Ohtahara syndrome

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Related to EIEE: Infantile epileptic encephalopathy, Infantile myoclonic encephalopathy
An early onset, extremely debilitating progressive epileptic encephalopathy characterised by tonic spasms, partial seizures and burst activity on electroencephalography
Management Ohtahara syndrome responds poorly to anticonvulsants and steroids
Prognosis Dismal
Well known patient Ivan, son of UK Prime Minister David Cameron, died of Ohtahara syndrome in 2009 at age 6

Ohtahara syndrome

(o-ta-ha'ra)
[S. Ohtahara, Japanese pediatric neurologist]
A form of progressive epileptic encephalopathy in newborns. The disease is difficult to treat and has a poor prognosis. Synonym: early infantile epileptic encephalopathy with suppression bursts