eMedicine Specialties > Neurology > Pediatric Neurology

Infantile Spasm (West Syndrome): Follow-up

Author: Tracy A Glauser, MD, Professor, Departments of Pediatrics and Neurology, University of Cincinnati College of Medicine, Children's Comprehensive Epilepsy Program, Children's Hospital Medical Center of Cincinnati
Coauthor(s): Diego A Morita, MD, Assistant Professor of Pediatrics and Neurology, Department of Pediatrics, Division of Neurology, Cincinnati Children's Hospital Medical Center, University of Cincinnati
Contributor Information and Disclosures

Updated: Apr 10, 2006

Follow-up

Complications

  • Complications include dose-related, idiosyncratic, or long-term adverse effects from medications, including death.

Prognosis

  • The long-term overall prognosis is poor and is related directly to the etiology.
    • Infants with idiopathic West syndrome have a better prognosis than do infants with symptomatic West syndrome. Only 14% of infants with symptomatic West syndrome have normal or borderline normal cognitive development, compared to 28-50% of infants with idiopathic West syndrome. Mental retardation is severe in 70% of patients, often with psychiatric problems such as autistic features or hyperactivity. Infrequently spasms may persist in adulthood. Fifty to seventy percent of patients develop other seizure types. Eighteen to fifty percent of patients will develop Lennox Gastaut syndrome.
    • Subsets of patients among the symptomatic West syndrome group seem to have a better prognosis. A retrospective study of 17 children with trisomy 21 and infantile spasms found that 13 of 16 survivors were seizure free for more than 1 year and 10 no longer were taking anticonvulsants.
    • A study of 15 children with neurofibromatosis type 1 and infantile spasms also reported a relatively benign seizure and cognitive outcome.
    • Prognosis appears to be worse in infants with other seizure types, persistent EEG abnormalities, poor response to ACTH, and delayed initiation of treatment. One study showed that later onset, normal-to-mild psychomotor delay at the time of diagnosis, and good seizure control were factors related to better prognosis.

Miscellaneous

Medicolegal Pitfalls

  • Failure to inform the patient's family of the risk for severe adverse effects, including death, from the use of either ACTH or oral steroids
  • Failure to inform the patient's family of the risk for severe idiosyncratic reactions from two commonly used antiepileptic medications for West syndrome
    • Valproate - Hepatotoxicity, pancreatitis
    • Lamotrigine - Steven-Johnson syndrome, toxic epidermal necrolysis
  • Failure to inform the patient's family of signs and symptoms to watch for that indicate severe adverse effects or idiosyncratic reactions
  • Failure to instruct the family on what to do if they notice signs and symptoms indicating severe adverse effects or idiosyncratic reactions
  • Failure to fully investigate and identify possible causes of the patient's West syndrome, including identification of tuberous sclerosis (a common cause of West syndrome), which can have implications for the entire family
  • Failure to recognize signs and symptoms of West syndrome, which could result in failure to select an appropriate AED with proven efficacy; this could increase the risk for uncontrolled seizures that in turn increase the risk for injury and death
 


More on Infantile Spasm (West Syndrome)

Overview: Infantile Spasm (West Syndrome)
Differential Diagnoses & Workup: Infantile Spasm (West Syndrome)
Treatment & Medication: Infantile Spasm (West Syndrome)
Follow-up: Infantile Spasm (West Syndrome)
Multimedia: Infantile Spasm (West Syndrome)
References
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References

  1. Aicardi J, Mumford JP, Dumas C, et al. Vigabatrin as initial therapy for infantile spasms: a European retrospective survey. Sabril IS Investigator and Peer Review Groups. Epilepsia. Jul 1996;37(7):638-42. [Medline].

  2. Appleton RE, Peters AC, Mumford JP, et al. Randomised, placebo-controlled study of vigabatrin as first-line treatment of infantile spasms. Epilepsia. Nov 1999;40(11):1627-33. [Medline].

  3. Asarnow RF, LoPresti C, Guthrie D, et al. Developmental outcomes in children receiving resection surgery for medically intractable infantile spasms. Dev Med Child Neurol. Jul 1997;39(7):430-40. [Medline].

  4. Baram TZ, Mitchell WG, Tournay A, et al. High-dose corticotropin (ACTH) versus prednisone for infantile spasms: a prospective, randomized, blinded study. Pediatrics. Mar 1996;97(3):375-9. [Medline].

  5. Blennow G, Starck L. High dose B6 treatment in infantile spasms. Neuropediatrics. Feb 1986;17(1):7-10. [Medline].

  6. Bobele GB, Bodensteiner JB. The treatment of infantile spasms by child neurologists. J Child Neurol. Oct 1994;9(4):432-5. [Medline].

