Abstract Details

Title Musicogenic Epilepsy: Expanding the Spectrum of GAD65 Neurological Autoimmunity

Topic Autoimmune Neurology

Presentation(s) Autoimmune Neurology Posters (7:00 AM-5:00 PM)

Poster/Presentation
Number 070

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.

Authors/Disclosures
Kelsey M. Smith, MD (Mayo Clinic) PRESENTER	The institution of Dr. Smith has received research support from CURE Epilepsy. The institution of Dr. Smith has received research support from UCB Pharmaceuticals.
Nicholas L. Zalewski, MD (Mayo Clinic)	Dr. Zalewski has nothing to disclose.
Adrian Budhram, MD (London Health Sciences Centre)	Dr. Budhram has nothing to disclose.
Jeffrey W. Britton, MD, FAAN (Mayo Graduate School of Medicine)	Dr. Britton has received personal compensation in the range of $0-$499 for serving as a Online course with American Clinical Neurophysiology Society.
Elson L. So, MD, FAAN (Mayo Clinic)	Dr. So has nothing to disclose.
Gregory D. Cascino, MD, FAAN (Mayo Clinic)	Dr. Cascino has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for International League Against Epilepsy . Dr. Cascino has received intellectual property interests from a discovery or technology relating to health care. Dr. Cascino has received publishing royalties from a publication relating to health care. Dr. Cascino has received publishing royalties from a publication relating to health care.
Andrew McKeon, MD (Mayo Clinic)	The institution of Dr. McKeon has received research support from National Institutes of Health. Dr. McKeon has received intellectual property interests from a discovery or technology relating to health care. Dr. McKeon has received intellectual property interests from a discovery or technology relating to health care. Dr. McKeon has received publishing royalties from a publication relating to health care.
Sean J. Pittock, MD, FAAN (Mayo Clinic Dept of Neurology)	Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arialys. The institution of 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 Alexion. The institution of 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. The institution of 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 Roche/Genentech. The institution of Dr. Pittock has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Alexion/AstraZeneka. The institution of Dr. Pittock has received research support from NIH. 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. Dr. Pittock has received publishing royalties from a publication relating to health care.
Divyanshu Dubey, MD, FAAN (Mayo Clinic)	The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Argenx. The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arialys. The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB . Dr. Dubey has received research support from Department of Defense . Dr. Dubey has received research support from Department of Defense . Dr. Dubey has received research support from UCB. Dr. Dubey has received research support from David J. Tomassoni ALS Research Grant Program . Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care.

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