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タイトル別名
  • A Study on Clinical Features of the Early Infantile Epileptic Encephalopathy with Suppression Burst or Ohtahara Syndrome
  • 大田原症候群としての提唱

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抄録

The early infantile epileptic encephalopathy with suppression burst (E. I. E. E.), which was first described by Ohtahara in 1976, is characterized by early onset (less than 3 months of age, f age), frequent tonic spasms, suppression burst EEG activity, poor prognosis and the transition to West syndrome. The purpose of the present study is to investigate the clinical features including cerebral CT and EEG findings of five cases with E. I. E. E.<BR>Results were as follows;<BR>1. Early onset of seizures; 1 to 9 days after birth.<BR>2. In all cases the EEGs showed the suppression burst pattern as a common denominator of this syndrome both in awake and sleep recordings. In most cases the suppression burst pattern disappeared after 3 months of age.<BR>3. The CT scan findings of all cases revealed mild or moderate brain atrophy at the time of admission.<BR>4. The underlying pathogenesis was not determined. Metabolic disorders and gross brain anomalies were excluded as far as possible by laboratory examinations.<BR>5. The types of seizures were tonic spasms and erratic seizures with or without series formation.<BR>6. Seizures were intractable in spite of various treatments including ACTH-Z therapy. Two patients died at 2 and 4 months of ages respectively. One case with the transition to Lennox syndrome died at 3 years of age, and remaining 2 cases have been suffering from severe psychmotor retardation.<BR>It was concluded that E. I. E. E. is the youngest type of age-dependent epilepsy, entirely separated from West syndrome, and it was proposed to call this type of epilepsy as Ohtahara syndrome.

収録刊行物

  • 脳と発達

    脳と発達 14 (4), 395-404, 1982

    THE JAPANESE SOCIETY OF CHILD NEUROLOGY

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