Long-term Cognitive Outcomes of a Cohort of Children With Cryptogenic Infantile Spasms Treated With High-Dose Adrenocorticotropic Hormone

Epilepsia. 2004 Mar;45(3):255-62. doi: 10.1111/j.0013-9580.2004.30503.x.

Abstract

Purpose: To evaluate the outcome of children with cryptogenic infantile spasms treated with high-dose synthetic adrenocorticotropic hormone (ACTH) and the relation between early treatment, within 1 month of onset, and outcome.

Methods: We assessed the long-term cognitive and seizure outcomes of 37 patients with cryptogenic infantile spasms (onset, age 3 to 9 months) receiving standardized treatment regimen of high-dose tetracosactide depot, 1 mg IM every 48 h for 2 weeks, with a subsequent 8- to 10-week slow taper and followed by oral prednisone, 10 mg/day for a month, with a subsequent slow taper for 5 months or until the infant reached the age of 1 year, whichever came later. Development was assessed before treatment. Seizure outcomes were followed up prospectively. Cognitive outcomes were determined after 6 to 21 years and analyzed in relation to treatment lag and pretreatment regression.

Results: Twenty-two infants were treated within 1 month of onset of infantile spasms, and 15 after 1 to 6.5 months. Normal cognitive outcome was found in all 22 (100%) patients of the early-treatment group, and in 40% of the late-treatment group. Normal cognitive outcome was found in all 25 (100%) patients who had no or only mild mental deterioration at presentation, including four in the late-treatment group but in only three of the 12 patients who had had marked or severe deterioration before treatment.

Conclusions: Early treatment of cryptogenic infantile spasms with a high-dose ACTH protocol is associated with favorable long-term cognitive outcomes. Once major developmental regression lasts for a month or more, the prognosis for normal cognitive outcome is poor. Further studies are needed on the optimal treatment regimen for this disorder.

MeSH terms

  • Administration, Oral
  • Brain / pathology
  • Child
  • Child, Preschool
  • Cognition Disorders / diagnosis*
  • Cognition Disorders / etiology
  • Cohort Studies
  • Cosyntropin / administration & dosage
  • Cosyntropin / therapeutic use*
  • Delayed-Action Preparations
  • Dose-Response Relationship, Drug
  • Drug Administration Schedule
  • Female
  • Humans
  • Infant, Newborn
  • Magnetic Resonance Imaging
  • Male
  • Neuropsychological Tests
  • Prednisone / therapeutic use
  • Prospective Studies
  • Severity of Illness Index
  • Spasms, Infantile / complications
  • Spasms, Infantile / diagnosis
  • Spasms, Infantile / drug therapy*
  • Time Factors
  • Treatment Outcome

Substances

  • Delayed-Action Preparations
  • Cosyntropin
  • Prednisone