West Syndrome: A Review and Guide for Paediatricians

Clin Drug Investig. 2018 Feb;38(2):113-124. doi: 10.1007/s40261-017-0595-z.

Abstract

West syndrome (WS), also known as infantile spasms, occurs in infancy with a peak between 4 and 7 months. Spasms, neurodevelopmental regression and hypsarrhythmia on electroencephalogram (EEG) basically define WS. The International League Against Epilepsy commission classifies the aetiologies of WS into genetic, structural, metabolic and unknown. Early diagnosis and a shorter lag time to treatment are essential for the overall outcome of WS patients. These goals are feasible with the addition of brain magnetic resonance imaging (MRI) and genetic and metabolic testing. The present work analysed the medical literature on WS and reports the principal therapeutic protocols of its management. Adrenocorticotropic hormone (ACTH), vigabatrin (VGB) and corticosteroids are the first-line treatments for WS. There is no unique therapeutic protocol for ACTH, but most of the evidence suggests that low doses are as effective as high doses for short-term treatment, which is generally 2 weeks followed by dose tapering. VGB is generally administered at doses from 50 to 150 mg/kg/day, but its related retinal toxicity, which occurs in 21-34% of infants, is most frequently observed when treatment periods last longer than 6 months. Among corticosteroids, a treatment of 14 days of oral prednisolone (40-60 mg/day) has been considered effective and well tolerated. Considering that an early diagnosis and a shorter lag time to treatment are essential for successful outcomes in these patients, further studies on efficacy of the different therapeutic approaches with evaluation of final outcome after cessation of therapy are needed.

Publication types

  • Review

MeSH terms

  • Adrenal Cortex Hormones / pharmacology
  • Adrenal Cortex Hormones / therapeutic use
  • Adrenocorticotropic Hormone / pharmacology
  • Adrenocorticotropic Hormone / therapeutic use
  • Anticonvulsants / pharmacology
  • Anticonvulsants / therapeutic use
  • Clinical Trials as Topic / methods
  • Electroencephalography / drug effects
  • Electroencephalography / methods
  • Female
  • Humans
  • Infant
  • Magnetic Resonance Imaging / methods
  • Male
  • Pediatricians / standards*
  • Practice Guidelines as Topic / standards*
  • Spasms, Infantile / diagnostic imaging*
  • Spasms, Infantile / drug therapy*
  • Spasms, Infantile / physiopathology
  • Treatment Outcome
  • Vigabatrin / pharmacology
  • Vigabatrin / therapeutic use

Substances

  • Adrenal Cortex Hormones
  • Anticonvulsants
  • Adrenocorticotropic Hormone
  • Vigabatrin