Abstract
Objectives: To assess efficacy of very high dose (8mg/kg/day, maximum 60mg/day) prednisolone in patients with West syndrome. Method: This was an observational study conducted at a tertiary level hospital from August 2014 to August 2015. Children, aged 2 to 23 months, presenting with infantile spasms with hypsarrhythmia or its EEG variants, were enrolled. Study participants were started on very high dose prednisolone (8 mg/kg/day, maximum 60mg/day). The primary outcome measure was complete cessation of spasms and clearance of hypsarrhythmia on EEG after 2 weeks of prednisolone treatment. The study was approved by the institutional ethical committee. Results: Forty children were started on very high dose prednisolone (8 mg/kg/day, maximum 60mg/day) of whom four did not come for the 2 week follow up and were excluded. Response rate in the remaining 36 patients was 55.6% after 2 weeks of steroid therapy Side effects seen during hormone therapy included increased appetite in 29 (80.6%) patients, irritability in 27 (75.0%) patients and weight gain in 26 (72.2%) patients. Conclusions: Use of a very high dose prednisolone regime (8mg/kg/day, maximum 60mg/day) resulted in a complete cessation of spasms and clearance of hypsarrhythmia on EEG after 2 weeks in 55.6% of children with West syndrome in this study. Sri Lanka Journal of Child Health , 2018; 47 (2): 137-140
Highlights
West syndrome comprises infantile spasms, a characteristic electroencephalogram (EEG) appearance plus frequent neurodevelopmental delay or regression[1]
Study participants were started on very high dose prednisolone (8 mg/kg/day, maximum 60mg/day)
Forty children were started on very high dose prednisolone (8 mg/kg/day, maximum 60mg/day) of whom four did not come for the 2 week follow up and were excluded
Summary
West syndrome comprises infantile spasms, a characteristic electroencephalogram (EEG) appearance (hypsarrhythmia and its variants) plus frequent neurodevelopmental delay or regression[1]. Intramuscular (IM) adrenocorticotropic hormone (ACTH) and oral prednisolone are commonly used in the treatment of infantile spasms[2,3,4,5,6]. There is no consensus on the role of oral steroids as first line therapy of infantile spasms[7]. Limitations like high cost, pain associated with IM injections and non-availability of skilled personnel to administer injections to young children precludes ACTH use in resource limited settings. Prednisolone is an inexpensive and available alternative in such settings. High dose prednisolone has been shown to be effective in infantile spasms[8, 9]
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