Advertisement

What are the reasons for the strikingly different approaches to the use of ACTH in infants with West syndrome?

  • Tallie Z Baram
    Correspondence
    Tel.: +1-949-824-1063; fax: +1-949-824-1106
    Affiliations
    Department of Pediatrics, ZOT 4475, University of California at Irvine, Irvine, CA 92697-4475, USA

    Department of Anatomy & Neurobiology, ZOT 4475; University of California at Irvine, Irvine, CA 92697-4475, USA

    Department of Neurology, ZOT 4475; University of California at Irvine, Irvine, CA 92697-4475, USA
    Search for articles by this author

      Abstract

      A large body of experience has been compiled in different countries, documenting the efficacy of adenocorticotropic hormone (ACTH) for infantile spasms. This is important, because it may serve as a key for understanding this disorder, as well as for designing better medicines. However, significant discrepancies exist among studies originating in different countries regarding the relative efficacy of small or large ACTH doses.
      These differences may be caused by a number of factors, including potential genetic or environmental-related differences in the biology of the disorder or associated genetic components that determine responsiveness to ACTH. In addition, striking differences in the preparations used around the world may be responsible. These include bio-availability and extent of blood brain barrier penetration, efficacy in activating the efficacy-mediating ‘ACTH receptors’, the presence in certain preparations of competing analogs, and others. These issues should not detract from the overall agreement that ACTH might be the most useful medication currently available to treat WS.

      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

        • Hrachovy R.A
        • Frost J.D
        • Kellaway P
        • Zion T.E
        Double-blind study of ACTH vs prednisone therapy in infantile spasms.
        J Pediatr. 1983; 103: 641-645
        • Hrachovy R.A
        • Frost J.D
        Infantile spasms.
        Pediatr Clin North Am. 1989; 36: 311-329
        • Glauser T.A
        • Clark P.O
        • Strawsburg R
        A pilot study of topiramate in the treatment of infantile spasms.
        Epilepsia. 1998; 39: 1324-1328
        • Takuma Y
        ACTH therapy for infantile spasms: a combination therapy with high-dose pyridoxal phosphate and low-dose ACTH.
        Epilepsia. 1998; 39: 42-45
        • 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
        • Baram T.Z
        • Mitchell W.G
        • Tournay A
        • Snead O.C
        • Hanson R.A
        • Horton E.J
        High dose ACTH versus prednisone for massive infantile spasms: a prospective randomized blinded study.
        Pediatrics. 1996; 97: 375-379
        • Snead III, O.C
        Treatment of infantile spasms.
        Pediatr Neurol. 1990; 3: 147-150
        • Yamamoto H
        • Asoh M
        • Murakami H
        • Kamiyama N
        • Ohta C
        Liposteroid (dexamethasone palmitate) therapy for West syndrome: a comparative study with ACTH therapy.
        Pediatr Neurol. 1998; 18: 415-419
        • 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
        • 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
        • Aso K
        • Kito 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
        • Brunson K.L
        • Khan N
        • Eghbal-Ahmadi Baram T.Z
        ACTH acts directly on amygdala neurons to down-regulate corticotropin releasing hormone gene expression.
        Ann Neurol. 2001; 49: 304-313
        • Heiskala H
        • Riikonen R
        • Santavuori P
        • Simell O
        • Airaksinen E
        • Nuutila A
        • et al.
        West syndrome: individualized ACTH therapy.
        Brain Dev. 1996; 18: 456-460
        • Baram T.Z
        Pathophysiology of massive infantile spasms (MIS): perspective on the role of the brain adrenal axis.
        Ann Neurol. 1993; 33: 231-237