Angappan, Dhanalakshmi , Jitendra K. Sahu, Prahbhjot Malhi, Pratibha
Singhi. Safety, tolerability, and effectiveness of oral zonisamide therapy in
comparison with intramuscular adrenocorticotropic hormone therapy in infants
with West syndrome. European Journal of Paediatric Neurology. In press.
Highlights
•First study on Zonisamide versus Adrenocorticotropic
hormone in a randomized controlled trial manner.
•Oral zonisamide had poor efficacy in infants with West
syndrome.
•Oral zonisamide appeared to be safe and tolerable in the
study in infants with West syndrome.
Abstract
West syndrome is a distinct, infantile onset, epileptic
encephalopathy, associated with poor neurodevelopmental outcome. The present
study was designed as a randomized, open-label, pilot study to evaluate the
safety, feasibility, and effectiveness of oral zonisamide therapy in comparison
with adrenocorticotropic hormone therapy in infants with West syndrome. Thirty
infants with West syndrome were randomized to receive treatment with either
synthetic, intramuscular adrenocorticotropic hormone (30-60IU) or oral
zonisamide (4-25mg/kg/day). The study participants had a long treatment lag and
preponderance of male sex (90%). The primary effectiveness outcome measure was
the cessation of epileptic spasms at 2 weeks of initiation of therapy and persistent
till 6 weeks as per West Delphi consensus statement recommendations. Comparison
of efficacies of zonisamide versus adrenocorticotropic hormone was as
following: the cessation of epileptic spasms (27% vs. 40%, p=0.70), resolution
of hypsarrhythmia at 14 days (20% vs. 33%, p=0.68) and resolution of
hypsarrhythmia at 6 weeks (36% vs. 71%, p=0.14). Overall, the study observed a
poor efficacy of both adrenocorticotropic hormone and zonisamide therapy, which
is probably due to long treatment lag and a high proportion of structural
etiology. However, oral zonisamide appeared to be safe and tolerable in the
study.
Courtesy of: https://www.mdlinx.com/journal-summaries/west-syndrome-zonisamide-adrenocorticotropic-hormone/2018/09/25/7545089?spec=neurology
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