Advertisement

Extremely low-dose ACTH therapy for West syndrome in Japan

      Abstract

      Adrenocorticotropic hormone (ACTH) therapy is a widely used and effective treatment for West syndrome. However, serious adverse effects can occur during or shortly after ACTH therapy. Synthetic ACTH has been reported to cause more adverse effects than natural ACTH. Currently, most Japanese pediatric neurologists try other non-hormonal treatments before using ACTH therapy, but even then, they use extremely low dosages of synthetic ACTH. The extremely low-dose synthetic ACTH therapy currently used in Japan is as effective for treatment of West syndrome as the higher doses reported in previous studies from Japan and Western countries. The dosage and duration of synthetic ACTH therapy should be as low as possible to avoid serious adverse effects. Close monitoring of the adverse effects, especially subdural hematoma, is necessary even when extremely low dosages of ACTH are administered.

      Keywords

      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to Brain and Development
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Riikonen R
        • Donner M
        ACTH therapy in infantile spasms: side effects.
        Arch Dis Child. 1980; 55: 664-672
        • Riikonen R
        • Simell O
        • Jääskeläinen J
        • Rapola J
        • Perheentupa J
        Disturbed calcium and phosphate homeostasis during treatment with ACTH of infantile spasms.
        Arch Dis Child. 1986; 61: 671-676
        • Perheentupa J
        • Riikonen R
        • Dunkel L
        • Simell O
        Adrenocortical hyporesponsiveness after treatment with ACTH of infantile spasms.
        Arch Dis Child. 1986; 61: 750-753
        • Rao J.K
        • Willis J
        Hypothalamo-pituitary-adrenal function in infantile spasms: effects of ACTH therapy.
        J Child Neurol. 1987; 2: 220-223
        • Glauser T.A
        • Roger M
        Cushing's ulcer as a consequence of adrenocorticotropic hormone administration for infantile spasms.
        J Child Neurol. 1990; 5: 111-113
        • Bobele G.B
        • Ward K.E
        • Bodensteiner J.B
        Hypertrophic cardiomyopathy during corticotropin therapy for infantile spasms. A clinical and echocardiographic study.
        Am J Dis Child. 1993; 147: 223-225
        • Okuno T
        • Ito M
        • Konishi Y
        • Yoshioka M
        • Nakano Y
        Cerebral atrophy following ACTH therapy.
        J Comput Assist Tomogr. 1980; 4: 20-23
        • Hara K
        • Watanabe K
        • Miyazaki S
        • Hakamada S
        • Yamada H
        • Nakamura S
        Apparent brain atrophy and subdural hematoma following ACTH therapy.
        Brain Dev. 1981; 3: 45-49
        • Satoh J
        • Takeshige H
        • Hara H
        • Fukuyama Y
        Brain shrinkage and subdural effusion associated with ACTH administration.
        Brain Dev. 1982; 4: 13-20
        • Ito M
        • Takao T
        • Okuno T
        • Mikawa H
        Sequential CT studies of 24 children with infantile spasms on ACTH therapy.
        Dev Med Child Neurol. 1983; 25: 475-480
        • Nozaki H
        • Horita H
        • Nisimoto H
        • Maekawa K
        A case of infantile spasms with marked subdural hematoma during ACTH therapy.
        Shonikashinryo. 1988; 51 (in Japanese): 501-504
        • Sorel L
        196 cases of infantile myoclonic encephalopathy with hypsarrhythmia (IMEH: West syndrome) treated with ACTH: danger of synthetic ACTH.
        Electroencephalogr clin Neurophysiol. 1972; 32: 571-577
        • Riikonen R.S
        Steroid or vigabatrin in the treatment of infantile spasms?.
        Pediatr Neurol. 2000; 23: 403-408
        • Ito M
        Antiepileptic drug treatment of West syndrome.
        Epilepsia. 1998; 39: 38-41
        • Ito M
        • Seki T
        • Takuma T
        Current therapy for West syndrome in Japan.
        J Child Neurol. 2000; 15: 424-428
        • Glaze D.G
        • Hrachovy R.A
        • Frost J.D
        • Zion T.E
        • Bryan R.N
        Computed tomography in infantile spasms: effects of hormonal therapy.
        Pediatr Neurol. 1986; 2: 23-27
        • Kusse M.C
        • Van Nieuwenhuizen O
        • Van Hufflen A.L
        • Van der Mey W
        • Thijssen J.H.N
        • Van Ree J.M
        The effect of non-depot ACTH1–24 on infantile spasms.
        Dev Med Child Neurol. 1993; 35: 1067-1073
        • Fukuyama Y
        Diagnosis and treatment of epilepsies in childhood.
        J Jpn Pediatr Soc (Tokyo). 1968; 72 (in Japanese): 1462-1483
        • Singer W.D
        • Rabe E.F
        • Haller J.S
        The effect of ACTH therapy upon infantile spasms.
        J Pediatr. 1980; 96: 485-489
        • Riikonen R
        A long-term follow-up study of 214 children with the syndrome of infantile spasms.
        Neuropediatrics. 1982; 13: 14-23
        • Lombroso C.T
        A prospective study of infantile spasms: clinical and therapeutic correlations.
        Epilepsia. 1983; 24: 135-158
        • Snead O.C
        • Benton J.W
        • Myers G.J
        ACTH and prednisone in childhood seizure disorders.
        Neurology. 1983; 33: 966-970
        • Fois A
        • Malandrini F
        • Balestri P
        • Giorgi D
        Infantile spasms – long-term results of ACTH treatment.
        Eur J Pediatr. 1984; 142: 51-55
        • Sher P.K
        • Sheikh M.R
        Therapeutic efficacy of ACTH in symptomatic infantile spasms with hypsarrythmia.
        Pediatr Neurol. 1993; 9: 451-456
        • Hrachovy R.A
        • Frost J.D
        • Glaze D.G
        High-dose, long-duration versus low-dose, short-duration corticotropin therapy for infantile spasms.
        J Pediatr. 1994; 124: 803-806
        • Kurokawa T
        • Goya N
        • Fukuyama Y
        • Suzuki M
        • Seki T
        • Ohtahara S
        West syndrome and Lennox–Gastaut syndrome: a survey of natural history.
        Pediatrics. 1980; 65: 81-88
        • Ito M
        • Miyajima T
        • Fujii T
        • Okuno T
        Subdural hematoma during low-dose ACTH therapy in patients with West syndrome.
        Neurology. 2000; 54: 2346-2347
        • Awaya Y
        • Sugie H
        • Motegi R
        • Fukuyama Y
        A search for a minimal effective dose of ACTH against childhood epileptic encephalopathy.
        in: Porter R.J Mattson R.H Ward Jr, A.A Dam M Advances in epileptology: XVth Epilepsy International Symposium. Raven Press, New York, NY1984: 391-394
        • Ichiba N
        • Yamatogi Y
        • Ohtahara S
        Reappraisal of the treatment for the age-dependent epileptic encephalopathy with ACTH.
        No To Hattatsu (Tokyo). 1987; 19 (in Japanese): 190-197
        • Ito M
        • Okuno T
        • Fujii T
        • Mutoh K
        • Oguro K
        • Shiraishi H
        • et al.
        ACTH therapy in infantile spasms: relationship between dose of ACTH and initial effect or long-term prognosis.
        Pediatr Neurol. 1990; 6: 240-244
        • Kuriyama M
        • Konishi Y
        • Fujii Y
        • Yasujima M
        • Sudo M
        • Konishi K
        • et al.
        Treatment of infantile spasms with long-term low-dose ACTH.
        No To Hattatsu (Tokyo). 1992; 24 (in Japanese): 469-474
        • Haga Y
        • Watanabe K
        • Negoro T
        • Asoh K
        • Kitou M
        • Maeda N
        • et al.
        A study on ACTH dosage for treatment of West syndrome.
        No To Hattatsu (Tokyo). 1993; 25 (in Japanese): 215-220
        • Sejima H
        • Hojo H
        • Aiba H
        • Oguro K
        Re-evaluation of ACTH therapy for the treatment of West syndrome – the relationship between ACTH dosage and initial effects and long-term seizure prognosis.
        J Jpn Pediatr Soc (Tokyo). 1994; 98 (in Japanese): 1599-1604
        • Takuma Y
        • Seki T
        Combination therapy of infantile spasms with high-dose pyridoxal phosphate and low-dose corticotropin.
        J Child Neurol. 1996; 11: 35-40
        • Yanagaki S
        • Oguni H
        • Hayashi K
        • Imai K
        • Funatuka M
        • Tanaka T
        • et al.
        A comparative study of high-dose and low-dose ACTH therapy for West syndrome.
        Brain Dev. 1999; 21: 461-467
      1. Ito M, Aiba H, Hashimoto K, Kuroki S, Tomiwa K, Okuno T, et al. Low-dose ACTH therapy for West Syndrome: initial effects and long-term outcome. Neurology in press.