  7. Chugani HT. Infantile spasms. Curr Opin Neurol. Apr 1995;8(2):139-44. [Medline].

  8. Cianchetti C, Pruna D, Coppola G. Low-dose lamotrigine in West syndrome. Epilepsy Res. Sep 2002;51(1-2):199-200. [Medline].

  9. Commission on Classification and Terminology of the International League Agains. Proposal for revised classification of epilepsies and epileptic syndromes. Commission on Classification and Terminology of the International League Against Epilepsy. Epilepsia. Jul-Aug 1989;30(4):389-99. [Medline].

  10. Czochanska J, Langner-Tyszka B, Losiowski Z, et al. Children who develop epilepsy in the first year of life: a prospective study. Dev Med Child Neurol. Apr 1994;36(4):345-50. [Medline].

  11. Debus OM, Kurlemann G. Sulthiame in the primary therapy of West syndrome: a randomized double-blind placebo-controlled add-on trial on baseline pyridoxine medication. Epilepsia. Feb 2004;45(2):103-8. [Medline].

  12. Donat JF, Lo WD. Asymmetric hypsarrhythmia and infantile spasms in west syndrome. J Child Neurol. Jul 1994;9(3):290-6. [Medline].

  13. Drury I, Beydoun A, Garofalo EA, et al. Asymmetric hypsarrhythmia: clinical electroencephalographic and radiological findings. Epilepsia. Jan 1995;36(1):41-7. [Medline].

  14. Eisermann MM, DeLaRaillère A, Dellatolas G. Infantile spasms in Down syndrome--effects of delayed anticonvulsive treatment. Epilepsy Res. Jun-Jul 2003;55(1-2):21-7. [Medline].

  15. Fejerman N, Cersosimo R, Caraballo R, et al. Vigabatrin as a first-choice drug in the treatment of West syndrome. J Child Neurol. Mar 2000;15(3):161-5. [Medline].

  16. Glauser TA, Clark PO, Strawsburg R. A pilot study of topiramate in the treatment of infantile spasms. Epilepsia. Dec 1998;39(12):1324-8. [Medline].

  17. Glauser TA. Pediatric epilepsy syndromes. Curr Opin Pediatr. Dec 1995;7(6):640-9. [Medline].

  18. Glauser TA. Following catastrophic epilepsy patients from childhood to adulthood. Epilepsia. 2004;45 Suppl 5:23-6. [Medline].

  19. Goh S, Kwiatkowski DJ, Dorer DJ. Infantile spasms and intellectual outcomes in children with tuberous sclerosis complex. Neurology. Jul 26 2005;65(2):235-8. [Medline].

  20. Hammoudi DS, Lee SS, Madison A. Reduced visual function associated with infantile spasms in children on vigabatrin therapy. Invest Ophthalmol Vis Sci. Feb 2005;46(2):514-20. [Medline].

  21. Hancock E, Osborne JP. Vigabatrin in the treatment of infantile spasms in tuberous sclerosis: literature review. J Child Neurol. Feb 1999;14(2):71-4. [Medline].

  22. Hrachovy RA, Frost JD Jr, Kellaway P. Hypsarrhythmia: variations on the theme. Epilepsia. Jun 1984;25(3):317-25. [Medline].

  23. Hrachovy RA, Frost JD Jr, Kellaway P, et al. Double-blind study of ACTH vs prednisone therapy in infantile spasms. J Pediatr. Oct 1983;103(4):641-5. [Medline].

  24. Ito M. Antiepileptic drug treatment of West syndrome. Epilepsia. 1998;39 Suppl 5:38-41. [Medline].

  25. Ito M, Seki T, Takuma Y. Current therapy for West syndrome in Japan. J Child Neurol. Jun 2000;15(6):424-8. [Medline].

  26. Kivity S, Lerman P, Ariel R. Long-term cognitive outcomes of a cohort of children with cryptogenic infantile spasms treated with high-dose adrenocorticotropic hormone. Epilepsia. Mar 2004;45(3):255-62. [Medline].

  27. Kondo Y, Okumura A, Watanabe K. Comparison of two low dose ACTH therapies for West syndrome: their efficacy and side effect. Brain Dev. Aug 2005;27(5):326-30. [Medline].

  28. Lux AL, Edwards SW, Hancock E. 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. Nov 2005;4(11):712-7. [Medline].

  29. Lux AL, Edwards SW, Hancock E. The United Kingdom Infantile Spasms Study comparing vigabatrin with prednisolone or tetracosactide at 14 days: a multicentre, randomised controlled trial. Lancet. Nov 13-19 2004;364(9447):1773-8. [Medline].

