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Abstract Details

Generalized Periodic Epileptiform Discharges Herald Impending Seizures
Clinical Neurophysiology
P02 - (-)
241
BACKGROUND: GPEDs are often thought to be synonymous with Creutzfeldt-Jakob disease. However, this EEG pattern can occur in other conditions, including epileptic syndromes. There is growing evidence that GPEDs may herald impending seizures. Periodic epileptiform discharges have also rarely been reported in association with cerebral visual impairment.
DESIGN/METHODS: Case-report and literature review.
RESULTS: A 10 month old boy presented with continuous right facial twitching and left upper extremity tonic posturing. This ceased following IV fosphenytoin, levetiracetam, midazolam, and propofol. However, he was subsequently unable to track or fixate on objects. Pupillary response to light and fundoscopic examination were normal. An emergency EEG revealed GPEDs, warranting prolonged EEG monitoring. During monitoring, he had two seizures of left central onset characterized by eye deviation, right arm posturing, and right facial twitching. After initiating the ketogenic diet, his GPEDs and seizure activity ceased. The child underwent multiple serologic, urine, and CSF studies; these returned within normal limits. MRI brain revealed brachycephaly. After learning that he had suffered from focal onset and generalized tonic clonic seizures since a vaccination at age 2 months, testing of the SCN1A gene was performed. This revealed a de novo mutation at C.667 C>T, consistent with Dravet syndrome. His vision improved over the next several months, and he was able to track and fixate on faces once again.
CONCLUSIONS: The detection of GPEDs is important, as such discharges can herald impending seizures and warrant further monitoring. Previous studies have demonstrated that up to 90% of patients with GPEDs can have seizures within the next 48 hours. Although uncommon in children, GPEDs can be detected at any age. As our case demonstrates, cerebral visual impairment can result from seizure activity.
Authors/Disclosures
Brian D. Moseley, MD
PRESENTER 		Dr. Moseley has received personal compensation for serving as an employee of UCB. Dr. Moseley has received personal compensation for serving as an employee of Neurocrine Biosciences Inc. Dr. Moseley has or had stock in UCB.
David S. Goldstein, MD, PhD (National Institutes of Health) Dr. Goldstein has received publishing royalties from a publication relating to health care.
Elaine C. Wirrell, MD, FAAN (Mayo Clinic/Dept of Child Neurology) Dr. Wirrell has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Biocodex. Dr. Wirrell has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Encoded. Dr. Wirrell has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Neurocrine. Dr. Wirrell has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for GRIN. Dr. Wirrell has received publishing royalties from a publication relating to health care.
Katherine C. Nickels, MD, FAAN (Mayo Clinic) The institution of Dr. Nickels has received research support from Zogenix. The institution of Dr. Nickels has received research support from Marinus. The institution of Dr. Nickels has received research support from Bio-Pharm Solutions. The institution of Dr. Nickels has received research support from Pediatric Epilepsy Research Foundation. Dr. Nickels has received personal compensation in the range of $500-$4,999 for serving as a Faculty with J. Kiffin Penry 好色先生al Programs.
Eric R. Eggenberger, DO, FAAN (Mayo Clinic Florida) Dr. Eggenberger has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Welch-Allyn. Dr. Eggenberger has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Illinois Institute of Technology (IITT). Dr. Eggenberger has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Argenx.
Eric R. Eggenberger, DO, FAAN (Mayo Clinic Florida) Dr. Eggenberger has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Welch-Allyn. Dr. Eggenberger has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Illinois Institute of Technology (IITT). Dr. Eggenberger has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Argenx.