Infantile Spasms and West Syndrome – A Clinician’s Perspective

Abstract

Infantile spasms, though long recognised, are still a cause of significant morbidity in children. The knowledge of their etiology and pathogenesis is still evolving. Even the management strategies vary among different centres. Hormonal treatments and vigabatrin have been recognised as effective but controversies prevail on the exact protocols that best balance the risk benefit ratio. Collaborative studies have begun to provide some clarity on some of the management issues but further large scale studies that further standardise protocols are a felt need. This article attempts to provide a clinically relevant summary of the current knowledge.

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References

  1. 1.

    Lux AL, Edwards SW, Hancock E, et al. The United Kingdom Infantile Spasms Study [UKISS] comparing hormone treatment with vigabatrin on developmental and epilepsy outcomes to age 14 months: a multicentre randomised trial. Lancet Neurol. 2005;4:712–7.

  2. 2.

    Hussain SA, Lay J, Cheng E, Weng J, Sankar R, Baca CB. Recognition of infantile spasms is often delayed: the ASSIST study. J Pediatr. 2017;190:215–21.

    Article  Google Scholar 

  3. 3.

    Chellamuthu P, Sharma S, Jain P, Kaushik JS, Seth A, Aneja S. High dose (4 mg/kg/day) versus usual dose (2 mg/kg/day) oral prednisolone for treatment of infantile spasms: an open-label, randomized controlled trial. Epilepsy Res. 2014;108:1378–84.

    CAS  Article  Google Scholar 

  4. 4.

    Osborne JP, Lux AL, Edwards SW, et al. The underlying etiology of infantile spasms (west syndrome): information from the United Kingdom Infantile Spasms Study (UKISS) on contemporary causes and their classification. Epilepsia. 2010;51:2168–74.

  5. 5.

    McTague A, Howell KB, Cross JH, Kurian MA, Scheffer IE. The genetic landscape of the epileptic encephalopathies of infancy and childhood. Lancet Neurol. 2016;15:304–16.

    Article  Google Scholar 

  6. 6.

    Chugani HT. Pathophysiology of infantile spasms. Adv Exp Med Biol. 2002;497:111–21.

    Article  Google Scholar 

  7. 7.

    Donat JF, Wright FS. Unusual variants of infantile spasms. J Child Neurol. 1991;6:313–8.

    CAS  Article  Google Scholar 

  8. 8.

    Donat JF, Wright FS. Seizures in series: similarities between seizures of the west and Lennox-Gastaut syndromes. Epilepsia. 1991;32:504–9.

    CAS  Article  Google Scholar 

  9. 9.

    Lux AL, Osborne JP. A proposal for case definitions and outcome measures in studies of infantile spasms and west syndrome: consensus statement of the West Delphi group. Epilepsia. 2004;45:1416–28.

    Article  Google Scholar 

  10. 10.

    Fois A. Infantile spasms: review of the literature and personal experience. Ital J Pediatr. 2010;36:15.

    Article  Google Scholar 

  11. 11.

    Wilmshurst JM, Ibekwe RC, O'Callaghan FJK. Epileptic spasms - 175 years on: trying to teach an old dog new tricks. Seizure. 2017;44:81–6.

    Article  Google Scholar 

  12. 12.

    Hussain SA, Shinnar S, Kwong G, et al. Treatment of infantile spasms with very high dose prednisolone before high dose adrenocorticotropic hormone. Epilepsia. 2014;55:103–7.

    CAS  Article  Google Scholar 

  13. 13.

    Ito M, Hashimoto K, Koroki S, et al. Low dose ACTH therapy for west syndrome. Initial effect and long term outcome. Neurology. 2002;58:110–4.

    CAS  Article  Google Scholar 

  14. 14.

    Oguni H, Yanagaki S, Hayashi K, et al. Extremely low dose ACTH step-up protocol for west syndrome: maximum therapeutic effect with minimal side effects. Brain Dev. 2006;28:8–13.

  15. 15.

    Mitchell WG, Shah NS. Vigabatrin for infantile spasms. Pediatr Neurol. 2002;27:161–4.

    Article  Google Scholar 

  16. 16.

    Buncic JR, Westall CA, Panton CM, Munn JR, MacKeen LD, Logan WJ. Characteristic retinal atrophy with secondary "inverse" optic atrophy identifies vigabatrin toxicity in children. Ophthalmology. 2004;111:1935–42.

