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SURGICAL APPROACHES TO EPILEPSY: SURGERY FOR OTHER TYPES OF SEIZURES – HEMISPHERECTOMY – BETH’S CASE HISTORY
SURGICAL APPROACHES TO EPILEPSY: SURGERY FOR OTHER TYPES OF SEIZURES – HEMISPHERECTOMY – BETH’S CASE HISTORYBeth was a bright, vivacious, almost five-year-old when she fell off of a see-saw and had a generalized seizure. A CT scan showed atrophy in the left hemisphere, but she had no neurological deficit. Shortly thereafter she began to have more seizures on the right side of her body. They did not respond to medication, and gradually she began to limp on the right leg and to have increasing difficulty with speech. Clearly something was continuing to happen to the left side of her brain. Another hospital diagnosed Rasmussen’s syndrome and said there was nothing they could do, that this viral-like process would continue to destroy her brain.Six months later her seizures were occurring several times a day and her speech and right-sided paralysis had worsened. She was seen at Johns Hopkins, where we agreed that she had Rasmussen’s syndrome. We told the family that this condition would inevitably become worse, that children with it become retarded and severely handicapped. We said that this progression would occur over several years. We told them that the only treatment was to remove the left side of Beth’s brain.Since at this time Beth was walking, talking, and had only slight intellectual deterioration, the family, quite understandably, was reluctant to subject their daughter to a risky operation that would leave her paralyzed on one side and might cause a problem with her ability to speak. They decided to wait.Six months later, when Beth was clearly having more difficulty with her seizures, with the right side of her body, and with school, they decided to have the operation.The operation was a success! After a stormy post-operative period, Beth has made a remarkable recovery. She is left-handed and uses her right hand only minimally. Her speech and reading are entirely normal. She has had no seizures since surgery and is on no medications. She is now in the regular third grade, doing well, keeping up with her classmates, telling “knock-knock” jokes, and learning how to play soccer. Clearly for Beth, half a brain is far better than a badly functioning whole brain.The choice is not always between removing a small part of one side of the brain and removing an entire hemisphere. These are the two extremes. Large parts of one side of the brain can, when appropriate, be removed. The risks and benefits of these operations depend on the abnormality causing the seizures and the area of the brain to be removed.*164\208\8*
Alcohol may undoubtedly precipitate seizures in those who already have had previous seizures.
There is also an association between chronic alcohol abuse and the occurrence of fits even when sober. Those who drink alcohol to excess are usually aware that they are running the risk of cirrhosis of the liver, but not many realize that chronic alcoholism can result in loss of cerebral nerve cells, seizures, and impairment of intellect.
Degenerative disorders-As advances in knowledge occur, fewer and fewer diseases will be assigned to this non-specific group. Creutzfeld-Jakob disease, the human equivalent of ‘mad cow’ disease for example, used to be regarded as degenerative , before it was shown to be caused by an infective agent. Pre-senile dementia (Alzheimer’s disease), in which the cerebral nerve cells gradually become fewer in number, is associated with seizures. Some cases are inherited, and almost certainly in most there is a biochemical abnormality responsible for this loss of nerve cells, and, hopefully, when this has been identified, some sort of pharmacological treatment will be possible. This sequence of events has already occurred in Parkinson’s disease. This was regarded as a degenerative disorder until 30 years ago. A defect in the metabolism of a transmitter called dopamine was identified, and a suitable drug (L-dopa) produced. A number of degenerative disorders which start in childhood (including one called Batten’s disease) present with frequent seizures.
Unfortunately, many of the above facilities are not yet available due to lack of funding, the relative lack of which also affects opportunities for research, and for attracting young researchers into the field. Epilepsy is, unfortunately, not regarded as important as many other clinical disorders when it comes to allocating funds for research. With the recent development of new anti-epileptic drugs, the pharmaceutical industry has provided generous support and sponsorship, particularly in areas of patient and professional education. Government and university departments and large charitable organizations are other important sources of funding for research and development.
Progress is likely to occur by the gradual accretion of new knowledge. There are journals, such as Epilepsia, Epilepsy Research, and Seizure devoted to publication of the results of the best research in epilepsy. There are also regular international meetings of those interested in epilepsy, so any real advance will be rapidly disseminated throughout the world.
The lot of those with epilepsy would be greatly improved, even if their seizures continued, if others—especially those involved with education and employers—showed greater understanding of their intermittent disability. The most probable benefit for the present generation of those with epilepsy is likely to result from such increased tolerance, rather than from any dramatic advances in treatment. Tolerance depends upon understanding the facts about epilepsy.
The understanding and treatment of epilepsy has improved considerably over the past fifty years. Most of this improvement has resulted from basic scientific research into how and why epileptic seizures start, and from the development of safer and more effective anti-epileptic drugs by the pharmaceutical industry. However, there is still much work which needs to be carried out, not just to improve our understanding and knowledge, but to improve the quality of life of a child or man or woman with epilepsy.
It is unlikely that a single ‘cure’ for all epileptic seizures and epilepsies will be found, due to the fact that there are so many different causes and types of epilepsy. It is also very unlikely that epilepsies will ever be completely prevented from occurring in the first place. A proportion of the epilepsies are inherited. It is improbable that much can be done to prevent these epilepsies, and it may not be possible (or appropriate) to remove these abnormal genes by ‘genetic engineering’ techniques. However, improved medical care should reduce the numbers of patients who develop epilepsy after meningitis or encephalitis. Improved safety measures on the roads, in cars, and the wider use of cycle helmets and protective head gear on industrial sites should reduce the incidence of posttraumatic epilepsy. In developing countries, better obstetric care and public health measures to eradicate parasitic diseases, (particularly cysticercosis) and bacterial diseases (particularly tuberculosis and other causes of meningitis) will play a part.
