Ｗｅｓｔ症候群治療法の現状と将来

DOI PubMed 被引用文献1件 参考文献22件
  • 関 亨
    慶應義塾大学医学部小児科

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タイトル別名
  • Treatment of West Syndrome: Present and Future Perspectives.

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The efficacy and side effects of various antiepileptic drugs (AEDs), especially pyridoxine, ACTH and valproate sodium (VPA), in the treatment of West syndrome were reviewed.<BR>ACTH remains to be the most effective treatment for West syndrome. However, there are significant adversive effects with prolonged ACTH therapy. The efficacy of pyridoxine at the dose of 40-50 mg/ kg/day is less encouraging, but there are no serious adversive effects, as opposed to ACTH.<BR>Some reports have indicated the possible efficacy of VPA in regular, large (40-100 mg/kg/day) and very high (100-300 mg/kg/day) doses. However, in patients under the age of 2 years, monotherapy using VPA in lower doses should be employed to minimize the risk of VPA-induced adversive effects, such as fatal hepatic toxicity.<BR>Consequently, a safer and more effective treatment is required.<BR>We have reported a pilot study on combination therapy with high-dose pyridoxine phosphate (40-50 mg/kg/day) and low-dose ACTH (0.01 mg/kg/day) in 25 children with West syndrome, mainly on the basis of neurochemical analysis of the ictal epileptic spasms.<BR>The response rate was similar to those achieved with high- dose ACTH, but a quicker cessation of spasms was obtained with the combination therapy than ACTH monotherapy. Transient increases in liver enzymes occurred in 50%, but none of the patients developed more serious side effects.<BR>Further study is required to determine the efficacy of this combination therapy.

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  • 脳と発達

    脳と発達 29 (2), 91-99, 1997

    THE JAPANESE SOCIETY OF CHILD NEUROLOGY

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