Skip to main content

Advertisement

Log in

Treatment of infantile spasms

  • Published:
Current Treatment Options in Neurology Aims and scope Submit manuscript

Opinion statement

Infantile spasms are associated with a diverse range of conditions, and treatment options are available. However, outcomes remain generally poor, particularly for those with symptomatic etiologies. First-line therapy is considered to be hormonal (adrenocorticotropic hormone; ACTH), which some evidence suggests is more effective when started early. However, side effects may place limits on its use acutely and long-term. There is additional evidence for vigabatrin, specifically for infantile spasms secondary to tuberous sclerosis complex. In refractory cases, candidacy for surgical management should be explored, along with new-generation anticonvulsants (eg, topiramate, zonisamide) and the ketogenic diet. There is urgent need for further treatment trials comparing anticonvulsants with ACTH and a satisfactory animal model for the study of spasms.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References and Recommended Reading

  1. West WJ: On a peculiar form of infantile convulsions. Lancet 1841, i:724–735.

    Article  Google Scholar 

  2. Niedermeyer E, Lopes da Silva FH: Electroencephalography: Basic Principles, Clinical Applications, and Related Fields, edn 5. Baltimore, MD: Lippincott Williams & Wilkins; 2005:538–542.

    Google Scholar 

  3. Hrachovy RA, Frost JD Jr: Infantile epileptic encephalopathy with hypsarrhythmia (infantile spasms/West syndrome). J Clin Neurophysiol 2003, 20:408–425. Historical and clinical overview of IS.

    Article  PubMed  Google Scholar 

  4. Frost JD, Hrachovy RA: Pathogenesis of infantile spasms: a model based on developmental desynchronization. J Clin Neurophysiol 2005, 22:25–36. This article reviews the known pathogenesis of IS, with attention to a model of temporal desynchronization of brain maturation caused by at least two brain insults.

    Article  PubMed  Google Scholar 

  5. Mikati MA, Lepejian GA, Holmes GL: Medical treatment of patients with infantile spasms. Clin Neuropharmacol 2002, 25:61–70.

    Article  PubMed  CAS  Google Scholar 

  6. Lado FA, Moshe SL: Role of subcortical structures in the pathogenesis of infantile spasms: what are possible subcortical mediators? Int Rev Neurobiol 2002, 49:115–140.

    PubMed  CAS  Google Scholar 

  7. Rho JM: Basic science behind the catastrophic epilepsies. Epilepsia 2004, 45(Suppl):5–11. A useful discussion of three theories on the pathogenesis of the catastrophic epilepsies.

    Article  PubMed  Google Scholar 

  8. Avanzini G, Panzica F, Franceschetti S: Brain maturational aspects relevant to pathophysiology of infantile spasms. Int Rev Neurobiol 2002, 49:353–365.

    PubMed  CAS  Google Scholar 

  9. Stafstrom CE, Holmes GL: Infantile spasms: criteria for an animal model. Int Rev Neurobiol 2002, 49:391–411.

    PubMed  Google Scholar 

  10. Mackay MT: Practice parameter: medical treatment of infantile spasms. Neurology 2004, 62:1668–1681. Essential evidence-based review and guidelines concerning the available data for medical treatments of IS according to the AAN.

    PubMed  CAS  Google Scholar 

  11. Gupta R: Corticosteroids in the management of paediatric epilepsies. Arch Dis Child 2005, 90:279–384. A very good summary of the often conflicting studies, potential mechanisms of action, and applications of steroids in pediatric epilepsies, including spasms.

    Article  Google Scholar 

  12. Riikonen R: The latest on infantile spasms. Curr Opin Neurol 2005, 18:91–95. An excellent review of current treatments and outcomes that incorporates the AAN review and the European literature.

    Article  PubMed  Google Scholar 

  13. Kondo Y, Okumura A, Watanabe K, et al.: Comparison of two low dose ACTH therapies for West syndrome: their efficacy and side effects. Brain Dev 2005, 27:326–330.

    Article  PubMed  Google Scholar 

  14. Wheless JW: Nonpharmacologic treatment of the catastrophic epilepsies of childhood. Epilepsia 2004, 45(Suppl):17–22. Reviews surgical treatment and use of the ketogenic diet in the catastrophic epilepsies.