  30. Mackay M, Weiss S, Snead OC. Treatment of infantile spasms: an evidence-based approach. Int Rev Neurobiol. 2002;49:157-84. [Medline].

  31. Mackay MT, Weiss SK, Adams-Webber T. Practice parameter: medical treatment of infantile spasms: report of the American Academy of Neurology and the Child Neurology Society. Neurology. May 25 2004;62(10):1668-81. [Medline].

  32. Mahdi AH, Yohannan MD, Patel PJ, et al. Computerized tomography of brain in infantile spasms (West syndrome). Pediatr Radiol. 1990;21(1):9-11. [Medline].

  33. Muroi J, Okuno T, Kuno C, et al. An MRI study of the myelination pattern in West syndrome. Brain Dev. May-Jun 1996;18(3):179-84. [Medline].

  34. Pies NJ, Beardsmore CW. West & West syndrome--a historical sketch about the eponymous doctor, his work and his family. Brain Dev. Mar 2003;25(2):84-101. [Medline].

  35. Pietz J, Benninger C, Schafer H, et al. Treatment of infantile spasms with high-dosage vitamin B6. Epilepsia. Jul-Aug 1993;34(4):757-63. [Medline].

  36. Riikonen R. The latest on infantile spasms. Curr Opin Neurol. Apr 2005;18(2):91-5. [Medline].

  37. Shields WD. Catastrophic epilepsy in childhood. Epilepsia. 2000;41 Suppl 2:S2-6. [Medline].

  38. Stafstrom CE, Konkol RJ. Infantile spasms in children with Down syndrome. Dev Med Child Neurol. Jul 1994;36(7):576-85. [Medline].

  39. Veggiotti P, Cieuta C, Rex E, et al. Lamotrigine in infantile spasms [letter]. Lancet. Nov 12 1994;344(8933):1375-6. [Medline].

  40. Wang MC, Mak SC, Chi CS, et al. A long-term follow-up study of West syndrome. Chung Hua I Hsueh Tsa Chih (Taipei). Mar 1994;53(3):158-62. [Medline].

  41. Watanabe K. Medical treatment of West syndrome in Japan. J Child Neurol. Mar 1995;10(2):143-7. [Medline].

  42. West WJ. Infantile spasm. Lancet. 1841;1:724.

  43. Wyllie E. The Treatment of Epilepsy: Principles and Practice. 1st ed. Philadelphia:. Lippincott Williams & Wilkins;1993.

  44. Yamauchi T, Aikawa H. Efficacy of zonisamide: our experience. Seizure. Dec 2004;13 Suppl 1:S41-8; discussion S49. [Medline].

  45. Yanagaki S, Oguni H, Yoshii K. Zonisamide for West syndrome: a comparison of clinical responses among different titration rate. Brain Dev. Jun 2005;27(4):286-90. [Medline].

  46. Zupanc ML. Infantile spasms. Expert Opin Pharmacother. Nov 2003;4(11):2039-48. [Medline].

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Further Reading

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Keywords

infantile spasms, hypsarrhythmia, developmental delay, West syndrome, mental retardation, epilepsy syndrome

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Contributor Information and Disclosures

Author

Tracy A Glauser, MD, Professor, Departments of Pediatrics and Neurology, University of Cincinnati College of Medicine, Children's Comprehensive Epilepsy Program, Children's Hospital Medical Center of Cincinnati
Tracy A Glauser, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, and Child Neurology Society
Disclosure: Nothing to disclose.

Coauthor(s)

Diego A Morita, MD, Assistant Professor of Pediatrics and Neurology, Department of Pediatrics, Division of Neurology, Cincinnati Children's Hospital Medical Center, University of Cincinnati
Diego A Morita, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Robert Baumann, MD, Program Director, Professor, Departments of Neurology and Pediatrics, University of Kentucky
Robert Baumann, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American College of Epidemiology, American Epilepsy Society, and Child Neurology Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Kenneth J Mack, MD, PhD, Senior Associate Consultant, Department of Child and Adolescent Neurology, Mayo Clinic
Kenneth J Mack, MD, PhD is a member of the following medical societies: American Academy of Neurology, Child Neurology Society, Phi Beta Kappa, and Society for Neuroscience
Disclosure: Nothing to disclose.

CME Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Nicholas Y Lorenzo, MD, Chief Editor, eMedicine Neurology; Consulting Staff, Neurology Specialists and Consultants
Nicholas Y Lorenzo, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Neurology
Disclosure: Nothing to disclose.

 
 
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