Mens Health Blog. Medical Blog » Epilepsy

EPILEPSY AND ITS SPECIAL FORMS/EPILEPSY SYNDROMES: STURGE-WEBER SYNDROME

admin — Fri, 13 May 2011 13:10:36 +0000

Sturge-Weber syndrome is not inherited. It is an abnormality of blood vessel formation occurring early in the development of the fetal face and brain. Children with this disease are born with a red birthmark (“port wine stain”) on the forehead and, at times, extending over the eye and lower face. Birthmarks that do not appear on the forehead are not associated with Sturge-Weber syndrome.
In this disease, blood vessel malformation causes atrophy (shrinking) of the underlying brain, leading to seizures and paralysis of the opposite side of the body. The seizures that accompany this shrinkage are usually one-sided and may be difficult to control with medication. Children with Sturge-Weber syndrome may be of normal intelligence but are often progressively handicapped by mental retardation, by the seizures, and by paralysis. Surgery to remove the affected portion of the brain is sometimes indicated and should be done early in treatment.
Laser surgery for the facial birthmark (hemangioma) shows promise of alleviating this disfiguring problem.
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CHILDREN WITH EPILEPSY: PARENTS’ CONCERNS

admin — Sat, 12 Feb 2011 14:46:32 +0000

In a recent survey, parents of children who had epilepsy were asked to list the things that concerned them most. Their top ten worries were these:
1. Whether their child might die in a seizure
The chances of this happening are very small. I can not say that they are non-existent because, very rarely, people unfortunately do die during a prolonged grand mal seizure. However they are usually young male adults, very seldom children. With any other type of seizure the risk is negligible.
Worrying that death during a seizure might happen will make your life miserable. Instead make sure that you know what to do if your child does have a prolonged convulsion, so that if an emergency arises it does not turn into a catastrophe.
2. Whether the child will suffer brain damage or become insane
Seizures themselves, provided they are not prolonged, do not cause brain damage, though it is possible for a child who has such severe seizures that they frequently fall over to suffer head injury and subsequent brain damage.
3. Whether the child might become insane
Seizures do not lead to mental illness, with the exception of a few adults and even fewer children who develop a very rare complication of a short mental illness after a run of generalized seizures. This illness usually only lasts for one or two weeks before the child returns to normal. However, it is such a very rare occurrence that it should not even figure on your worry-scale.
4. Guilt, because parents think that they may have done something to cause the condition
It is very common for parents whose children have epilepsy to blame themselves. You have to remind yourself that you had no responsibility for your own genetic make-up and thus no responsibility for the genes that you pass on to your children. It is no one’s fault; it is just the way things are. Overcoming these feelings of guilt is all part of you coming to terms with your child’s epilepsy. Unless you do come to terms with it you cannot help your child to do so.
5. Frustration at not knowing the cause of the seizures, especially if they have been given no adequate explanation
This is a situation which really need not arise nowadays. It is certainly much less common than it used to be, because of the marked improvement in neuroimaging technology which allows a much more detailed view of the structure of the brain and its function. Doctors can now discover the cause of epilepsy in about 90 per cent of cases. Even if you have not been told the cause of your child’s epilepsy, the chances are that your doctor knows. If he does not, ask to be referred to a specialist.
6. Worry about the development of emotional or behavioural problems in the child
Some children with epilepsy do behave badly and have emotional problems. So do some children without epilepsy. Remember that this kind of problem is not usually due to the epilepsy alone. It is more likely to be caused by the way the child’s epilepsy is handled in the family and at school, by the attitude of other children, or by the medication that the child is taking. These are all factors over which you have some control. Never assume that any problem your child has is because of their epilepsy. If you do, you may never look for the true cause, which may be bullying by classmates, an unsympathetic teacher or falling behind in schoolwork — any one of the myriad of reasons that any child has for periods of unhappiness, and which can lead them to behave badly. Once you have found the reason, enlist the help of your doctor or the school in tackling the problem.
7. The impact that the epilepsy may have on the child’s future career
There are only a few clear-cut limitations on the employment of anyone who has epilepsy. Your child may never become an HGV driver or a pilot, for example, but, on the whole, predictions about a child’s future career can only be made when the cause of their epilepsy is known and they have had a proper assessment by a neuropsychologist. Once you know what their intellectual attainment should be, and what their strengths and weaknesses are, you will be in a better position to help your child make realistic choices about their future.
8. The stigma of epilepsy, and the prejudice the child may have to face
Unfortunately no one can control other people’s ill-founded beliefs and prejudices. Your child is bound to meet them sometime; what matters is your child’s ability to believe that prejudice is the result of ignorance or stupidity and is no reflection on them personally. Your child will largely adopt and reflect your own attitudes. If you have always tried to talk frankly about their epilepsy and give them a positive attitude towards it, you will go a long way to counterbalance other people’s prejudices.
9. School problems, especially the effects on learning and memory of epilepsy and medication
Epilepsy and, more usually, the drugs used to treat it, can have an effect on learning and memory. Sometimes a change of medication can make a huge difference, so talk to your doctor if you think the drugs your child is taking are slowing them down. When your child goes to junior school, and again when they enter secondary school at 11, they should be assessed by a neuropsychologist so that everyone concerned knows what the child is capable of and, without having unrealistic expectations of them, encourages them to reach their full potential. Some children will do very well and have no difficulty; others may need special help.
10. The difficulty parents find in having a social life outside
the family
This problem may operate on several different levels. It may be that the child can not be left by the parents. Or it may be that the parents feel that they can not leave their child – a very different matter. The obstacle may also be the parents’ feelings about the way other people view them; they may feel so uncomfortable or defensive about their child’s epilepsy that it makes social contact difficult for them. Or it may simply be that the parents’ lives have become so bound up with caring for their child that they have little time to keep up old friendships or to make new ones. They may, in fact, have got out of the habit of having a social life.
There are partial solutions to all these problems, though it is unrealistic not to accept that without help or the means to buy help in, it can be very difficult for those few parents whose children have very severe epilepsy to develop some sort of life of their own. Social services may be able to arrange respite care, and some social services benefits may allow you to employ help and thus get some freedom.
That said, the vast majority of families never face anything like such a difficult situation. Often there is no reason why a child with epilepsy can not spend time apart from their parents if sensible arrangements are made with a friend, relative or babysitter. Parents may feel guilty about being away from their child, or may feel no one else is capable of looking after their child properly, but these feelings are usually unrealistic. Epilepsy support groups are a good way for parents to wean themselves out of the house into an environment where other people will be understanding and sympathetic to the way they feel. A child’s epilepsy can not and should not become the whole focus of the parent’s life. It is not fair on the parents, neither is it good for the child.
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EPILEPSY: THE FACTS-LONG-TERM OUTLOOK IN CHILDREN

