OHTAHARA SYNDROME
Ohtahara Syndrome (OS) is a rare epilepsy syndrome seen in neonates/infants. It is also known as early infantile epileptic encephalopathy or early infantile epileptic encephalopathy with burst suppression pattern.
It is usually diagnosed before age 3 months, but in retrospect the first seizure could be felt towards the last trimester (when the baby is in the mother’s womb) or is seen during the first 10 days of life. Motor and cognitive problems can be seen in a baby that may get progressively worse. Many children progress to have infantile spasms (West Syndrome) or Lennox-Gastaut Syndrome (LGS).
Anti-seizure medications are routinely used, but seizures with OS are usually difficult to control. Medications that are often tried include clobazam (Onfi), clonazepam (Klonopin), vigabatrin (Sabril), topiramate (Topamax), zonisamide (Zonegran), Phenobarbital, valproate, or felbamate (Felbatol).
Epilepsy surgery may be an option in children with seizures starting in one area or involving one side of the brain. In these situations, a focal resection (removal of one area) or a hemispherectomy (removing most of one side of the brain) may be considered.
The Rare Epilepsy Network (REN) helps connect families with research in these areas