Although corticotropin (ACTH) is still the most effective drug for the treatment of West syndrome, a variety of other treatment modalities have been tried because of corticotropin's frequent and sometimes serious side effects. A recent survey on the treatment of this devastating disorder by Japanese child neurologists disclosed different therapeutic approaches from those taken by American or European child neurologists. Most Japanese child neurologists use vitamin B6 as the first agent and corticotropin as the third or second drug. Moreover, the dosage of corticotropin used by them is considerably smaller. Therefore, the current status of medical treatment of West syndrome is reviewed, especially comparing Japan with other countries. (J Child Neurol 1995;10:143-147).
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References
1.
Sorel L., Dusaucy-Bauloye A.: A propos de 21 cas d'hypsarhythmia de Gibbs. Son traitement spectaculaire par l'ACTH. Acta Neurol Psychiatr Belg1958 ;58:130-141.
2.
Bobele GB, Bodensteiner JB: The treatment of infantile spasms by child neurologists in the United States: A 1991 survey. Ann Neurol1991;3:461.
3.
Singer WD, Rabe EF, Haller JS: The effect of ACTH therapy upon infantile spasms. J Pediatr1980;96:485-489.
4.
Riikonen R. : A long-term follow-up study of 214 children with the syndrome of infantile spasms. Neuropediatrics1982;13:14-23.
5.
Lerman P., Kivity S.: The efficacy of corticotropin in primary infantile spasms. J Pediatr1982;101:294-296.
6.
Hrachovy RA, Frost JD, Kellaway P., Zion TE: Double-blind study of ACTH vs prednisone therapy in infantile spasms . J Pediatr1983;103:641-645.
7.
Snead OC, Benton JW, Myers GJ: ACTH and prednisone in childhood seizure disorders. Neurology1983;33:966-970.
8.
Lombroso CT: A prospective study of infantile spasms: Clinical and therapeutic correlations . Epilepsia1983;24:135-158.
9.
Fois A., Malandrini F., Balestri P., Giorgi D.: Infantile spasms. Long-term results of ACTH treatment. Eur J Pediatr1984;142:51-55.
10.
Glaze DG, Hrachovy RA, Frost JD, et al: Prospective study of outcome of infants with infantile spasms treated during controlled studies of ACTH and prednisone. J Pediatr1988;112:389-396.
11.
Snead OC, Benton JW, Hosey LC, et al: Treatment of infantile spasms with high-dose ACTH: Efficacy and plasma levels of ACTH and cortisol. Neurology1989;39:1027-1031.
12.
Nolte R.: West-Syndrom. Manatsschr Kinderheilkd1992 ;140: 446-450.
13.
Sher PK, Sheikh MR: Therapeutic efficacy of ACTH in symptomatic infantile spasms with hypsarrhythmia . Pediatr Neurol1993;9:451-456.
14.
Koo B., Hwang PA, Logan WJ: Infantile spasms: Outcome and prognostic factors of cryptogenic and symptomatic groups. Neurology1993;43:2322-2327.
15.
Kurokawa T. , Goya N., Fukuyama Y., et al: West syndrome and Lennox-Gastaut syndrome: A survey of natural history. Pediatrics1980 ;65:81-88.
16.
Matsumoto A. , Watanabe K., Negoro T., et al: Long-term prognosis after infantile spasms: A statistical study of prognostic factors in 200 cases . Dev Med Child Neurol1981 ;23:51-65.
17.
Suzuki N., Seki T., Yamawaki H., et al: Long-term prognosis of infantile spasms after standard ACTH treatment in Japan. No To Hattatsu1986;18:365-371.
18.
Matsuzaki M. : Long-term prognosis of 319 patients with West syndrome: Evaluation of repeated courses of ACTH in terms of 5-year prognosis of seizures and intellectual development. J Tokyo Womens Med Coll1993;63:178-187.
19.
Negoro T., Watanabe K.: Treatment of epilepsy in infancy with special emphasis on ACTH therapy . Jpn J Psychiatr Neurol1986;40:315-321.
20.
Parekh HU, Dunn DW: Infantile spasms. Ann Neurol1990;28: 473-474.
21.
Hrachovy RA , Frost JD, Glaze DG: High-dose, long-duration versus low-dose, short-duration corticotropin therapy for infantile spasms . J Pediatr1994;124:803-806.
22.
