Adrenocorticotropic hormone versus pulsatile dexamethasone in the treatment of infantile epilepsy syndromes

Pediatr Neurol. 2010 Jan;42(1):21-7. doi: 10.1016/j.pediatrneurol.2009.07.011.

Abstract

For treatment of intractable epilepsies, there are no data comparing conventional adrenocorticotropic hormone and pulsatile corticoid therapy with dexamethasone. A retrospective comparison of efficacy was therefore conducted for both forms of application. Between 1989 and 2001, a series of 11 children with West syndrome and 3 with Lennox-Gastaut syndrome were treated with adrenocorticotropic hormone (group 1); between 2003 and 2006, 7 children with West syndrome, 5 with electrical status epilepticus during slow sleep, and 2 with Lennox-Gastaut syndrome were treated with pulsatile corticoid therapy (group 2). In group 1 (n = 14), 9/11 West syndrome patients became seizure free, but none with Lennox-Gastaut syndrome (0/3). In group 2 (n = 14), 4/7 West syndrome patients became seizure-free, 1/2 with Lennox-Gastaut syndrome exhibited seizure-frequency reduction, and 2/5 patients with electrical status epilepticus during slow-wave sleep exhibited significant improvement according to electroencephalograms. In West syndrome, pulsatile corticoid therapy was an effective alternative treatment to adrenocorticotropic hormone, whereas in Lennox-Gastaut syndrome in general steroids did not lead to a significant seizure reduction. In electrical status epilepticus during slow-wave sleep, treatment with pulsatile corticoid therapy seems to be effective and should be investigated in a larger group of patients.

Publication types

  • Comparative Study

MeSH terms

  • Adrenocorticotropic Hormone / administration & dosage
  • Adrenocorticotropic Hormone / adverse effects
  • Adrenocorticotropic Hormone / therapeutic use*
  • Anticonvulsants / administration & dosage
  • Anticonvulsants / adverse effects
  • Anticonvulsants / therapeutic use*
  • Brain / physiopathology
  • Child
  • Child, Preschool
  • Dexamethasone / administration & dosage
  • Dexamethasone / adverse effects
  • Dexamethasone / therapeutic use*
  • Electroencephalography
  • Epilepsy / drug therapy*
  • Epilepsy / physiopathology
  • Female
  • Humans
  • Infant
  • Male
  • Periodicity
  • Retrospective Studies
  • Seizures / drug therapy
  • Seizures / physiopathology
  • Sleep Stages / physiology
  • Spasms, Infantile / drug therapy*
  • Spasms, Infantile / physiopathology
  • Status Epilepticus / drug therapy*
  • Status Epilepticus / physiopathology
  • Syndrome
  • Treatment Outcome

Substances

  • Anticonvulsants
  • Dexamethasone
  • Adrenocorticotropic Hormone