Professional
Technical
IVT
Ohtahara syndrome
Pronunciation: oh-tah-HAH-rah
Early infantile epileptic encephalopathy with tonic spasms and burst-suppression EEG pattern.
Full Definition
The earliest form of age-related epileptic encephalopathy, typically manifesting within the first three months of life with frequent tonic spasms and a characteristic burst-suppression pattern on EEG during both sleep and wakefulness. Also known as early infantile epileptic encephalopathy (EIEE), this syndrome is often associated with severe structural brain malformations and carries a poor prognosis with significant developmental delays and high mortality. The condition may evolve into West syndrome or Lennox-Gastaut syndrome as the child ages.
Usage
Usage note: Always capitalize 'Ohtahara' as it is an eponymous syndrome.
In Context
- "The newborn presented with Ohtahara syndrome characterized by frequent tonic spasms and burst-suppression on EEG." — Neonatal neurology consultation
- "Despite aggressive treatment, the infant's Ohtahara syndrome remained refractory to multiple antiepileptic medications." — Pediatric epilepsy clinic note
Also known as
EIEE
early infantile epileptic encephalopathy
Don't confuse with
West syndrome
early myoclonic encephalopathy