[Syndrome of Ohtahara]

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K Yelin1, I Alfonso, O Papazian

  • 1Departamento de Neurología, Miami Children's Hospital, FL 33155-4079, USA.

Revista De Neurologia
|May 8, 2000
PubMed

Insights

Ohtahara syndrome and early myoclonic encephalopathy are likely the same condition, characterized by early infantile epileptic encephalopathy with specific seizure types and EEG patterns. Further research is needed to confirm the relationship and identify underlying causes.

Area of Science:

  • Neurology
  • Pediatric Neurology
  • Epileptology

Context:

  • Ohtahara syndrome and early infantile epileptic encephalopathy are severe early-onset epilepsy syndromes.
  • A literature review identified 51 cases with characteristics including early seizures, specific EEG patterns, and developmental delay.

Purpose:

  • To review the literature on Ohtahara syndrome and early infantile epileptic encephalopathy.
  • To determine if these syndromes represent a single clinical entity.

Summary:

  • The review of 51 cases revealed common features such as early-onset seizures, tonic seizures, burst-suppression EEG, developmental delay, and poor prognosis.
  • Seizure onset occurred within the first 86 days of life, with diffuse brain atrophy being the most common imaging finding.
  • While causes were often unidentified, treatments like ACTH and hemispherectomy showed limited success.

Impact:

  • This review suggests Ohtahara syndrome and early myoclonic encephalopathy may be the same entity, impacting diagnostic approaches.
  • Understanding the shared characteristics can guide future research into the etiology and treatment of these devastating early-life epilepsies.
Abstract

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