Thursday, February 26, 2009

Death of British political leader's young son brings attention to CP, epilepsy

From The Daily Mail in the UK. Ivan Cameron, 6, who died Feb. 25, was the son of British Tory party leader David Cameron.

Although he seemed healthy at birth, Ivan's parents knew within days that something was wrong. (Ivan is pictured with his father.)

Doctors soon gave the Camerons a distressing prognosis - their little boy was not expected to survive beyond his third birthday.

He was severely disabled by both cerebral palsy and epilepsy.

The combination of these conditions is quite common, but in Ivan's case it was even more serious because he also had Ohtahara syndrome.

This is an extremely rare complication of epilepsy, affecting just one in 500 sufferers, and boys more than girls.

It is caused by an underlying structural brain abnormality which might have a genetic origin or, more likely, is the result of brain damage before or around the time of birth.

It is rarely an inherited disorder and it is thought only four families in the world have two affected children.

Seizures start before the baby is three months old. Most die before the age of three, often due to chest infections or pneumonia.

A phenomenon known as sudden unexplained death in epilepsy is also a constant

Babies with Ohtahara syndrome - which was first described in medical textbooks only 30 years ago - are often very floppy, excessively sleepy and over time develop stiffness in their limbs.

Medication has limited effect and the children make little developmental progress, being totally dependent on others.

They often feed poorly and their sleep is punctuated by seizures and muscle spasms - between ten and 300 every 24 hours, which make round-the-clock care a necessity. Some people with cerebral palsy suffer only minor problems but, as in Ivan's case, others have severe disabilities.

Depending on which part of the brain is damaged, the cerebral palsy can lead to problems with sight, hearing, perception and learning difficulties.

There are three types of cerebral palsy: spastic, dyskinetic and ataxic and generally relate to which part of the brain has been affected.

Treatments to reduce the impact of the condition include physiotherapy, occupational therapy, and speech therapy - much provided by Scope, the charity once known as the Spastics Society. Surgery can correct severe deformities and medication can remove muscle spasms.

No two cases of cerebral palsy are the same - with the condition ranging from minor walking problems to severe disabilities.

Ivan was unable to walk or talk. He was fed drugs and liquid food through a tube in his abdomen and required 24-hour care.

All the different types of epilepsy affect more than 450,000 people in the UK.
Epilepsy in general is defined by having recurrent seizures (sometimes called fits).

A seizure is caused by a sudden burst of excess electrical activity in the brain, which causes disruption to the way messages pass along the body's systems.

The number and type of seizures will vary from person to person and there are many different associated syndromes.

Scientists know about 40 different types of seizure alone and a person may have more than one type.

Sometimes the reasons for epilepsy are known - such as brain damage suffered during a difficult birth or a stroke which starves the brain of oxygen.

However, most people with the condition (60 per cent) will not know the reason behind their epilepsy.

There is no current cure for epilepsy but medication can help control seizures and allow people to live as normal a life as possible.