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Abstract Details

Reliable Count of Seizures using Diaries versus Video Electroencephalography for Seizures of KCNQ2-DEE
Epilepsy/Clinical Neurophysiology (EEG)
P7 - Poster Session 7 (5:30 PM-6:30 PM)
12-007
We sought evidence as to whether seizures in infants and young children with KCNQ2 Development Epileptic Encephalopathy (KCNQ2-DEE) could be reliably counted using diaries, with seizure behaviors previously confirmed using video electroencephalography (VEEG).
The Food and Drug Administration has recommended VEEG to measure the outcome of anti-seizure mediation clinical trials for infants and young children.  Reasons include difficulty with seizure recognition due to clinically subtle seizure behaviors, and occurrence of subclinical electro-encephalographic seizures in this age groups.  However, use of VEEG presents technical and feasibility challenges, and limits enrollment to subjects with very high seizure frequencies. 
PubMED was searched for papers in which patients with KCNQ2-DEE included seizure and EEG correlate descriptions.  Additionally, a survey of caregivers from the Cure Alliance (Patient Advocacy Group) was reviewed for information regarding recognition of seizure occurrence.
Sixteen papers were included, encompassing 124 KCNQ2-DEE patients.  Each described stereotyped focal tonic seizures beginning on days 1-5 after birth.  Focal tonic seizure was the predominant and often only seizure type present in the loss-of-function variants. The seizures were readily observed and were associated with a clear EEG correlate of contralateral amplitude suppression.  There were no reports of electro-encephalographic subclinical seizures.  To understand the ability of caregivers to record seizures, a survey of KCNQ2-DEE families was undertaken, with 55 evaluable responses obtained.  On a scale of 1 to10 (low to high) confidence in recognizing seizures after training using VEEG, 75% of caregivers reported high confidence (8 or above).
We conclude that the seizures of KCNQ2-DEE are stereotyped, clinically evident and do not occur without an observable clinical correlation.  Parents/caregivers are recognizing seizures confidently.  This strategy of training families to count seizures accurately using VEEG mitigates the need to use VEEG directly as the method to assess primary outcome in a KCNQ2-DEE anti-seizure medication treatment trial.  
Authors/Disclosures
Cynthia L. Harden, MD (Xenon Pharmaceuticals Inc)
PRESENTER 		Dr. Harden has received personal compensation for serving as an employee of Xenon Pharmaceuticals Inc.. Dr. Harden has stock in Xenon Pharmaceuticals Inc.. Dr. Harden has received intellectual property interests from a discovery or technology relating to health care.
Celene Grayson, PhD (Xenon Pharmaceuticals Inc) Dr. Grayson has received personal compensation for serving as an employee of Xenon Pharmaceuticals Inc.. Dr. Grayson has stock in Xenon Pharmaceuticals Inc..
Constanza Luzon Constanza Luzon has received personal compensation for serving as an employee of Xenon Pharmaceuticals Inc.. Constanza Luzon has stock in Xenon Pharmaceuticals.
Noam Butterfield, PhD (Xenon Pharmaceuticals) Dr. Butterfield has received personal compensation for serving as an employee of Xenon Pharmaceuticals.
Ernesto Aycardi, MD (Kyowa Kirin) Dr. Aycardi has received personal compensation for serving as an employee of XENON. Dr. Aycardi has received stock or an ownership interest from XENON.
No disclosure on file
John J. Millichap, MD, FÂ鶹´«Ã½Ó³»­ Dr. Millichap has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Xenon. Dr. Millichap has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Praxis. Dr. Millichap has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biohaven. Dr. Millichap has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Praxis. Dr. Millichap has received publishing royalties from a publication relating to health care.