Ohtahara Syndrome (Early Infantile Epileptic Encephalopathy)

What is Ohtahara Syndrome?

Ohtahara syndrome is a neurological disorder characterised by seizures.  The disorder affects newborns, usually within the first three months of life (most often within the first 10 days) in the form of epileptic seizures.  Infants have primarily tonic seizures, but may also experience partial seizures, and rarely, myoclonic seizures.  Ohtahara syndrome is most commonly caused by metabolic disorders or structural damage in the brain, although the cause or causes for many cases can’t be determined.  Most infants with the disorder show significant underdevelopment of part or all of the cerebral hemispheres.  The EEGs of infants with Ohtahara syndrome reveal a characteristic pattern of high voltage spike wave discharge followed by little activity.  This pattern is known as “burst suppression.” Doctors have not yet seen any inherited cases of Ohtahara syndrome, but have observed that boys are more often affected than girls.

Is there any treatment?

Antiepileptic drugs are used to control seizures, but are unfortunately not usually very effective for this disorder.  Corticosteroids are occasionally helpful.  In cases where there is a focal brain lesion (damage contained to one area of the brain) surgery may be beneficial.  Other therapies are symptomatic and supportive. 

What is the prognosis?

The course of Ohtahara syndrome is severely progressive.  Seizures become more frequent, accompanied by physical and mental retardation.   Some children will die in infancy; others will survive but be profoundly handicapped.  As they grow, some children will progress into other epileptic disorders such as West syndrome and Lennox-Gestaut syndrome.

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