-onset seizures in the context of AE. We compared clinical and paraclinical findings in patients with and without evidence of antibodies.
Results Overall, 263 patients (138 females; median age 55 years, range 4–86) were followed up for a median time of 30 months (range 12–120). Antineuronal antibodies were detected in 63.50%.
Antibody-positive patients had multiple seizure types (p=0.01) and prevalent involvement of temporal regions (p=0.02). A higher prevalence of episodes of SE was found in the antibody-negative group (p<0.001).
Immunotherapy was prescribed in 88.60%, and effective in 61.80%. Independent predictors of favourable outcome of the AE were early immunotherapy (p<0.001) and the detection of antineuronal surface antibodies (p=0.01).
Autoimmune-associated epilepsy was the long-term sequela in 43.73%, associated with cognitive and psychiatric disturbances in 81.73%. Independent predictors of developing epilepsy were difficult to treat seizures at onset (p=0.04), a high number of antiseizure medications (p<0.001), persisting interictal epileptiform discharges at follow-up (p<0.001) and poor response to immunotherapy during the acute phase (p<0.001).
Conclusions The recognition of seizures secondary to AE represents a rare chance for aetiology-driven seizures management. Early recognition and treatment at the pathogenic level may reduce the risk of long-term irreversible sequelae. However, the severity of seizures at onset is the major risk factor for the development of chronic epilepsy.
This study provides class IV evidence for management recommendations.
- AUTOIMMUNE ENCEPHALITIS
Data availability statement
Data are available on reasonable request.
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Collaborators Collaborators: Immune Epilepsies Study Group of the Italian League Against Epilepsy (LICE) Collaborators: Umberto Aguglia, Vincenzo Belcastro, Luana Benedetti, Simone Beretta, Stefania Maria Bova, Claudia Cagnetti, Susanna Casellato, Lorenzo Celli, Elisabetta Cesaroni, Eduard Cesnick, Giangennaro Coppola, Edvige Correnti, Giuseppe Didato, Giuseppe D’Orsi, Elena Fallica, Alessandra Ferrari, Alessandro Ferretti, Carlo Andrea Galimberti, Annateresa Giallonardo, Loretta Giuliano, Angela La Neve, Claudio Liguori, Carla Marini, Federico Massa, Massimo Mastrangelo, Laura Mumoli, Carlotta Mutti, Francesca Felicia Operto, Elena Pasini, Chiara Pastori, Daniela Passarelli, Giada Pauletto, Chiara Pizzanelli, Francesca Ragona, Patrizia Riguzzi, Romana Rizzi, Marta Elena Santarone, Delia Simula, Vito Sofia, Maria Tappatà, Elena Tartara, Francesca Vanadia, Alberto Verrotti, Laura Tassi, Lucia Zinno.
Contributors SM is responsible for the overall content as the guarantor. SM, TG, EF, SL, FD and FV conceptualised and designed the study. SM, SC, FD, EF, SL, AS, SS, MN, CDB, GDG, IP, SM, GG, EZ, SB, SM, SF, LZ, MZ, AV, RM, AG, EF, LF, and FV selected and enrolled patients, critically reviewing all medical charts and records. SM, TG, FD, SM, SB, SL and FV were involved with the dataset and analysis. SM, TG and FV drafted the manuscript. All collaborators of the Immune Epilepsies Study Group of the Italian League Against Epilepsy (LICE) are responsible for each case data collection and diagnostic process. All authors and collaborators edited the manuscript.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.
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