Treatment with High-Dose Prednisolone in Vigabatrin-Refractory Infantile Spasms

Can J Neurol Sci. 2022 Jul;49(4):532-539. doi: 10.1017/cjn.2021.156. Epub 2021 Jul 2.

Abstract

Objectives: This research aimed to study the short-term seizure outcomes following treatment with 8 mg/kg/day prednisolone in children with infantile spasms (IS) refractory to vigabatrin. We hypothesized that high-dose prednisolone may result in similar rates of electroclinical remission when compared to published ACTH rates.

Methods: All consecutive children with hypsarrhythmia or hypsarrhythmia variant on EEG with/without IS, who had been treated with vigabatrin as first-line anti-seizure medication (ASM) followed by high-dose oral prednisolone (8 mg/kg/day; maximum 60 mg/day) in cases who did not respond to vigabatrin, were included. Clinical and electroclinical response (ECR) at 2 weeks following initiation of treatment and adverse effects were assessed.

Results: Sixty-five children were included. A genetic etiology was seen in 38.5% cases. Complete ECR was seen in 30.8% (20/65) of the patients 2 weeks after vigabatrin. Complete ECR was noted in 77.8% (35/45) of the patients, 2 weeks after prednisolone initiation in children who failed vigabatrin, and this was sustained at 6 weeks in 66.7% (30/45) patients. Prednisolone was generally well tolerated.

Conclusions: High-dose (8 mg/kg/day) oral prednisolone resulted in sustained complete ECR (at 6 weeks) in two-thirds of the children with hypsarrhythmia or hypsarrhythmia variant on EEG with/without parentally reported IS. It was generally well tolerated and found to be safe.

Keywords: Epilepsy surgery; Epileptic encephalopathy; Genetic epilepsy; Hemispherectomy; Hormonal; Infantile spasms; KCNQ2; Ketogenic diet; Prednisolone; Steroids.

MeSH terms

  • Anticonvulsants / therapeutic use
  • Child
  • Humans
  • Infant
  • Prednisolone / adverse effects
  • Prednisolone / therapeutic use
  • Spasms, Infantile* / drug therapy
  • Treatment Outcome
  • Vigabatrin* / therapeutic use

Substances

  • Anticonvulsants
  • Prednisolone
  • Vigabatrin