West’s Syndrome (Infantile Spasms)

Clinical Description and Diagnosis
  • Richard A. Hrachovy
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 497)

Abstract

Infantile spasms are a unique disorder, peculiar to infancy and early childhood. These seizures, which have been recognized as an epileptic phenomenon since they were first described by William West in 184153 are referred to by a variety of names, including “flexion spasms”, “jackknife seizures”, “massive spasms” and “infantile myoclonic seizures”. In 1952, Gibbs and Gibbs16 described the interictal EEG pattern, hypsarrhythmia, which was noted to occur in a large number of infantile spasms patients. Most patients with infantile spasms have some degree of mental and developmental retardation, and the triad of infantile spasms, hypsarrhythmia and retardation has become known as “West’s syndrome”. In 1958, a major breakthrough in the treatment of this disorder was reported by Sorel and Dusaucy-Bauloye48 who noted improvement in the EEG and cessation of spasms in patients treated with adrenocorticotrophic hormone (ACTH).

Keywords

Attenuation Lactate Peri Prednisone Triad 

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References

  1. 1.
    Bour, F., Chiron, C., Dulac, O., and Plouin, P. Caract¨¦res ¨¦lectrocliniques des crises dans le syndrome d’AicardiEEG Neurophysiol. Clin.16 (1986) 341–353.Google Scholar
  2. 2.
    Carrazana, E.J., Barlow, J.K., and Holmes, G.L. Infantile spasms provoked by partial seizures, JEpilepsy3 (1990) 97–100.CrossRefGoogle Scholar
  3. 3.
    Carrazana, E.J., Lombroso, C.T., Mikati, M., Helmers, S., and Holmes, G.L. Facilitation of infantile spasms by partial seizuresEpilepsia34 (1993) 97–109.PubMedCrossRefGoogle Scholar
  4. 4.
    Chugani, H.T. and Conti, J.R. Classification of infantile spasms in 139 cases: The role of positron emission tomographyEpilepsia35 (1994) 19.CrossRefGoogle Scholar
  5. 5.
    Chugani, H.T., Shewmon, D.A., Sankar, R., Chen, B.J., and Phelps, M.E. Infantile spasms: II. Lenticular nuclei and brain stem activation on positron emission tomographyAnn. Neurol.31 (1992) 212–219.PubMedCrossRefGoogle Scholar
  6. 6.
    Constad, H., Wagner, R.S., and Caputo, A.R. Aicardi syndrome in one dizygotic twinPediatrics76 (1985) 450–453.PubMedGoogle Scholar
  7. 7.
    de Jong, J.G.Y., Delleman, J.W., Houben, M., Manschot, WA., de Minjer, A., Mol, J., and Slooff, J.L. Agenesis of the corpus callosum, infantile spasms, ocular anomalies (Aicardi’s syndrome): clinical and pathologic findingsNeurology26 (1976) 1152–1158.PubMedCrossRefGoogle Scholar
  8. 8.
    Donat, J.F. and Wright, F.S. Simultaneous infantile spasms and partial seizuresJ Child Neurol.6 (1991) 246–250.PubMedCrossRefGoogle Scholar
  9. 9.
    Druckman, R. and Chao, D. Massive spasms in infancy and childhoodEpilepsia4 (third series) (1955) 61–72.PubMedCrossRefGoogle Scholar
  10. 10.
    Drury, I., Beydoun, A., Garofalo, E.A., and Henry, T.R. Asymmetric Hypsarrhythmia: Clinical electroencephalographic and radiological findingsEpilepsia36 (1995) 41–47.PubMedCrossRefGoogle Scholar
  11. 11.
    Fariello, R.G., Chun, R.W, Doro, J.M., Buncic, J.R., and Prichard, J.S. EEG recognition of Aicardi’s syndromeArch. Neurol.34 (1977) 563–566.PubMedCrossRefGoogle Scholar
  12. 12.
    Frost, J.D., Jr., Hrachovy, R.A., Kellaway, P., and Zion, T. Quantitative analysis and characterization of infantile spasmsEpilepsia19 (1978) 273–282.PubMedCrossRefGoogle Scholar
  13. 13.
    Fukuyama, Y. Studies on the etiology and pathogenesis of flexor spasmsAde Neurol. Sci. (Tokyo)4 (1960) 861–867.Google Scholar
  14. 14.
    Glaze, D.G., Hrachovy, R.A., Frost, J.D., Jr., Kellaway, E, and Zion, T.E. Prospective study of outcome of infants with infantile spasms treated during controlled studies of ACTH and prednisone, J.Pediatr.112 (1988) 389–396.PubMedCrossRefGoogle Scholar
  15. 15.
