A Talk on Epilepsy
- Author:
ALCOCK, M.R.C.P.,
Registrar, National Hospital for Epilepsy and Nervous Diseases Most of my readers have probably seen a number of fits, but there are so many points about an actual fit which I want to emphasise that a description of one seems to be the only logical way of beginning this article.
In the normally functioning brain, cells only react when they are stimulated by another cell in some other part. A fit is caused by a cell somewhere in the brain beginning to discharge spontaneously. As an illustration one may liken the brain to a smoothly running factory. In this factory one individual suddenly becomes an agitator and starts a revolution, infecting first his immediate neighbours and finally the whole factory.
From this original focus in the brain a wave of excitation spreads to the rest of the brain cells, travelling faster and faster as it moves further away from the starting point. The wave may either die away?in which case it only produces a minor fit?or it may involve the whole brain, and in that case it produces a generalised or major fit. The first stage of the fit is, therefore, the local manifestation produced by the stimulation of a small area round the original cell which began the fit. This first stage of the fit is known as the aura. As a * This articlc is based on a lecture delivered to the Staffordshire Association for Mental Welfare, Ath October, 1937,
fit may start anywhere in the brain, we have therefore a correspondingly large number of potential aurae, each of which represents in one of its purer forms the result of excitation of a small area of the brain.
Present knowledge of the brain itself is still limited; we know the function of only about a third of it. The rest we call the ” silent areas ” because if a ” silent area ” is stimulated there is no apparent result. Of the areas of which we have knowledge the best defined are (1) the motor area which produces movements of the body, (2) the sensory area, lying in the parietal region, (3) the visual area, in the occipital pole, and (4) the area for speech which lies at the lower end of the motor and sensory areas for the two sides of speech?the expressive and the receptive.
A fit may start in any of these areas. If it starts somewhere in the motor area, we get movement in some part of the body, perhaps the twitching of a thumb, and then the disturbance spreads to neighbouring areas of the motor area of the brain and we get twitching of the side of: the face and the rest of the hand. Then the disturbance spreading faster and faster goes on to include first the whole of that side of the body and then the other side of the body, so that the convulsion becomes generalised.
More interesting perhaps are the aurae that start on the receptive or sensory side. These may begin by a feeling in one part?described in various ways, a ” sort of numbness “, ” pins and needles “, ” electricity “; often the patient says that it is an ” indescribable feeling.”
The aura may be a visual one, either negative, such as a sense of blindness, or positive, such as flashes of light, and occasionally even more elaborate things, such as one case in which the patient always saw a country scene with ” a little, old lady in brown ” before his fits.
There are also aurae of taste and smell, and these are interesting in that they are often accompanied by a sense of familiarity, what the French doctors call “deja vue “?something that the patient feels he knows very well but can’t describe. One patient, for instance, said to me recently that ” it seems to come from long ago like an old music hall It is interesting perhaps to compare these aurae with the well known power that a smell has of bringing back associations.
It may be asserted by those who have seen lots of fits that many of them have no aura?no distinguishable first stage. This is undoubtedly true and is probably explained in two ways. In some there was a sensory aura but there is nothing to be seen by an observer and by the time the patient has come round he has forgotten about it. In other cases the fit began in one of the silent areas of the brain and by the time it has reached an area of known function it is spreading so quickly that only a generalised fit is observable.
I have not tried to tell you of every type of aura and if you observe the beginning of fits carefully and question your patient carefully you will probably discover many types which I have not mentioned, as for example the well-known epileptic cry.
A fit may consist only of the aura and then stop or it may go on to a generalised convulsion, composed of five stages :?(1) the aura, (2) the tonic stage when the patient falls, becomes unconscious and all his muscles go into a firm contraction, producing a rigid state in which the arms and legs are outstretched and stiff, and the hands and teeth are clenched. This lasts for some seconds and is followed by (3) the clonic stage, in which the muscles contract and relax alternatively and produce convulsions. These convulsions often involve the tongue and the jaws and it is the alternate projection and retraction of the tongue between the moving jaws that leads to the biting of the tongue. Then the convulsions die away, usually lasting 60 to 90 seconds and are succeeded by (4) the stage of relaxation, in which all the muscles go limp and it is in this stage that relaxation of the sphincters occurs. After this the patient gradually regains consciousness and passes into (5) the post-epileptic stage. In this there is practically always headache and many patients are drowsy, and sleep for a period. Occasionally in this stage there develops a condition known as automatism. In this the patient may perform complicated actions and afterwards have no recollection of them at all. Usually this stage of automatism is only a matter of a minute or two, but even though short it may be a matter of deep concern to the patient, for in this state he may, for example, undress himself or use obscene language in a way quite foreign to his usual nature.
Types of Fits
There are many varying types of fits, and it is important to recognise them, as although some cases run true to one type all their lives, some begin with minor fits and then, if untreated, may develop major ones later. If these cases are recognised while only having minor fits the fits may be stopped more easily. The mildest fits are those that consist of an aura only, and they may be difficult to recognise by themselves; more often they are interspersed with larger attacks which begin with the same aura but instead of dying away, go on to a full fit. Thus the aura can be recognised when it occurs by itself.
