Clinical Features and Treatment Attitudes in Autoimmune Epilepsy: A Multicenter Retrospective Cohort Study
Abstract number :
2.123
Submission category :
4. Clinical Epilepsy / 4B. Clinical Diagnosis
Year :
2018
Submission ID :
501960
Source :
www.aesnet.org
Presentation date :
12/2/2018 4:04:48 PM
Published date :
Nov 5, 2018, 18:00 PM
Authors :
Flavio Villani, IRCCS Foundation Neurological Institute Carlo Besta; AnnaTeresa Giallonardo, University of Rome; Tiziana Granata, Foundation IRCCS Neurological Institute ‘‘C. Besta’’ Sara Casciato, University of Rome; Roberto Michel
Rationale: Autoimmune epilepsy is still an under-recognized condition with poorly standardized diagnostic and management guidelines. To assess the epileptic phenotypes of patients with definite or possible autoimmune epilepsy, and to analyze the treatment attitudes towards this condition. Methods: The Autoimmune Epilepsies Study Group of the Italian League Against Epilepsy performed a multicenter retrospective observational cohort study over a 10 years period. The series comprised 204 patients (40 children, 164 adults) with epileptic seizures as predominant or only symptom, in whom an autoimmune etiology was defined by the detection of pathogenic antibodies or suspected on clinical basis. The latter group included patients in whom paraclinical biomarkers supported CNS inflammation and immune activation, or patients successfully treated with immunotherapy, or with a history of systemic autoimmunity or recent infective disease. Results: Follow-up lasted at least 24 months. Autoantibodies were detected in 59% of cases. Most patients had focal seizures (76%), drug refractory in 85% of cases. High seizure frequency was recorded in 73%, mainly at disease onset, and status epilepticus occurred in 38%. In 89% of patients, cognitive compromise, behavioral disturbances, and movement disorders were also observed. 80% of patients were treated with immunotherapy with first- and/or second-line drug trials. A favorable response, with significant (= 50%) reduction in seizure frequency, was detected in patients who received early immunotherapy, and in those with cell-surface antibodies (p<0.05). Long-term outcomes were marked by persisting psychiatric and cognitive deficits in 23% of patients. Conclusions: The role of neuroinflammation and autoimmunity is increasingly recognized in triggering and sustaining epileptic activity. A thorough clinical and paraclinical characterization of definite and suspected autoimmune epilepsy may improve early recognition and treatment, with likely positive repercussions on long-term outcome. Funding: None