    Article  PubMed  Google Scholar 

  15. Kossoff EH, Pyzik PL, McGrogan JR, et al.: Efficacy of the ketogenic diet for infantile spasms. Pediatrics 2002, 9:780–783.

    Article  Google Scholar 

  16. Shields DW: Medical versus surgical treatment: which treatment when. Int Rev Neurobiol 2002, 49:253–267.

    Article  PubMed  Google Scholar 

  17. Riikonen R: Infantile spasms: therapy and outcome. J Child Neurol 2004, 19:401–404.

    PubMed  Google Scholar 

  18. Kivity S, Lerman P, Ariel R, et al.: Long-term cognitive outcomes of a cohort of children with cryptogenic infantile spasms treated with high dose adrenocorticotropic hormone. Epilepsia 2004, 45:255–262.

    Article  PubMed  CAS  Google Scholar 

  19. Capovilla G, Beccaria F, Montagnini A, et al.: Short-term nonhormonal and nonsteroid treatment in West syndrome. Epilepsia 2003, 44:1085–1088.

    Article  PubMed  CAS  Google Scholar 

  20. Baram TZ, Mitchell WG, Tournay A, et al.: High-dose corticotropin (ACTH) versus prednisone for infantile spasms: a prospective, randomized, blinded study. Pediatrics 1996, 97:375–379.

    PubMed  CAS  Google Scholar 

  21. Hrachovy RA, Frost JD Jr, Glaze DG: High-dose, longduration versus low-dose, short-duration corticotropin therapy for infantile spasms. J Pediatr 1994, 124:803–806.

    Article  PubMed  CAS  Google Scholar 

  22. Lux AL, Edwards SW, Hancock E, et al.: The United Kingdom Infantile Spasm Study comparing vigabatrin with prednisolone or tetracosactide at 14 days: a multicenter, randomised controlled trial. Lancet 2004, 364:1773–1778.

    Article  PubMed  CAS  Google Scholar 

  23. Appleton RE, Peters AC, Mumford JP, Shaw DE: Randomised, placebo-controlled study of vigabatrin as first-line treatment of infantile spasms. Epilepsia 1999, 40:1627–1633.

    Article  PubMed  CAS  Google Scholar 

  24. Spencer EL, Harding GF: Examining visual field defects in the paediatric population exposed to vigabatrin. Documenta Ophthalmologica 2003, 107:281–287.

    Article  PubMed  CAS  Google Scholar 

  25. Hosain SA, Merchant S, Solomon GE, Chutorian A: Topiramate for the treatment of infantile spasms. J Child Neurol 2006, 21:17–19.

    PubMed  Google Scholar 

  26. Grosso S, Galimberti D, Farnetani MA, et al.: Efficacy and safety of topiramate in infants according to epilepsy syndromes. Seizure 2005, 14:183–189.

    Article  PubMed  CAS  Google Scholar 

  27. Hachad H, Ragueneau-Majlessi I, Levy RH: New antiepileptic drugs: review of drug interactions. Ther Drug Monit 2002, 24:91–103.

    Article  PubMed  CAS  Google Scholar 

  28. Lotze TE, Wilfong AA: Zonisamide treatment for symptomatic infantile spasms. Neurology 2004, 62:296–298.

    PubMed  CAS  Google Scholar 

  29. Yanagaki S, Oguni H, Yoshii K, et al.: Zonisamide for West syndrome: a comparison of clinical responses among different titration rates. Brain Dev 2005, 27:286–290.

    Article  PubMed  Google Scholar 

  30. Fisher E, Siemes H, Pund R: Valproate metabolites in serum and urine during antiepileptic therapy in children with infantile spasms: abnormal metabolite pattern associated with reversible hepatotoxicity. Epilepsia 1992, 33:165–171.

    Article  PubMed  CAS  Google Scholar 

  31. Siemes H, Spohr HL, Michael T, Nau H: Therapy of infantile spasms with valproate: results of a prospective study. Epilepsia 1988, 29:553–560.

    PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Overby, P.J., Kossoff, E.H. Treatment of infantile spasms. Curr Treat Options Neurol 8, 457–464 (2006). https://doi.org/10.1007/s11940-006-0035-5

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11940-006-0035-5

Keywords

Navigation