        • Bobele G.B
        • Bodensteiner J.B
        The treatment of infantile spasms by child neurologists.
        J Child Neurol. 1994; 9: 432-435
        • Ito M
        • Okuno T
        • Hattori H
        • Fujii T
        • Mikawa H
        Vitamin B6 and valproic acid in treatment of infantile spasms.
        Pediatr Neurol. 1991; 7: 91-96
        • Ito M
        • Mikawa H
        • Taniguchi T
        Cerebrospinal fluid GABA levels in children with infantile spasms.
        Neurology. 1984; 34: 235-238
        • Aicardi J
        • Sabril I.S
        • Mumford J.P
        • Dumas C
        • Wood S
        • Investigator and Peer Review Groups
        Vigabatrin as initial therapy for infantile spasms: an European retrospective survey.
        Epilepsia. 1996; 37: 638-642
        • Appleton R.E
        The treatment of infantile spasms by paediatric neurologists in UK and Ireland.
        Dev Med Child Neurol. 1996; 38: 278-279
        • Chung S.-H
        • Cox R.A
        Determination of pyridoxal phosphate levels in the brains of audiogenic and normal mice.
        Neurochem Res. 1983; 8: 1245-1259
        • Fariello R.G
        • Varasi M
        • Smith M.C
        Mechanism of action.
        in: Levy R.H Mattson R.H Meldrum B.S Antiepileptic drugs. Raven Press, New York, NY1995: 581-588
        • Ito M
        • Yu O
        • Chiu T.H
        Interaction of ACTH4–10 and ACTH1–24 with l-[3H]glutamate binding sites and GABA/benzodiazepine/picrotoxin receptor complexes in vitro.
        Brain Dev. 1988; 10: 106-109