PT - JOURNAL ARTICLE AU - Bozzetti, Silvia AU - Rossini, Fabio AU - Ferrari, Sergio AU - Delogu, Rachele AU - Cantalupo, Gaetano AU - Marchioretto, Fabio AU - Zanette, Giampietro AU - Zanoni, Tiziano AU - Turatti, Marco AU - Vitale, Giuseppina AU - Cadaldini, Morena AU - Rossi, Francesca AU - Di Tizio, Luca AU - Zuco, Carmela AU - Maniscalco, Giorgia Teresa AU - Soldani, Fabio AU - Monaco, Salvatore AU - Trinka, Eugen AU - Hoeftberger, Romana AU - Mariotto, Sara TI - Epileptic seizures of suspected autoimmune origin: a multicentre retrospective study AID - 10.1136/jnnp-2020-323841 DP - 2020 Nov 01 TA - Journal of Neurology, Neurosurgery & Psychiatry PG - 1145--1153 VI - 91 IP - 11 4099 - http://jnnp.bmj.com/content/91/11/1145.short 4100 - http://jnnp.bmj.com/content/91/11/1145.full SO - J Neurol Neurosurg Psychiatry2020 Nov 01; 91 AB - Objective To analyse autoantibody status in a well-defined European multicentre cohort of patients with epilepsy of unknown aetiology and to validate the recently proposed Antibody Prevalence in Epilepsy (APE2) and Response to ImmunoTherapy in Epilepsy (RITE2) scores.Methods We retrospectively collected clinical and paraclinical data of 92 patients referred to the Neurology Units of Verona and Salzburg between January 2014 and July 2019 with new-onset epilepsy, status epilepticus or chronic epilepsy of unknown aetiology. Fixed and live cell-based assays, tissue-based assays, immunoblot, and live rat hippocampal cell cultures were performed in paired serum/cerebrospinal fluid (CSF) to detect antineuronal and antiglial antibodies. The APE2 and RITE2 scores were then calculated and compared with clinical and laboratory data.Results Autoantibodies were detected in 29/92 patients (31.5%), with multiple positivity observed in 6/29 cases. The APE2 score (median 5, range 1–15) significantly correlated with antibody positivity (p=0.014), especially for the presence of neuropsychiatric symptoms (p<0.01), movement disorders (p<0.01), dysautonomia (p=0.03), faciobrachial dyskinesias (p=0.03) and cancer history (p<0.01). Status epilepticus was significantly more frequent in antibody-negative patients (p<0.01). Among the items of the RITE2 score, early initiation of immunotherapy correlated with a good treatment response (p=0.001), whereas a cancer history was significantly more common among non-responders (p<0.01). Persistence of neuropsychiatric symptoms and seizures correlated with antiepileptic maintenance after at least 1 year.Conclusions This is the first study that independently validates the APE2 and RITE2 scores and includes the largest cohort of patients whose paired serum and CSF samples have been tested for autoantibodies possibly associated with autoimmune epilepsy.