Musicogenic Epilepsy: Expanding the Spectrum of GAD65 Neurological Autoimmunity (1850)

Objective: To describe the clinical features of patients with GAD65 autoimmunity and musicogenic epilepsy. Background: Description of GAD65 IgG seropositivity (GAD65 IgG+) among musicogenic epilepsy patients remains largely limited to a few case reports. Design/Methods: Electronic medical records were searched for the terms “musicogenic epilepsy” and “musicogenic seizures,” and laboratory data was utilized to determine patients with serum GAD65 titers >20 nnmol/L. Results: Fourteen patients were determined to have musicogenic epilepsy, of which 7 had serological evaluation and were all GAD65 IgG+. An additional patient was identified through the Neuroimmunology database. Of the 8 patients, 7 were women with a median age of seizure onset of 23 years (range 14–46). Median serum GAD65 titer was 173 nmol/L (range 20.3–3005 nmol/L) and CSF titer (n=3) was 4.2 nmol/L (range 3.6–25.1 nmol/L). Other clinical/neurological comorbidities included type-1 diabetes (n=3; 38%), thyroid disease (n=3; 38%), cognitive dysfunction (n=3; 38%), stiff person syndrome (n=1; 13%), and gait ataxia (n=1; 13%). All patients had temporal lobe epilepsy. Right temporal lobe seizures were most frequently captured when seizures were induced by music on EEG (3/4; 75%). The majority (n=7, 88%) recognized certain provoking songs/genre. IV methylprednisolone and/or IVIG was trialed for seizures in 4 (50%) with one patient having >50% reduction in seizures. Rituximab (n=2) and mycophenolate (n=1) were ineffective. Median duration of seizure onset to immunotherapy initiation was 4 years (range 1–16 years). Two patients underwent right temporal resections but continued to have seizures. VNS reduced seizures in 1 patient by 50%; another patient was essentially seizure free by avoiding provoking music. Conclusions: GAD65 antibodies should be tested in all patients with musicogenic epilepsy given implications for management and screening for comorbid autoimmune conditions. Early recognition and early immunotherapy initiation may have the potential to lead to better outcomes. Disclosure: Dr. Smith has nothing to disclose. Dr. Zalewski has nothing to disclose. Dr. Budhram has nothing to disclose. The institution of Dr. Britton has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB. Dr. Britton has received personal compensation in the range of $500-$4,999 for serving as a Webinar participant with American Clinical Neurophysiology Society. Dr. So has nothing to disclose. Dr. Cascino has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for American Academy of Neurology. Dr. Cascino has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for International League Against Epilepsy . The institution of Dr. Cascino has received research support from Neurelis, Inc.. The institution of Dr. Cascino has received research support from Epilepsy Study Consortium. The institution of Dr. Cascino has received research support from NINDS. The institution of Dr. Cascino has received research support from Xenon, Inc.. The institution of Dr. Cascino has received research support from UCB Biosciences, Inc.. Dr. Cascino has received intellectual property interests from a discovery or technology relating to health care. Dr. Cascino has received intellectual property interests from a publication relating to health care. Dr. Cascino has received intellectual property interests from a publication relating to health care. The institution of Dr. McKeon has received research support from Euroimmun AG. Dr. McKeon has received intellectual property interests from a discovery or technology relating to health care. Dr. Pittock has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Genentech, Inc.. Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sage Therapeutics, Inc.. The institution of Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Astellas. Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB, Inc. Dr. Pittock has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Hoffman/LaRoche AG. Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genetech. The institution of Dr. Pittock has received research support from Grifols. The institution of Dr. Pittock has received research support from NIH. The institution of Dr. Pittock has received research support from Viela Bio/MedImmune. The institution of Dr. Pittock has received research support from Alexion Pharmaceuticals. The institution of Dr. Pittock has received research support from Alexion Pharmaceuticals. Dr. Pittock has received intellectual property interests from a discovery or technology relating to health care. Dr. Pittock has received intellectual property interests from a discovery or technology relating to health care. The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB. The institution of Dr. Dubey has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Astellas. Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB. Dr. Dubey has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for AAN. Dr. Dubey has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for AGRIMS. Dr. Dubey has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Advances in Neurology . Dr. Dubey has received research support from Center of Individualized Medicine, Mayo Clinic .