admin — Tue, 28 Apr 2009 12:38:00 +0000

It is difficult, if not impossible to provide an overall prognosis for epilepsy in children, because of the differing ages of onset, different epilepsy syndromes, differing causes of epilepsy, and the varying response to treatment.

However, certain factors are known to be associated with a poor outcome, with seizures unresponsive or only partly responsive to treatment. These factors include:

• epilepsy that starts before the age of two or three years;

• seizure types that include myoclonic (jerk) or atonic (drop) seizures;

• seizures that are initially difficult to control;

• the need for more than one anti-epileptic drug to obtain control of the seizures;

• the association of other neurological problems, such as moderate or severe learning difficulties, or a physical handicap such as cerebral palsy; and

• if a cause has been identified (e.g. abnormal development of the

brain, as in tuberous sclerosis, or following meningitis or encephalitis.

There are other factors which indicate a good prognosis. These include:

• epilepsy that starts after 5 but before 13 years of age;

• seizure types that include typical absence (petit mal) seizures or tonic-clonic (grand mal) seizures;

• ready control of seizures, using just one anti-epileptic drug;

• a lack of other associated neurological problems;

• the absence of an identified cause; and

• the presence of a strong family history of epilepsy.

Many of the epilepsies in children can be classified into epilepsy syndromes. One of the purposes of this classification is to give some guidance on the prognosis or outcome. Syndromes that have a poor prognosis include the West syndrome (the seizure type in which is infantile spasms or myoclonic seizures) and the Lennox-Gastaut syndrome (seizure types include atonic, tonic, and myoclonic seizures). Seizures in both these syndromes start before the age of 3 years (rarely between 3 and 7 years in Lennox-Gastaut syndrome), and this in itself carries an unfavourable prognosis. Syndromes that have a good outcome include typical absence epilepsy (petit mal) and some partial epilepsies (for example, benign partial epilepsy with centro-temporal (rolandic) spikes). In typical absence epilepsy, between 70 and 75 per cent of children will stop having absence seizures by the age of 14-16 years and the anti-epileptic medication can be withdrawn. The remaining 25-30 per cent may need to continue taking medication into adult life, perhaps even for the rest of their life. The children that are likely to fall into this group are those in whom absences began after the age of 11 or 12 years, were associated with generalized tonic-clonic (grand mal) seizures, and in whom the seizures were difficult to control.

Benign partial epilepsy with centro-temporal (rolandic) spikes is, as the name suggests, really benign. All the children with this epilepsy syndrome will have stopped having seizures by 14-16 years of age, and medication can be withdrawn with no risk of relapse (recurrence) of seizures.

Some syndromes have an intermediate outlook. One of these is juvenile myoclonic epilepsy which usually starts between 10 and 16 years of age. The seizures (myoclonic and generalized tonic-clonic) are usually easily controlled by one drug (sodium valproate), but if the medication is withdrawn, the seizures may recur. Many (but not all) patients who have this type of epilepsy will have to take the treatment for the rest of their lives.

Overall, approximately 30-40 per cent of children will ‘outgrow’ their epilepsy before they become an adult. This means that the anti-epileptic medication can be withdrawn. Over 70 per cent of children with typical absence epilepsy will probably be able to have their medication withdrawn after they have been seizure-free for between two and three years. In contrast, over 90 per cent of children with Lennox-Gastaut syndrome will probably need to take anti-epileptic drugs for most of their lives.

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