Awaya Y., Sugie H., Motegi R., Fukuyama Y.: A search for a minimal effective dose of ACTH against childhood epileptic encephalopathy, in Porter RJ, Matson RH, Ward AA Jr, Dam M (eds): Advances in Epileptology. XVth Epilepsy International Symposium. New York, Raven Press , 1984, pp 391-394.
23.
Imaizumi T. , Awaya Y., Fukuyama Y.: A search for a minimal effective dose of ACTH against infantile spasms—An evaluation of short term effects. J Tokyo Womens Med Coll1987 ;57: 568-572.
24.
Ichiba N., Yamatogi Y., Ohtahara S.: Reappraisal of the treatment for the age-dependent epileptic encephalopathy with ACTH. No To Hattatsu1987;19:190-197.
25.
Ito M., Okuno T., Fujii T., et al: ACTH therapy in infantile spasms: Relationship between dose of ACTH and initial effect or long-term prognosis. Pediatr Neurol1990;6:240-244.
26.
Kuriyama M. , Konishi Y., Fujii Y., et al: Treatment of infantile spasms with long-term low-dose ACTH. No To Hattatsu1992 ; 24:469-474.
27.
Haga Y., Watanabe K., Negoro T., et al: A study on ACTH dosage for treatment of West syndrome. No To Hattatsu1993;25: 215-220.
28.
Okuno T., Ito M., Konishi Y., et al: Cerebral atrophy following ACTH therapy. J Comput Assist Tomogr1980;4:20-23.
29.
Hara K., Watanabe K., Miyazaki S., et al: Apparent brain atrophy and subdural hematoma following ACTH therapy. Brain Dev1981;3:45-49.
30.
Satoh J., Takashige H., Hara H., Fukuyama Y.: Brain shrinkage and subdural effusion associated with ACTH administration . Brain Dev1982;4:13-20.
31.
Hrachovy RA , Frost JD: Infantile spasms. Pediatr Clin North Am1989;36:311-329.
32.
Bellman MH : Infantile spasms, in Pedley TA , Meldrum BS (eds): Recent Advances in Epilepsy , vol 1. Edinburgh, Churchill-Livingstone, 1983, pp 113-138.
33.
Aicardi J. : Infantile spasms and related syndromes, in Aicardi J (ed): Epilepsy in Children. New York, Raven Press , 1994, pp 18-43.
34.
Shamir R., Garty B-Z., Rachmel A., et al: Risk of infection during adrenocorticotropic hormone treatment in infants with infantile spasms. Pediatr Infect Dis1993;12:913-916.
35.
Konishi Y., Yasujima M., Kuriyama M., et al: Magnetic resonance imaging in infantile spasms: Effects of hormonal therapy. Epilepsia1992;33:304-309.
36.
Bobele GB, Ward KE, Bodensteiner JB: Hypertrophic cardiomyopathy during corticotropin therapy for infantile spasms. A clinical and echocardiographic study. Am J Dis Child1993; 147:223-225.
37.
Urashima M. , Akashi M., Kawame F., et al: Two cases of myocarditis during ACTH therapy for infantile spasms. No To Hattatsu1991;23:486-491.
38.
Liebling MS , Starc TJ, McAlister WH, et al: ACTH induced adrenal enlargement in infants treated for infantile spasms and acute cerebellar encephalopathy. Pediatr Radiol1993;23:454-456.
39.
Otani K., Tagawa T., Futagi Y., et al: Induced microseizures in West syndrome. Brain Dev1991;13:19Cr199.
40.
Holmes GL, Weber DA: Effects of ACTH on seizure susceptibility in the developing brain . Ann Neurol1986;20:82-88.
41.
Izumi T., Shishikura T., Fukuyama Y.: Suppression of 23-cyclic nucleotide 3-phosphohydrolase activity in suckling rat brain by ACTH. Brain Dev1986 ;8:500-504.
42.
Izumi T., Fukuyama Y., Nomoto T., Kato N.: Administration of ACTH to suckling rats results in hyperkinetic behavior . Brain Dev1986;8:575-579.
43.
Ohta S., Shimada M.: The effects of high dose ACTH treatment on the neuronal development of suckling mice. No To Hattatsu1989;21:26-36.
44.
Kusse MC, van Nieuwenhuizen AC, van der Mey W., et al: The effect of non-depot ACTH1-24 on infantile spasms. Dev Med Child Neurol1993;35:1067-1073.
45.
Farwell J., Milstein J., Opheim K., et al: Adrenocorticotropic hormone controls infantile spasms independently of cortisol stimulation. Epilepsia1984;25:605-608.
