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Medical notes: Ohtahara syndrome | Medical notes: Ohtahara syndrome |
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David Cameron's son Ivan has died of a rare condition which some doctors refer to as Ohtahara syndrome, a very rare form of epilepsy which also exhibits symptoms akin to those of cerebral palsy. | David Cameron's son Ivan has died of a rare condition which some doctors refer to as Ohtahara syndrome, a very rare form of epilepsy which also exhibits symptoms akin to those of cerebral palsy. |
Cameron's son died at the age of six | Cameron's son died at the age of six |
What exactly is it? | What exactly is it? |
Ohtahara syndrome is either inherited or develops as a result of trauma at birth. It is characterised by seizures which start in the first days of life. | |
Some babies have as many as 100 of these spasms everyday. | |
In the case of Ivan Cameron, his parents noted soon after his birth in 2002 that something was wrong: he had occasional spasms and seemed sleepy. | |
After extensive tests, they were told he had the syndrome and would be unlikely to ever walk or talk. | |
As well as being excessively sleepy, children with Otahara syndrome are often very floppy. | |
They usually go on to develop stiffness in their limbs - similar to children with cerebral palsy - which gives them severe impairments. | |
Behavioural abnormalities and learning difficulties are also expected. | |
There is still some debate as to whether it should be classified as a distinct condition, as Dr S Otahara did in 1976, or whether it should be seen as severe early onset epilepsy with a range of differing symptoms. | There is still some debate as to whether it should be classified as a distinct condition, as Dr S Otahara did in 1976, or whether it should be seen as severe early onset epilepsy with a range of differing symptoms. |
How rare is it? | How rare is it? |
It is thought that Ohtahara syndrome affects about 0.2% of children with epilepsy, which in turn affects about 0.5% of the population. Boys are thought to be slightly more affected than girls. | It is thought that Ohtahara syndrome affects about 0.2% of children with epilepsy, which in turn affects about 0.5% of the population. Boys are thought to be slightly more affected than girls. |
Is it always fatal? | Is it always fatal? |
The mortality rate is very high, according to Professor Brian Neville at UCL's Institute of Child Health. As many as half will die in infancy or childhood. | |
What is known as Sudden Unexplained Death in Epilepsy - or Sudep - affects a significant minority of all people with epilepsy, and this is a common cause of death for those with the syndrome. | |
The risk of Sudep may be reduced with medication to control seizures, but Ohtahara syndrome is often resistant to many of the anti-epileptic drugs available. Older medications such as phenobarbitone as still the preferred choice. | |
Some babies with a clear abnormality in the brain may be able to have surgery to remove the affected area, but this is unusual. | Some babies with a clear abnormality in the brain may be able to have surgery to remove the affected area, but this is unusual. |
Breathing difficulties are often associated with the condition, and those affected are more susceptible to illness. | |
Pneumonia is not uncommon, and children often die within the first two years of life as a result of such chest infections. |