​Ohtahara Syndrome

The information provided below is through the Epilepsy Foundation

Seizures Testing

​(EEG testing)


An electroencephalogram (EEG) is a test that detects electrical activity in your brain.

Recognizing signs of seizure activity. 


We immediately got Joey in to see a neurologist following some strange movements. He had his first EEG at 3 weeks. 

What is Ohtahara Syndrome?

Ohtahara Syndrome (OS) is a rare epilepsy syndrome seen in neonates/infants. It is also known as early infantile epileptic encephalop​athy 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 can get progressively worse. Ohtahara syndrome can affect both boys and girls.


Is there any treatment?

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.

What is the prognosis?

The outlook for children with OS may be grim. Many children progress to have infantile spasms (West Syndrome) or Lennox-Gastaut Syndrome (LGS).  Some children with OS may die within the first 2 years of life. Those who survive are typically left with severe physical and cognitive disabilities.

Hope for children with Ohtahara Syndrome relies on the medical and research community understanding more about the causes and what treatment may work best in OS.

The Rare Epilepsy Network (REN) helps connect families with research in these areas.


​Authored by: Elaine Kiriakopoulos MD, MSc | Drug Resistant and Rare Epilepsies Editor | Angel W. Hernandez-Mulero MD | Pediatrics Editor on 3/2015