Abstracts

Rationale:
To report a case of multi-focal musicogenic and auditory right temporal lobe epilepsy with unique stereotactic EEG (sEEG) findings when the patient was exposed to musical triggers, and resolution of the musicogenic component following right anterior temporal lobectomy (RATL).
Method:
Clinical case report derived from chart review and phone interview. Case Summary: AP is a 30-year-old right-handed woman with intractable autoimmune focal epilepsy and obsessive-compulsive disorder. Seizure onset was at age 11. Initial seizures were described as “sirens” with isolated perception of siren-like sounds heard internally, occurring 1-2 times per month. In her early 20s, AP developed a second seizure type that was often triggered by music, beginning with an aura of a sudden jolt through the body, hyper-awareness of surroundings and “déjà vu on steroids” with progression to oral automatisms and staring spells and frequent LOC. This then became her primary seizure type. Autoimmune testing demonstrated elevated anti-GAD65 antibodies in the serum and CSF. Pre-surgical evaluation included Phase I (which localized all seizures and interictal abnormalities to the right hemisphere), SPECT, and sEEG. sEEG consisted of 9 right hemispheric depth electrodes recorded on Natus 128-channel sEEG.  
Results:
SEEG localized the “siren” seizures to the right Heschl’s gyrus (superior temporal gyrus or STG) and the “déjà vu” seizures to the hippocampus, amygdala and uncus. sEEG also recorded a third electrographic seizure onset in the right insular region. When AP was exposed to her typical musical triggers (e.g. “I want it that way” by Backstreet Boys and certain country music), a rhythmic delta pattern emerged in the entorhinal cortex that synchronized with the beat of the song; the pattern attenuated when AP was distracted or the music stopped. AP underwent RATL sparing the STG. Post-ATL, the “déjà vu” seizures stopped and her seizures were no longer triggered by music, but the “siren” seizures persisted. AP subsequently underwent RNS system placement with depth electrodes to the 1) right Heschl’s gyrus, and 2) insular region. RNS stimulation was complicated by AP experiencing a consistently-reproducible beeping sound whenever stimulation is delivered. She continues to have siren seizures 2 to 3 times per month, but quality of life has greatly improved with cessation of “déjà vu” seizures and convulsions.  
Conclusion:
This case report highlights the complexity and multi-focal localization of musicogenic and auditory epilepsy. Treatment required a multi-faceted approach and there were specific complications with neurostimulation.
Funding:
:NIH funded BUILD EXITO research training program