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Abstract Details

Seizures in the Leukodystrophies
Epilepsy/Clinical Neurophysiology (EEG)
P6 - Poster Session 6 (8:00 AM-9:00 AM)
1-001
Identify seizure risk factors and quantify the efficacy of anti-seizure medications in patients with leukodystrophy 

Seizures are an understudied feature of leukodystrophies. Poorly understood aspects include disease-specific prevalence, clinical characteristics, and EEG patterns conferring the greatest risk of seizures. The natural history and optimum treatment for seizures in leukodystrophies are not well known.  

 
This is a cross-sectional retrospective analysis of 706 pediatric patients across 8 health centers (Appendix 1) from 1/1/2013 to 3/31/2020. This study made use of a limited dataset from an established database, so no consent process was required. Patient characteristics were reported with median values and interquartile ranges for continuous variables and frequency and percentage for categorical variables. Wilcoxon rank sum test, Chi-squared test and Fisher’s exact test were used to calculate p values. Logistic regression was performed to assess seizure risk factors.  
Seizures occur in 29% of patients. Every one-year increase in age, the patient is 1.13 times more likely to have seizure (p<0.001). Patients with focal slowing are 3.58 times more likely to have seizure (p<0.001), and those with generalized epileptiform discharges are 13.46 times more likely to have seizure (p=0.019). Diffuse slowing and transplant status were not significantly associated with seizure (p=0.159 and p=0.377, respectively). 37% of patients with seizures require more than one antiseizure medication to control their seizures. 80% of patients taking lacosamide and 72% of patients taking levetiracetam report these medications as beneficial, while only 33% of patients taking oxcarbazepine report benefit.
Age, focal slowing, and epileptiform discharges are significant predictors for seizure in this population. Abnormal MRI brain and generalized slowing on EEG are widespread in this cohort and do not confer increased seizure risk. Patients with generalized discharges have higher risk of seizures compared with focal discharges. Levetiracetam and lacosamide were deemed effective antiseizure medications in this population.  
Authors/Disclosures
Ashley N. Hackett, MD (Texas Scottish Rite for Children)
PRESENTER 		Dr. Hackett has nothing to disclose.
Katie Liu No disclosure on file
Helena Yan, MD Ms. Yan has nothing to disclose.
Camille S. Corre, MD (University of Rochester Medical Center) Dr. Corre has nothing to disclose.
No disclosure on file
Daniel C. Kelly Mr. Kelly has nothing to disclose.
Gabrielle T. Ghabash Ms. Ghabash has nothing to disclose.
Jordan Goodman No disclosure on file
William Benko, MD (WellSpan Neurology) Dr. Benko has received personal compensation in the range of $500-$4,999 for serving as a Expert- Surveys with Connected Research .
Robert I. Thompson-Stone, MD, FAAN (University of Rochester) Dr. Thompson-Stone has received personal compensation in the range of $500-$4,999 for serving as a Krabbe Disease expert with Advisory Committee on Heritable Disorders in Newborns and Children (ACHDNC). Dr. Thompson-Stone has a non-compensated relationship as a Editorial Board Member with Neurology: 好色先生 Journal that is relevant to AAN interests or activities.
Seyed A. Fatemi, MD, MBA (Kennedy Krieger Institute, Johns Hopkins Medical Institute) Dr. Fatemi has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for RCO law. The institution of Dr. Fatemi has received research support from Minoryx. The institution of Dr. Fatemi has received research support from Viking Therapeutics. The institution of Dr. Fatemi has received research support from Swanbio Therapeutics . The institution of Dr. Fatemi has received research support from Autobahn Therapeutics. The institution of Dr. Fatemi has received research support from Nuvelution. The institution of Dr. Fatemi has received research support from NIH. The institution of Dr. Fatemi has received research support from A Cure for Ellie Foundation. The institution of Dr. Fatemi has received research support from Brian's Hope Foundation . Dr. Fatemi has received intellectual property interests from a discovery or technology relating to health care. Dr. Fatemi has a non-compensated relationship as a Director (Board member) with ALDConnect that is relevant to AAN interests or activities.
Gerald Raymond, MD (Johns Hopkins) Dr. Raymond has received personal compensation in the range of $50,000-$99,999 for serving as a Consultant for Bluebird bio. Dr. Raymond has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Ionis. Dr. Raymond has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Travere. Dr. Raymond has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant with Dept of HHS.
Stephanie Keller, MD (Children's Healthcare of Atlanta/Emory Universit) Dr. Keller has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Applied Therpeutics. Dr. Keller has received personal compensation in the range of $0-$499 for serving as an officer or member of the Board of Directors for United Leukodystrophy Foundation. The institution of Dr. Keller has received research support from CHOP/NIH. The institution of Dr. Keller has received research support from IONIS. The institution of Dr. Keller has received research support from Biomarin. The institution of Dr. Keller has received research support from Boehring-Ingelheim.
Florian Eichler, MD (Massachusetts General Hospital) An immediate family member of Dr. Eichler has received personal compensation for serving as an employee of UpToDate. Dr. Eichler has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Atlas Venture. Dr. Eichler has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Acadia Pharmaceuticals. Dr. Eichler has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Leal Therapeutics. Dr. Eichler has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Orchard Tx. Dr. Eichler has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Ionis Therapeutics. The institution of Dr. Eichler has received research support from ASPA Therapeutics. The institution of Dr. Eichler has received research support from Abbvie. The institution of Dr. Eichler has received research support from Ionis Pharmaceuticals.
No disclosure on file
Joshua Bonkowsky, MD, PhD Dr. Bonkowsky has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Denali. Dr. Bonkowsky has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Neurogene. Dr. Bonkowsky has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Passage Bio. Dr. Bonkowsky has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Autobahn. Dr. Bonkowsky has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sanofi. Dr. Bonkowsky has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Ionis. Dr. Bonkowsky has stock in Orchard. The institution of Dr. Bonkowsky has received research support from NIH. The institution of Dr. Bonkowsky has received research support from ELA. An immediate family member of Dr. Bonkowsky has received intellectual property interests from a discovery or technology relating to health care. Dr. Bonkowsky has received publishing royalties from a publication relating to health care. Dr. Bonkowsky has received publishing royalties from a publication relating to health care.
Eric J. Mallack, MD (New York Presbyterian Hospital, Cornell, Child Neurology) Dr. Mallack has received research support from NINDS.