Ohtahara syndrome
Pronunciation: oh-tah-HAH-rah
A severe early infantile epileptic encephalopathy characterized by tonic seizures and suppression-burst pattern on EEG.
Full Definition
Ohtahara syndrome, also known as early infantile epileptic encephalopathy, is one of the earliest and most severe forms of epilepsy, typically beginning within the first three months of life. The condition is characterized by frequent tonic seizures and a distinctive suppression-burst pattern on EEG that persists during both sleep and wake states. The syndrome is often associated with structural brain abnormalities and carries a poor prognosis with severe developmental delays. Many patients may progress to West syndrome or Lennox-Gastaut syndrome as they age.
Usage
Usage note: Named after Shunsuke Ohtahara; represents the earliest form of epileptic encephalopathy.
In Context
- "The neonate's intractable tonic seizures and suppression-burst EEG pattern were diagnostic of Ohtahara syndrome." — Pediatric neurology consultation
- "Despite multiple antiepileptic medications, seizures remained refractory in this case of Ohtahara syndrome." — Epilepsy clinic note