    Glaze, D.G., Hrachovy, R.A., Frost, J.D., Jr., Zion, T.E., and Bryan, R.N. Computed tomography in infantile spasms: effects of hormonal therapyPediatr. Neurol.2 (1986) 23–27.PubMedCrossRefGoogle Scholar
  16. 16.
    Gibbs, F.A. and Gibbs, E.L.Epilepsy(Atlas of electroencephalography; vol. 2), Addison-Wesley, Cambridge, Massachusetts, 1952.Google Scholar
  17. 17.
    Gastaut, H., Gastaut, J.L., Regis, H., Bernard, R., Pinsard, N., Saint-Jean, M., Roger, J., and Dravet, C. Computed tomography in the study of West’s syndromeDee Med. Child Neurol.20 (1978) 2127.Google Scholar
  18. 18.
    Hoefer, P.F.A., de Napoli, R.A., and Lesse, S. Periodicity and hypsarrhythmia in the EEG: a study of infantile spasms, diffuse encephalopathies, and experimental lesions of the brainArch. Neurol.9 (1963) 424–436.PubMedCrossRefGoogle Scholar
  19. 19.
    Hrachovy, R.A. and Frost, J.D., Jr. Infantile spasms, Pediatr. Clin. North Am.36(2) (1989) 311–329.Google Scholar
  20. 20.
    Hrachovy, R.A. and Frost, J.D., Jr. Intensive monitoring of infantile spasms. In: D. Schmidt and P.L. Morselli (Eds.)Intractable Epilepsy: Experimental and Clinical AspectsRaven Press, New York (1986) pp. 87–97.Google Scholar
  21. 21.
    Hrachovy, R.A. and Frost, J.D., Jr. Severe encephalopathic epilepsy in infants: infantile spasms. In: W.E. Dodson and J.M. Pellock (Eds.)Pediatric Epilepsy: Diagnosis and TherapyDemos, New York (1993) pp. 135–145.Google Scholar
  22. 22.
    Hrachovy, R.A., Frost, J.D., Jr., and Glaze, D.G. Coupling of focal electrical seizure discharges with infantile spasms: incidence during long-term monitoring in newly diagnosed patients, J.Clin. Neurophysiol.11(4) (1994) pp. 461–464.PubMedCrossRefGoogle Scholar
  23. 23.
    Hrachovy, R.A., Frost, J.D., Jr., and Glaze, D.G. High dose/long duration vs. low dose/short duration corticotropin therapy in infantile spasms: A single blind studyJ. Pediatr.124 (1994) 803–806.PubMedCrossRefGoogle Scholar
  24. 24.
    Hrachovy, R.A., Frost, J.D., Jr., and Kellaway, P. Hypsarrhythmia: Variations on the themeEpilepsia25 (1984) 317–325.PubMedCrossRefGoogle Scholar
  25. 25.
    Hrachovy, R.A., Frost, J.D., Jr., and Kellaway, P. Sleep characteristics in infantile spasmsNeurology31 (1981) 688–694.PubMedCrossRefGoogle Scholar
  26. 26.
    Hrachovy, R.A., Frost, J.D., Jr., Kellaway, P., and Zion, T. A controlled study of ACTH therapy in infantile spasmsEpilepsia21 (1980) 631–636.PubMedCrossRefGoogle Scholar
  27. 27.
    Hrachovy, R.A., Frost, J.D., Jr., Kellaway, P., and Zion, T. A controlled study of prednisone therapy in infantile spasmsEpilepsia20 (1979) 403–407.PubMedCrossRefGoogle Scholar
  28. 28.
    Hrachovy, R.A., Frost, J.D., Jr., Kellaway, P., and Zion, T.E. Double-blind study of ACTH vs prednisone therapy in infantile spasmsJ Pediatr.103 (1983) 641–645.PubMedCrossRefGoogle Scholar
  29. 29.
    Hrachovy, R.A., Glaze, D.G., and Frost, J.D., Jr. A retrospective study of spontaneous remission and long-term outcome in patients with infantile spasmsEpilepsia32 (1991) 212–214.PubMedCrossRefGoogle Scholar
  30. 30.
    Jeavons, P.M. and Bower, B.D. Infantile spasms. In: P.J. Vinken and G.W. Bruyn (Eds.)The epilepsies(Handbook of clinical neurology; vol 15), Elsevier, New York (1974) pp. 219–234.Google Scholar
  31. 31.
    Jeavons, P.M. and Bower, B.D.Infantile spasms: a review of the literature and a study of 112 casesLondon: Spastics Society and Heineman (Clinics in developmental medicine no. 15) (1964).Google Scholar
  32. 32.
    Jeavons, P.M., Bower, B.D., and Dimitrakoudi, M. Long-term prognosis of 150 cases of “West syndrome”Epilepsia14 (1973) 153–164.PubMedCrossRefGoogle Scholar
  33. 33.
    Jeavons, P.M., Harper, J.R., and Bower, B.D. Long-term prognosis of infantile spasms: A follow-up report on 112 casesDev. Med. Child Neurol.12 (1970) 413–421.PubMedCrossRefGoogle Scholar
  34. 34.
