Ohtahara syndrome

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
References in periodicals archive ?
Three-year-old Callum suffers from Ohtahara syndrome - a rare epilepsy condition - and was referred to Ty Hafan in 2013.
Ivan lived with cerebral palsy and a rare and severe form of epilepsy known as Ohtahara syndrome.
And the Prime Minister's first child, Ivan, was born with Ohtahara syndrome, a rare form of cerebral palsy and epilepsy.
Ohtahara syndrome which is one of the severe epileptic encephalopathy pictures observed in newborns was defined in 1976 for the first time.
That's because 14-year-old Connor suffers from Ohtahara syndrome - a very rare and extreme complication of epilepsy - which has left him disabled for life.
Ohtahara syndrome is a rare neurological disorder of infancy, which consists of frequent generalized seizures and continuous abnormalities on electroencephalogram.
Ivan had a rare epilepsy condition accompanied by severe cerebral palsy called Ohtahara Syndrome.
Only now do we understand how the family lived on a knifeedge, consumed by the 24-hour-a-day care of a child who suffered from a tragic combination of cerebral palsy, severe epilepsy and the extremely rare neurological disorder Ohtahara syndrome.
Ivan, six, died 45 minutes after being rushed to London's St Mary's Hospital, Paddington, following a seizure on Wednesday He suffered from cerebral palsy and Ohtahara syndrome, a very rare form of epilepsy which caused frequent spasms.
Ivan, who suffered from Ohtahara Syndrome, a severe form of cerebral palsy which also involves a rare and severe epilepsy syndrome, was taken ill overnight and died yesterday, shortly after arriving at St Mary's Hospital in Paddington, London.