This is an epileptic syndrome that affects newborns within the first three months of life, typically within the first 10 days of birth. Majority of these infants have tonic seizures, with some experiencing partial or myoclonic seizures. It is characterized by an abnormal electroencephalogram (EEG) pattern known as “burst suppression” which is a high voltage spike wave discharge followed by little activity.
The prognosis for Ohthahara syndrome is poor. These infants experience increasingly severe seizures that either impairs their cognitive and physical growth that progressively leads to other epileptic syndromes like West or Lennox-Gestaut syndrome.
Currently, antiepileptic drugs are used to control the seizures in these patients but are not very effective in most cases. Corticosteroids are helpful in certain cases and I case of focal lesions, brain surgery is recommended. Researchers are trying to find specific and effective treatment for this syndrome.
Cain Lab researcher, Dr. Mingshan Xue is currently investigating mechanisms that lead to Ohthahara syndrome with the ultimate goal of identifying potential therapeutic targets.