自動症を伴う非定型的West症候群の1例

  • 福水 道郎
    国立精神・神経センター武蔵病院小児神経科
  • 黒川 徹
    国立精神・神経センター武蔵病院小児神経科
  • 吉川 秀人
    国立精神・神経センター武蔵病院小児神経科
  • 花岡 繁
    国立精神・神経センター武蔵病院小児神経科

書誌事項

タイトル別名
  • Pedaling Automatism in Atypical West Syndrome: a Case Report.

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説明

A seven-month-old girl with atypical West syndrome with pedaling automatism was reported. She started to have early infantile epileptic encephalopathy with suppression-burst (EIEE) at age 14 days followed by infantile spasms at 3 months of age. She began to have spasms with automatism at 4 month, mainly consisting of pedaling movements of both feet associated with tonic spasms of upper extremities and writhing of trunk. Ictal EEG showed irregular slow waves with occasional spikes predominantly over the right hemisphere mixed with artifacts. Interictal EEG during sleep showed suppression-burst patterns. Cerebral atrophy and microcephaly were revealed on brain CT, immature myelination on MRI and decreased blood flow in the frontal, temporal, and parietal lobes on the right on PET scan. Pedaling automatism is rare in infancy and its clinical significance was discussed in relation to West syndrome and complex partial seizures.

収録刊行物

  • 脳と発達

    脳と発達 23 (6), 617-622, 1991

    THE JAPANESE SOCIETY OF CHILD NEUROLOGY

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