Pediatric Neurology
Volume 30, Issue 3 , Pages 216-218, March 2004

A successful treatment with pyridoxal phosphate for West syndrome in hypophosphatasia

  • Hitoshi Yamamoto, MD

      Affiliations

    • Department of Pediatrics, St. Marianna University School of Medicine, Kawasaki, Japan
    • Corresponding Author InformationCommunications should be addressed to:Dr. Yamamoto; Department of Pediatrics; St. Marianna University School of Medicine; Kawasaki 216-8511; Japan.
    • ,
  • Yuka Sasamoto, MD

      Affiliations

    • Department of Pediatrics, St. Marianna University School of Medicine, Kawasaki, Japan
    • ,
  • Yusaku Miyamoto, MD

      Affiliations

    • Department of Pediatrics, St. Marianna University School of Medicine, Kawasaki, Japan
    • ,
  • Hiroshi Murakami, MD

      Affiliations

    • Department of Pediatrics, St. Marianna University School of Medicine, Kawasaki, Japan
    • ,
  • Noriko Kamiyama, MD

      Affiliations

    • Department of Pediatrics, St. Marianna University School of Medicine, Kawasaki, Japan

Received 15 May 2003; accepted 6 August 2003.

Abstract 

We report a 2-month-old male with West syndrome associated with infantile hypophosphatasia. The male infant was born at term to a healthy mother after an uneventful pregnancy. He was born by cesarean section because of breech presentation. He was observed to have short extremities, and radiographs were consistent with achondroplasia. The serum alkaline phosphatase level was 2 IU/dL. Intractable tonic seizures developed 2 days after birth, and an electroencephalogram revealed a burst-suppression pattern for the first 2 months of life. The seizures were uncontrollable with conventional antiepileptic drugs. At the age of 2 months, he had a series of infantile spasms, and the electroencephalogram indicated hypsarrhythmia. Treatment with high-dose pyridoxal phosphate eliminated his seizures.

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PII: S0887-8994(03)00442-9

doi:10.1016/j.pediatrneurol.2003.08.003

Pediatric Neurology
Volume 30, Issue 3 , Pages 216-218, March 2004

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