    Article  Google Scholar 

  17. 17.

    Hammoudi DS, Lee SS, Madison A, et al. Reduced visual function associated with infantile spasms in children with vigabatrin therapy. Invest Ophtalmol Vis Sci. 2005;46:514–20.

    Article  Google Scholar 

  18. 18.

    O’Callaghan FJK, Edwards SW, Alber FD, et al; International Collaborative Infantile Spasms Study (ICISS) investigators. Vigabatrin with hormonal treatment versus hormonal treatment alone (ICISS) for infantile spasms: 18-month outcomes of an open-label, randomised controlled trial. Lancet Child Adolesc Health. 2018;2:715–25.

  19. 19.

    Dressler A, Benninger F, Trimmel-Schwahofer P, et al. Efficacy and tolerability of the ketogenic diet versus high-dose adrenocorticotropic hormone for infantile spasms: a single-center parallel-cohort randomized controlled trial. Epilepsia. 2019;60:441–51.

    CAS  Article  Google Scholar 

  20. 20.

    Sharma S, Sankhyan N, Gulati S, Agarwala A. Use of the modified Atkins diet in infantile spasms refractory to first-line treatment. Seizure. 2012;21:45–8.

    Article  Google Scholar 

  21. 21.

    Asano E, Chugani DC, Juhász C, Muzik O, Chugani HT. Surgical treatment of west syndrome. Brain Dev. 2001;23:668–76.

  22. 22.

    Korinthenberg R, Schreiner A. Topiramate in children with west syndrome: a retrospective multicenter evaluation of 100 patients. J Child Neurol. 2007;22:302–6.

    Article  Google Scholar 

  23. 23.

    Chandra S, Bhave A, Bhargava R, Kumar C, Kumar R. West syndrome: response to valproate. Front Neurol. 2012;3:166.

    Article  Google Scholar 

  24. 24.

    Suzuki Y. Zonisamide in West syndrome. Brain Dev. 2001;23:658–61.

  25. 25.

    Chamberlain MC. Nitrazepam for refractory infantile spasms and the Lennox-Gastaut syndrome. J Child Neurol. 1996;11:31–4.

    CAS  Article  Google Scholar 

  26. 26.

    Hussain SA, Zhou R, Jacobson C, et al. Perceived efficacy of cannabidiol-enriched cannabis extracts for treatment of pediatric epilepsy: a potential role for infantile spasms and Lennox-Gastaut syndrome. Epilepsy Behav. 2015;47:138–41.

    Article  Google Scholar 

  27. 27.

    Chen JW, Borgelt LM, Blackmer AB. Cannabidiol: a new hope for patients with Dravet or Lennox-Gastaut syndromes. Ann Pharmacother. 2019;53:603–11.

    CAS  Article  Google Scholar 

  28. 28.

    Toribe Y. High dose vitamin B(6) treatment in west syndrome. Brain Dev. 2001;23:654–7.

  29. 29.

    Kuo MF, Wang HS. Pyridoxal phosphate response in epilepsy with resistance to pyridoxine. Pediatr Neurol. 2002;26:146–7.

    Article  Google Scholar 

  30. 30.

    O’Callaghan FJ, Lux AL, Darke K, et al. The effect of lead time to treatment and of age of onset on developmental outcome at 4 years in infantile spasms: evidence from the United Kingdom Infantile Spasms Study. Epilepsia. 2011;52:1359–64.

  31. 31.

    Jóźwiak S, Kotulska K, Domańska-Pakieła D, et al. Antiepileptic treatment before the onset of seizures reduces epilepsy severity and risk of mental retardation in infants with tuberous sclerosis complex. Eur J Paediatr Neurol. 2011;15:424–31.

    Article  Google Scholar 

  32. 32.

    Results of the International EPISTOP Research. Available at: https://www.iimcb.gov.pl/en/press-office/news/news/970-results-of-the-international-epistop-research. Accessed 15th Aug 2019.

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Dr Shyam Kukreja, Director and Head of Department of Pediatrics, Max Super Speciality Hospital, Patparganj, New Delhi.

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Correspondence to Saurabh S. Chopra.

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Chopra, S.S. Infantile Spasms and West Syndrome – A Clinician’s Perspective. Indian J Pediatr (2020). https://doi.org/10.1007/s12098-020-03279-y

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Keywords

  • Infantile spasms
  • West syndrome