Orgasm in both men and women is presumably accompanied by some sort of ‘paroxysmal discharge of cerebral nerve cells’. This, and the impossibility of control of orgasm beyond a certain point, suggests an analogy with seizures. In fact seizures during or immediately after intercourse are exceptionally uncommon. When they do occur, they probably represent one of the types of reflex epilepsy. Except in these rarer cases, there is certainly no reason for avoiding intercourse on the grounds that seizures may be provoked.
Unfortunately, epilepsy is sometimes accompanied by a decline in sexuality. Many adolescents find their initial dates worrying enough (‘Which cinema should I take her to?’ ‘Should I let him kiss me?’), but how much more worrying must it be to take a girl out knowing that there is a chance, albeit a remote chance, that a seizure will occur during the date? Anxiety about contact with the opposite sex may have its basis in such entirely understandable problems of adolescence. However, there is evidence that a decline in sexuality may occur more frequently in those with seizures arising in the temporal lobe than in other types of seizures. Rarely, libido and potency may improve after temporal lobe surgery.
Sometimes patients may complain about loss of libido and sexual performance after beginning anticonvulsant drugs. This seems to happen most frequently after phenobarbitone and primidone, but it is difficult to be sure how much is due to the drugs, and how much to social and psychological factors.
Many relatives of people with epilepsy are naturally concerned as to what may happen during a seizure if they are not present to assist. We have known this anxiety carried to extremes. One of our patients, an epileptic woman of 30, was still sharing her parents’ bedroom, as they were concerned that she might come to harm during a nocturnal seizure, even though she had had none for 15 years! In practice, harm resulting from seizures is exceptionally rare, but there are a few sensible precautions.
Epilepsy as a social weapon-Families must be aware of ways in which epilepsy can be ‘used’. The child or young person with epilepsy, knowing of his parents’ anxiety about him, may manipulate them into granting him unreasonable requests. They may give in to feeling sorry for his difficulties, or they may feel that they should avoid an emotional upset that might precipitate a seizure. There is no reason why a child with epilepsy should not experience the same discipline as his siblings, who will themselves become jealous and unruly if they feel that one member of the family is being spoiled.
The other side of the coin is that parents may use the threat of epilepsy as a means of controlling behaviour which they otherwise cannot control. Examples we have met include limiting the hours of television watched, and the lateness of the hour by which an adolescent with epilepsy must return home.
As mentioned above, the first choice drug should be used alone (monotherapy) and in the lowest dose to control seizures without producing any unacceptable side-effects. If the initial control of seizures is less than complete, then the dose of that drug should be increased gradually until either complete control is achieved, or side-effects develop. If unacceptable side-effects occur before control is reached, then there are two alternatives: either a different drug can be used to replace the first drug, or an additional drug can be added to the first drug. Which alternative is chosen depends on the individual patient and also on the doctor. If there has been some reasonable control with the first drug, it is our practice to add the next most appropriate
anti-epileptic drug without withdrawing the first drug. If complete seizure control is then achieved, we will attempt to withdraw the first drug after a period of two to three months free from seizures. If the initial drug has been ineffective we would simultaneously replace the first drug with the second. In some children and adults, therapy with two anti-epileptic drugs is justified, as this may result in further significant (even complete) control in an additional 5 per cent to 10 per cent of children. It is unlikely that polytherapy with three drugs will result in any further control, and there is certainly an increased risk and frequency of side-effects and toxicity due to interactions between this many drugs.
This is often called MRI (magnetic resonance imaging) or nuclear magnetic resonance (NMR). The technique has nothing to do with radiation or X-rays, but records energy given out by atoms as they change their orientation after a brief magnetic pulse. The pictures or images produced have the same general appearance as CT scans, because the information processed by the computer is much the same as. Again it is necessary for the patient to lie still while the images are being taken.
The procedure is noisier than CT scanning and may, in some patients produce a claustrophobic feeling, as the patient is almost entirely enclosed in a tunnel. MRI usually takes about 25-35 minutes, but may take longer. Occasionally some contrast dye is injected into a vein, as in CT scanning, and then the scan repeated to demonstrate some additional details. Children may find the procedure more uncomfortable than having a CT scan and because of this more often need to have a brief general anaesthetic so that they lie still.
MRI gives a much clearer picture of those areas of the brain (the temporal lobes) which are most often responsible for intractable epilepsy, and so patients who are considered possibly to be suitable for surgery will certainly need an MRI. MRI is also useful for children in whom the epilepsy is thought to be due to a congenital malformation of the brain. Because of its greater costs (at present) MRI is unlikely to replace completely CT scanning, but there is no doubt that the level of detail obtained is far superior with MRI.
Some women gain three or four pounds (1-2 kg) in weight in the few days preceding their menstrual period. This gain is largely fluid, manifested by feeling bloated, with distended, sore breasts. Some women with epilepsy, particularly those with partial seizures, may notice an increase in frequency of seizures at the same time. It is not known if water retention is the responsible factor, or whether there is some more complicated hormonal cause. Dehydration with diuretic drugs has been used in attempts to abolish clusters of seizures occurring in relation to menstruation, but with limited success.
The weight gain associated with oral contraception does not seem to precipitate seizures. Oral contraception for women with epilepsy is satisfactory, provided that they understand the interactions between the pill and anti-epileptic drugs.
Stress and worry-It is impossible to quantify stress and worry. Problems perceived as molehills by some may be mountains to others. A period of hard work at school or office, or a time of emotional unhappiness at home, is often associated with an increased number of seizures. A vicious circle may arise, whereby stress and worry precipitate seizures, which in themselves cause further anxiety and hence further seizures. Sometimes an increased number of seizures leads to some crisis in employment, and the anxiety this causes results in a further deterioration in both epilepsy and job prospects.