February 25, 2009

experts react to the news of the death of Ivan Cameron

David Cameron’s son died at the age of six from complications arising from severe epilepsy and cerebral palsy. He suffered from Ohtahara Syndrome, a rare neurological condition.

Dr Richard Appleton, Consultant Paediatric Neurologist, Alder Hey Children’s NHS Foundation Trust, Liverpool, said:

“Ohtahara Syndrome, named after a Japanese neurologist, is a very rare epilepsy syndrome, and just one of the 20-25 different epilepsy syndromes that occur in childhood. We have no idea of the prevalence in the UK as these data aren’t collected. In my 19 years as a consultant who specialises in epilepsy in children, I have seen just 5 children with the syndrome.

“It has characteristic criteria and that’s how we identify this syndrome. The criteria are tonic seizures and spasms followed by focal motor seizures that start when the child is aged 1 week to 2 months. They occur from 20 – 100 times per day, every day. The seizures may occur during wakefulness but also from sleep. The EEG (electroencephalogram) shows a characteristic pattern in the first few weeks or months. This pattern is termed ‘burst-suppression’, and is very abnormal. As soon as the seizures start to occur, child development is already impaired and with either no, or only minimal progress.

“We don’t always find a cause for Ohtahara Syndrome. When a cause is found, it is either a ‘brain malformation’ (which would have occurred within the first 10-12 weeks of the mother’s pregnancy), or a metabolic or biochemical cause. Many of the metabolic but only some of the brain malformation causes will have a genetic basis.

“It is very difficult, if not impossible to fully control the seizures with drugs or any other treatments, including brain surgery. The children continue to have seizures and make little or no developmental progress throughout their short lives. The majority will probably die before their 3rd birthday. Good nursing and medical care, including at home, can help to increase their life-expectancy.”

Prof Mark Richardson, Professor of Epilepsy, King’s College London, said:

“David Cameron is a loyal supporter of many epilepsy organisations, and our thoughts are with him and his family at this very sad time. In the face of this tragedy, there is an important message about epilepsy related deaths, which are much higher than most people appreciate and many of the deaths are amongst children. There are about 1000 deaths a year from epilepsy, some due to severe untreatable brain conditions, but about 400 of which we know could be prevented by better medical investigations and treatment. However, the cause of the majority of epilepsy-related deaths is not known, and needs to be the focus of intensive research.”

Additional information from Prof Mark Richardson: According to the National Society for Epilepsy, epilepsy is the most common serious neurological condition, with about 456,000 people suffering from epilepsy in the UK. Epilepsy can be caused by many different brain diseases, including brain tumours, stroke, brain infections, head injury and brain malformations. A national audit of epilepsy-related mortality in the UK (Hanna et al., 2002) established that every year nearly 1,000 people in England die of epilepsy-related causes, about 400 of which are avoidable; about 365 of those deaths are young adults and children. The leading cause of epilepsy-related death is so-called “Sudden Unexpected Death in Epilepsy” (SUDEP); this is a sudden death in a person with epilepsy, with no specific cause identified. Other important causes of epilepsy-related death are accidents (eg. drowning in a seizure), and deaths due to the underlying brain disease. The cause of SUDEP is not known and most SUDEP deaths are unwitnessed, although the best evidence points to death during a seizure or immediately afterwards (Tomson et al., 2008). It is possible that the seizure results in malfunction of the parts of the brain which control breathing and heart function. Epilepsy-related death is a neglected issue – there are more deaths due to epilepsy than from cot death and deaths from HIV combined in the UK. It is vital to fund research to better understand, and therefore avoid, epilepsy-related deaths. We know already that about 40% of epilepsy-related deaths could be prevented through better medical investigation and treatment (Hanna et al., 2002), but the cause of death in the other 60% is not well understood. Further research to understand and prevent SUDEP is vital. Ohtahara Syndrome is a very rare and extremely serious disorder affecting children (Ohtahara and Yamatogi, 2003). Severe epilepsy begins in the first few weeks of life accompanied by delayed cognitive and motor development. Epilepsy in very young babies is quite rare, and Ohtahara syndrome is the cause of about 10% of all cases of epilepsy in babies. In all children under the age of 10 with epilepsy, Ohtahara Syndrome is the cause in only a very small percentage (0.04-0.2%). The prognosis is poor, with death occurring before the age of 2 years in 25%, and severe epilepsy and severe learning difficulty persisting in those who survive into older life. There is not yet good information on longer-term survival of older children with Ohthara syndrome. The cause of Ohtahara Syndrome is often not established, but in some cases appears to be malformation of the brain. Cerebral Palsy is defined as follows: “Cerebral Palsy describes a group of disorders of the development of movement and posture, causing activity limitations, that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain. The motor disorders of cerebral palsy are often accompanied by disturbances of sensation, cognition, communication, perception and/or by a seizure disorder.” Therefore, Ohtahara Syndrome might in some children be associated with cerebral palsy. References: Bax M, Goldstein M, Rosenbaum P, Leviton A, Paneth N, Dan B, Jacobsson B, Damiano D; Executive Committee for the Definition of Cerebral Palsy Developmental Medicine and Child Neurology. 2005 volume 47 page 571-6. Hanna NJ, Black M, Sander JW, Smithson WH, Appleton R, Brown S, Fish DR. National sentinel clinical audit of epilepsy-related death. Norwich: The Stationary Office, 2002 Ohtahara S, Yamatogi Y. Epileptic encephalopathies in early infancy with suppression-burst. Journal of Clinical Neurophysiology. 2003 volume 20 pages 398-407. Tomson T, Nashef L, Ryvlin P. Sudden unexpected death in epilepsy: current knowledge and future directions. Lancet Neurol. 2008 volume 7 pages 1021-31.