46.
Baram TZ: Pathophysiology of massive infantile spasms: Perspective on the putative role of the brain adrenal axis. Ann Neurol1993 ;33:231-236.
47.
Ohtsuka Y. , Matsuda M., Ogino T., et al: Treatment of the West syndrome with high-dose pyridoxal phosphate . Brain Dev1987;9:418-421.
48.
Yoshida R. : High dose pyridoxal phosphate therapy for West syndrome: Clinical study of 59 patients with special attention to side effects. J Tokyo Womens Med Coll1993;63:1156-1184.
49.
Benninger JPC , Schaefer H., Sontheimer D., et al: Treatment of infantile spasms with high-dosage vitamin B6. Epilepsia1993; 34:757-763.
50.
Ito M. , Okuno T., Hattori H., et al: Vitamin B6 and valproic acid in treatment of infantile spasms . Pediatr Neurol1991 ; 7:91-96.
51.
Seki T.: Combination treatment of high-dose pyridoxal phosphate and low-dose ACTH in children with West syndrome and related disorders. Jpn J Psychiatr Neurol1990;44:219-237.
52.
Pavone L., Incorpora G., La Rosa M., et al: Treatment of infantile spasms with sodium dipropylacetic acid . Dev Med Child Neurol1981;23:454-461.
53.
Bachman DS: Use of valproic acid in treatment of infantile spasms. Arch Neurol1982;39:49-52.
54.
Siemes H., Spohr HL, Michael TH, Nau H.: Therapy of infantile spasms with valproate: Results of a prospective study . Epilepsia1988;29:553-560.
55.
Prats JM, Garaizar C., Rua MJ, et al: Infantile spasms treated with high doses of sodium valproate: Initial responses and follow-up. Dev Med Child Neurol1991;33:617-625.
56.
Ohtsuka Y., Amano R., Mizukawa M., et al: Treatment of intractable childhood epilepsy with high-dose valproate . Epilepsia1992;33:158-164.
57.
Fisher E., Siemes H., Pund R., et al: Valproate metabolites in serum and urine during antiepileptic therapy in children with infantile spasms: Abnormal metabolite pattern associated with reversible hepatotoxicity. Epilepsia1992;33:165-171.
58.
Vassella F. , Pavlincova E., Schneider HJ, et al: Treatment of infantile spasms and Lennox-Gastaut syndrome with clonazepam (Rivotril) . Epilepsia1973;14:1.65-175.
59.
Dreifuss F. , Farwell J., Holmes G., et al: Infantile spasms. Comparative trial of nitrazepam and corticotropin. Arch Neurol1986;43:1107-1110.
60.
Ariizumi M. , Baba K., Hibio S., et al: Immunoglobulin therapy in the West syndrome. Brain Dev1987 ;9:422-425.
61.
Shiihara H. , Ariizumi M.: Immunoglobulin therapy of West syndrome. Shoni Naika1991;23:1421-1427.
62.
Echenne B., Dulac O., Parayre-Chanez MJ, et al: Treatment of infantile spasms with intravenous gamma-globulin . Brain Dev1991;13:313-319.
63.
Matsumoto A. , Kumagai T., Takeuchi T., et al: TRH therapy for West syndrome. Jpn J Psychiatr Neurol1987;41:432-434.
64.
Minagawa K. , Tanabe C.: The short-term effect of TRH therapy for intractable epilepsy in childhood. J Jpn Epilepsy Soc1989;7:13-20.
65.
Matsumoto A. , Kumagai T., Takeuchi T., et al: Factors influencing effectiveness of thyrotropin-releasing hormone therapy for severe epilepsy in childhood: Significance of serum prolactin levels. Epilepsia1989;30:45-49.
66.
Yagi K, Seki T (eds): Zonisamide in Epilepsy Therapy. Remarkable Improvement: 100 Cases. Tokyo, Sozo Shuppan, 1994.
67.
Gram L., Sabers A., Dulac O.: Treatment of pediatric epilepsies with gamma-vinyl GABA (Vigabatrin) . Epilepsia1992;33:S26-S29.
68.
Appleton RE : The role of vigabatrin in the management of infantile epileptic syndrome. Neurology1993 ;43:S21-S23.
69.
Vles JSH , van der Heyden Amhg, Ghijs A., Troost J.: Vigabatrin in the treatment of infantile spasms. Neuropediatrics1993;24: 230-231.
