Advertisement
Review article| Volume 23, ISSUE 7, P654-657, November 2001

High-dose vitamin B6 treatment in West syndrome

  • Yasuhisa Toribe
    Correspondence
    Tel.: +81-725-56-1220; fax: +81-725-56-5682
    Affiliations
    Division of Pediatric Neurology, Osaka Medical Center and Research Institute for Maternal and Child Health,840, Murodo-cho, Izumi, Osaka 594-1101, Japan
    Search for articles by this author

      Abstract

      Approximately 10–30% of patients with West syndrome respond to high-dose vitamin B6 treatment. The response to vitamin B6 is rapid; seizures disappear within the first 2 weeks of treatment. Mild side effects, such as gastrointestinal symptoms and liver dysfunction, are observed in 40–70%, but these resolve after discontinuation or a reduction of the dosage of vitamin B6. High-dose vitamin B6 treatment is useful as a first line agent in treating West syndrome.

      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

        • Coursin D.B
        Convulsive seizures in infants with pyridoxine-deficiency diet.
        J Am Med Assoc. 1954; 154: 406-408
        • Hunt A.D
        • Stokes J
        • McCrory W.W
        • Stroud H.H
        Pyridoxine dependency: report of a case of intractable convulsions in an infant controlled by pyridoxine.
        Pediatrics. 1954; 13: 140-145
        • Bankier A
        • Turner M
        • Hopkins I.J
        Pyridoxine dependent seizures – a wider clinical spectrum.
        Arch Dis Child. 1983; 58: 415-418
        • Goutieres F
        • Aicardi J
        Atypical presentations of pyridoxine-dependent seizures: a treatable cause of intractable epilepsy in infants.
        Ann Neurol. 1985; 17: 117-120
        • Gordon N
        Pyridoxine dependency: an update.
        Dev Med Child Neurol. 1997; 39: 63-65
        • Gospe M.S
        Current perspectives on pyridoxine-dependent seizures.
        J Pediatr. 1998; 132: 919-923
        • Baxter P
        Epidemiology of pyridoxine dependent and pyridoxine responsive seizures in the UK.
        Arch Dis Child. 1999; 81: 431-433
        • Hansson O
        • Hagberg B
        Effect of pyridoxine treatment in children with epilepsy.
        Acta Soc Med Ups. 1968; 73: 35-43
        • Ekelund H
        • Gamstorp I
        • Von Studniyz W
        Apparent response of impaired development, minor motor epilepsy and ataxia to pyridoxine.
        Acta Paediat Scand. 1969; 58: 572-576
        • Spies T.D
        • Hightower D.P
        • Hubbard I.H
        Some recent advances in vitamin therapy.
        J Am Med Assoc. 1940; 115: 292-297
        • Fox J.T
        • Tullidge G.M
        Pyridoxine (vitamin B6) in epilepsy.
        Lancet. 1946; 2: 345
        • Livingston S
        • Hsu J.M
        • Petersen D.C
        Ineffectiveness of pyridoxine (vitamin B6) in the treatment of epilepsy.
        Pediatrics. 1955; 16: 250-251
        • French J.H
        • Grueter B.B
        • Druckman R
        • O'Brien D
        Pyridoxine and infantile myoclonic seizures.
        Neurology. 1965; 15: 101-113
        • Ohtahara S
        A study on the age-dependent epileptic encephalopathy (in Japanese).
        No To Hattatsu (Tokyo). 1977; 9: 2-21
        • Watanabe K
        Medical treatment of West syndrome in Japan.
        J Child Neurol. 1995; 10: 143-147
        • Ito M
        • Seki T
        • Takuma Y
        Current therapy for West syndrome in Japan.
        J Child Neurol. 2000; 15: 424-428
        • Ito M
        • Mikawa H
        • Taniguchi T
        Cerebrospinal fluid GABA levels in children with infantile spasms.
        Neurology. 1984; 34: 235-238
        • Kurlemann G
        • Deufel T
        • Schuierer G
        Pyridoxine-responsive West syndrome and gamma-aminobutyric acid.
        Eur J Pediatr. 1997; 156: 158-159
        • Ohtsuka Y
        • Matsuda M
        • Kohno C
        • Iyoda K
        • Ishida S
        • Yamatogi Y
        • et al.
        Pyridoxal phosphate in the treatment of West syndrome.
        in: Akimoto H Kazamatsuri H Seino M Wada A Advances in epileptology: XII Epilepsy International Symposium. Raven, New York, NY1982: 311-313
        • Ohtsuka Y
        • Yamatogi Y
        • Yoshida H
        • Matsuda M
        • Iyoda K
        • Terasaki T
        • et al.
        High-dose pyridoxal phosphate in the treatment of the West and the Lennnox syndrome (in Japanese).
        No To Hattatsu (Tokyo). 1983; 15: 225-233
        • Ohtsuka Y
        • Matsuda M
        • Ogino T
        • Kobayashi K
        • Ohtahara S
        Treatment of West syndrome with high-dose pyridoxal phosphate.
        Brain Dev. 1987; 9: 418-421
        • Yoshida R
        High dose pyridoxal phosphate therapy for West syndrome: clinical study of 59 patients with special attention to side effects (in Japanese).
        Tokyo Joshi Ikadaigaku Zasshi (Tokyo). 1993; 63: 1156-1184
        • Suzuki Y
        • Kita T
        • Mano T
        • Arai H
        • Matsuoka T
        • Kodaka R
        • et al.
        Outcome of initial treatment with high-dose vitamin B6, valproate sodium or clonazepam in West syndrome (in Japanese).
        No To Hattatsu (Tokyo). 1996; 28: 398-402
        • Blennow G
        • Starck L
        High dose B6 treatment in infantile spasms.
        Neuropediatrics. 1986; 17: 7-10
        • Pietz J
        • Benninger C
        • Schafer H
        • Sontheimer D
        • Mittermaier G
        • Rating D
        Treatment of infantile spasms with high-dosage vitamin B6.
        Epilepsia. 1993; 34: 757-763
        • Scholl J
        • Benninger C
        • Ebinger F
        • Breitbach-Faller N
        • Bast T
        • Rating J
        • et al.
        Treatment of infantile spasms with high-dose vitamin B6: the Heidelberg experience 1989–1999.
        Neuropediatrics. 2000; 31: A3-A4
      1. Toribe Y, Uede H, Suzuki Y, An evaluation on efficacy and side effects of the massive vitamin B6 therapy against West syndrome (abstract in Japanese). In: Mayanagi Y, compiled. Program and abstracts of the 34th Annual Meeting of the Japan Epilepsy Society, Tokyo. September 21–22, 2000. p. 99.

        • Ohtsuka Y
        • Ogino T
        • Asano T
        • Hattori J
        • Ohta H
        • Oka E
        Long-term follow-up of vitamin B6-responsive West syndrome.
        Pediatr Neurol. 2000; 23: 202-206
        • Seki T
        Combination treatment of high-dose pyridoxal phosphate and low-dose ACTH in children with West syndrome and related disorders.
        Jpn J Psychiatry Neurol. 1990; 44: 219-237
        • 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
        • Mizuno T
        • Tanaka I
        • Shima M
        • Nakai H
        • Ueyama T
        • Yoshioka A
        A case report: rhabdomyolysis associated with high dose vitamin B6 in West syndrome (in Japanese).
        Nihon Shonika Gakkai Zasshi (Tokyo). 2000; 104: 1215-1216