Leigh Slocombe, Chief Executive of Epilepsy Research UK, said:

“The death of Ivan must be a terrible shock to David and his family. Everyone at Epilepsy Research UK is thinking of them at this sad time. David has supported the charity for most of Ivan’s short life and we are enormously grateful to him for his commitment which has undoubtedly helped to raise the profile of epilepsy.

“Sadly, 1,000 people die of epilepsy related causes every year and until we find more effective treatments, many more will do so. ERUK will continue to work with researchers and scientists in the hope that one day tragedies like this will be a thing of the past.”

Notes to editors from Epilepsy Research UK: Ohtahara syndrome is a neurological disorder characterized by seizures. The disorder affects newborns, usually within the first three months of life (most often within the first 10 days) in the form of epileptic seizures. Infants have primarily tonic seizures, but may also experience partial seizures, and rarely, myoclonic seizures. Ohtahara syndrome is most commonly caused by metabolic disorders or structural damage in the brain, although the cause or causes for many cases can’t be determined. Epilepsy is one of the most common neurological conditions. -It affects over 450,000 people in the UK alone. -75 people are diagnosed with epilepsy every day in the UK. -Every year 1,000 people die as a result of epilepsy. -Epilepsy can be caused by a stroke, a head injury or an inherent genetic defect, but in over 60% of cases there is no known cause. -Epilepsy can take many forms from a momentary blank spell to a dramatic major convulsion.

Alastair Kent, Director of Genetic Interest Group, said:

“For parents of children with life-limiting and disabling diseases, facing the possibility of their untimely death is a daily prospect. When this happens it is a tragedy that no amount of anticipation can dull. On behalf of the thousands of parents who have lost children to rare life-limiting conditions we offer our sympathy and condolences to David and Samantha Cameron and their family over the loss of their son Ivan. As research progresses more can be done to help those with rare conditions. We earnestly hope that as our knowledge advances, fewer families in the future will have to experience what David and Samantha Cameron and many like them are going through today.”

The National Society for Epilepsy, in a statement, said:

“The National Society for Epilepsy (NSE) has been saddened to hear of the death of David Cameron’s son Ivan, who had cerebral palsy and epilepsy. David Cameron became a vice president of the charity soon after his son was born and hosted an event at Westminster to raise awareness of NSE’s ground breaking research work.

“Epilepsy is the UK’s most common serious neurological condition affecting almost half a million people in the UK. Around 50 per cent of people with a severe learning disability also have epilepsy.

“NSE’s Chief Executive Graham Faulkner said: “This is very sad news. Our thoughts are with the Cameron family.”

“Every year nearly 1,000 people in England die of epilepsy related causes, about 400 of which are avoidable. Around 365 of those deaths are young adults and children.

“In 2007 an inquiry by the All Party Parliamentary Group on Epilepsy highlighted how the NHS was failing people with epilepsy. Their report ‘Wasted Money Wasted Lives’, concluded that 69,000 people were living with unnecessary seizures, 74,000 people were taking drugs they did not need, £189 million was needlessly spent each year and 400 deaths were avoidable. Their report called on the Government to take positive action by improving services which would also make significant cost savings.”