A survey of antiepileptic drug responses identifies drugs with potential efficacy for seizure control in Wolf–Hirschhorn syndrome

Seizures are present in over 90% of infants and children with Wolf–Hirschhorn syndrome (WHS). When present, they significantly affect quality of life. The goal of this study was to use caregiver reports to describe the comparative efficacies of commonly used antiepileptic medications in a large popu...

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Bibliographic Details
Published in:Epilepsy & behavior 2018-04, Vol.81, p.55-61
Main Authors: Ho, Karen S., Markham, Leah M., Twede, Hope, Lortz, Amanda, Olson, Lenora M., Sheng, Xiaoming, Weng, Cindy, Wassman, E. Robert, Newcomb, Tara, Carey, John C., Battaglia, Agatino
Format: Article
Language:English
Subjects:
4p16.3 deletion
Adolescent
Adult
Anticonvulsant
Anticonvulsants - therapeutic use
Carbamazepine
Carbamazepine - therapeutic use
Child
Child, Preschool
Clobazam
Clobazam - therapeutic use
Female
Humans
Infant
Lamotrigine - therapeutic use
Levetiracetam
Levetiracetam - therapeutic use
Male
Middle Aged
Oxcarbazepine - therapeutic use
Phenobarbital - therapeutic use
Phenytoin - therapeutic use
Quality of Life
Seizure treatments
Seizures - drug therapy
Topiramate - therapeutic use
Wolf-Hirschhorn Syndrome - drug therapy
Young Adult
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Summary:Seizures are present in over 90% of infants and children with Wolf–Hirschhorn syndrome (WHS). When present, they significantly affect quality of life. The goal of this study was to use caregiver reports to describe the comparative efficacies of commonly used antiepileptic medications in a large population of individuals with WHS. A web-based, confidential caregiver survey was developed to capture seizure semiology and a chronologic record of seizure treatments as well as responses to each treatment. Adverse events for each drug were also cataloged. We received 141 complete survey responses (47% response rate) describing the seizures of individuals ranging in age from 4months to 61years (90 females: 51 males). Using the Early Childhood Epilepsy Severity Scale (E-Chess), WHS-associated seizures are demonstrably severe regardless of deletion size. The best-performing antiepileptic drugs (AEDs) for controlling seizures in this cohort were broad spectrum drugs clobazam, levetiracetam, and lamotrigine; whereas, the three commonly used carboxamide class drugs: carbamazepine, phenytoin, and oxcarbazepine, were reported to have little effect on, or even exacerbate, seizures. The carboxamide class drugs, along with phenobarbital and topiramate, were also associated with the highest rate of intolerance due to cooccurrence of adverse events. Levetiracetam, clobazam, and clonazepam demonstrated higher tolerability and comparatively less severe adverse events (Wilcoxon rank sum comparison between performance of levetiracetam and carboxamide class drugs gives a p
ISSN:1525-5050
1525-5069
DOI:10.1016/j.yebeh.2017.12.008