    Kellaway, P. Neurologic status of patients with hypsarrhythmia. In: F.A. Gibbs (Ed.)Molecules and Mental HealthJ.B. Lippincott, Philadelphia (1959) pp. 134–149.Google Scholar
  35. 35.
    Kellaway, P., Hrachovy, R.A., Frost, J.D., Jr., and Zion, T. Precise characterization and quantification of infantile spasmsAnn. Neurol.6 (1979) 214–218.PubMedCrossRefGoogle Scholar
  36. 36.
    Lacy, J.R. and Penry, J.K.Infantile spasmsRaven Press, New York (1976).Google Scholar
  37. 37.
    Lerman, P. HypsarrhythmiaElectroencephalogr. Clin. Neurophysiol.27 (1969) 216.CrossRefGoogle Scholar
  38. 38.
    Lombroso, C.T. A prospective study of infantile spasms: clinical and therapeutic considerationsEpilepsia24 (1983) 135–158.PubMedCrossRefGoogle Scholar
  39. 39.
    Lombroso, C.T. and Fejerman, N. Benign myoclonus of early infancyAnn. Neurol.1 (1977) 138–143.PubMedCrossRefGoogle Scholar
  40. 40.
    Maheshwari, M.C. and Jeavons, P.M. The prognostic implications of suppression-burst activity in the EEG in infancyEpilepsia16 (1975) 127–131.PubMedCrossRefGoogle Scholar
  41. 41.
    Ohtahara, S. Electroencephalographic studies in infantile spasmsDev. Med. Child Neurol.7 (1965) 707.Google Scholar
  42. 42.
    Ohtahara, S. Seizure disorders in infancy and childhoodBrain Dev.6 (1984) 509–519.PubMedGoogle Scholar
  43. 43.
    Pavone, L., Mollica, E, Incorpora, G., and Pampiglione, G. Infantile spasms syndrome in monozygotic twinsArch. Dis. Child55 (1980) 870–872.PubMedCrossRefGoogle Scholar
  44. 44.
    Plouin, P, Jahn, C., Dulac, O., and Chiron, C. Enregistrement ambulatoire de l’EEG pendant 24 heures dans les spasmes infantiles ¨¦pileptiquesRev. EEG Neurophysiol. Clin.17 (1987) 309–318.Google Scholar
  45. 45.
    Riikonen, R. A long-term follow-up study of 214 children with the syndrome of infantile spasmsNeuropediatrics13 (1982) 14–23.PubMedCrossRefGoogle Scholar
  46. 46.
    Senga, P., Mayanda, H.F., and Yidika, M Spasmes infantiles chez deux jumeaux monozygotiques: une novelle observationPresse Med15 (1986) 485.PubMedGoogle Scholar
  47. 47.
    Singer, W.D., Haller, J.S., Sullivan, L.R., Wolpert, S., Mills, C., and Rabe, E.F. The value of neuroradiology in infantile spasms J.Pediatr. 100 (1982) 47–50.PubMedCrossRefGoogle Scholar
  48. 48.
    Sorel, L. and Dusaucy-Bauloye, A. A propos de 21 cas d’hypsarhythmia de Gibbs. Son traitement spectaculaire par l’ACTHActa Neurol. Psychiatr. Belg.58 (1958) 130–141.PubMedGoogle Scholar
  49. 49.
    Tijiam, A.T., Stefanko, S., Schenk, VM.D., and de Vlieger, M. Infantile spasms associated with hemihypsarrhythmia and hemimegalencephalyDev. Med. Child Neurol.20 (1978) 779–798.CrossRefGoogle Scholar
  50. 50.
    Van den Berg, B.J. and Yerushalmy, J. Studies on convulsive disorders in young children. I. Incidence of febrile and nonfebrile convulsions by age and other factorsPediatr. Res.3 (1969) 298–304.PubMedCrossRefGoogle Scholar
  51. 51.
    Watanabe, K., Iwase, K., and Hara, K. The evolution of EEG features in infantile spasms: a prospective studyDey. Med. Child Neurol.15 (1973) 584–596.CrossRefGoogle Scholar
  52. 52.
    Weinman, H.M. Lennox-Gastaut Syndrome and Its Relationship to Infantile Spasms (West Syndrome). In: E. Niedermeyer and R. Degen (Eds.)The Lennox-Gastaut SyndromeAlan R. Liss, Inc., New York (1988) pp. 301–316.Google Scholar
  53. 53.
    West, W.J. On a peculiar form of infantile convulsionsLancet1 (1841) 724–725.CrossRefGoogle Scholar
  54. 54.
    Yamamoto, N., Watanabe, K., Negoro, I., Furune, S., Takahashi, I., Nomura, K., and Matsumoto, A. Partial seizures evolving to infantile spasmsEpilepsia29 (1988) 34–40.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2002

Authors and Affiliations

  • Richard A. Hrachovy
    • 1
  1. 1.Baylor College of Medicine Department of Neurology One Baylor Plaza Houston

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