Then we have those in which there may be just a momentary blankness, a second’s unconsciousness. (It is important when dealing with epilepsy to be sure that the terms ” minute “, ” second ” and ” moment ” are used correctly, as many patients are in the habit of using ” minute ” for ” second ” and vice versa, a habit which may make evaluation of their story difficult.) These patients with momentary attacks are often cuffed at home or scolded at school for not attending, before the real nature of the condition is realised; or if they come only infrequently they may escape recognition altogether. The next type is that in which the patient just falls to the ground for a moment and then gets up again quite normal?and so the fits gradually become more marked until we have the typical major attack. In a certain number of cases one fit seems to set off a trigger and you get one fit succeeding another before the patient has had time to regain consciousness?what is known as status epilepticus?this in its severer types may last anything up to 48 hours or even longer and during that time the patient may have literally hundreds of fits.
Some cases only seem to have ” status and I know of one case who comes into hospital every two or three years and for 36-48 hours has fits every 3-5 minutes and then has no more at all until the next attack. This tendency?that one fit predisposes the brain to have another?is one of the main features of epilepsy and most cases have what is known as ” grouped epilepsy that is, they have two or three fits in the course of a day and are then free for a period until they again have another group. This factor is of importance in planning treatment and the earlier it is started and the more regularly the patients take their medicine the more chance there is of stopping their epilepsy. It also means that adequate doses must be quickly worked up to, and only reduced very slowly.
The Differential Diagnosis of Epilepsy
In deciding whether or not a patient has epilepsy we have two separate questions to solve : first, did the patient have a fit, and?secondly?if so, what was the cause of the fit?
Dealing with the first; in a typical major attack with generalised convulsions, tongue biting, etc., there is no difficulty excepted occasionally with hysteria, and many of the readers of Mental Welfare will be familiar with the various points of difference.
There are just a few, however, which I think are worth emphasising. Epileptic phenomena are characterised by their brevity and an actual fit, as separate from the post epileptic phase, does not last more than 2?3 minutes; a hysteric is seldom content with less than twenty minutes. If in any patient fits occur at night, they are almost certainly epileptic, and lastly if there is any doub?:, remember that true epilepsy is much more common than hysterical fits.
With minor fits the chief difficulty is in distinguishing them from faints, and this may be quite difficult especially in the early stages. There are, however, some things which will help; a faint usually has some obvious cause, such us a stuffy atmosphere, emotion, convalescence, or starvation (which is perhaps the commonest cause of all, and by which I mean being without food for any period of over 6?7 hours, a fact which may not have been thought unusual enough to be mentioned by the patient). In epilepsy there may be an aura of some sort; the fits tend to come on more frequently and more regularly and without precipitating causes, and even in minor attacks the headache and drowsiness afterwards tend to be more marked than with simple faints. A family historv of epilepsy is often useful too.
With regard to the second question, it is a mistake to regard epilepsy as a disease itself. Any brain if sufficiently stimulated will produce a fit. Some brains, however require a much slighter stimulus than others. A fit, therefore, may be the reaction of a healthy brain to some disease outside the nervous system altogether : for example some cases of kidney disease have fits; or it may be some disease in the brain itself, the commonest being a tumour growing in or on the brain.
On the other hand some brains seem to produce fits for no obvious cause and these are the cases which we call ” idiopathic epilepsy This is really just a nice word to cover our ignorance as undoubtedly there is some pathological cause for them, but so far we have not been able to discover the exact thing. Perhaps one should say rather it is the combination of two factors?one the unknown and the other the peculiar liability of that brain to have fits. This liability is often hereditary to a certain degree and so one finds that a proportion of epileptics have a family history of epilepsy. This is shown sometimes by cases of head injury. A severe injury to the head may be followed by epilepsy and often in the cases which develop epilepsy after a head injury it is found that there is a history either of convulsions as a child or of epilepsy in other members of the family, showing that again we have the two factors,?the inherited liability of the brain to have fits and the precipitating cause of the injury which brings this out.
In the great majority of cases we cannot find the second or precipitating cause and so we have to treat the other side, that is, the over-excitability of the brain.
Treatment
In an actual fit, all that one needs to do is to prevent the patient hurting himself and in particular to prevent him from biting his tongue. You should therefore lay him down on the ground, loose any tight covering and put something between his teeth. There is no object in trying to stop the convulsions and it is better to let the patient move as he will and observe the form the fit is taking. If you are interested in epilepsy, remember that the instructive part of a fit is the beginning because, as I have explained, once the disturbance has become general throughout the brain, fits tend to fall into the same pattern. After a fit the patient should just be left to have his sleep out.
General Treatment
Here one should just advise a normal routine, perhaps avoiding excitement a little more than in the ordinary patient. It is also a good rule that no epileptic should be allowed to bathe. The question of marriage often comes up, and at the hospital we are in the habit of allowing those who have no family history of epilepsy to have no restrictions, but if there is a family history advising against children.
Drugs
By much the most important part of the treatment is by drugs and the two most useful drugs are luminal and bromide; there are some others such as belladonna which are occasionally useful. The most important principle in treatment and what I chiefly want to emphasise is the fact that if a patient has one epileptic fit then he ought to take medicine every day for the rest of his life. It may not be necessary to take a very large dose, but I am sure that this is the right principle. This may seem rather sweeping but the fact that a patient has, without obvious cause, had one fit means that there is a constitutional instability of the brain and that this will require treatment permanently. The point also comes in here that one fit predisposes the brain to others and the more fits a patient has the more difficult the fits are to control.
Many people will compromise with saying that it is sufficient to take medicine for two years after the last fit, but the following case may illustrate the dangers of this. A patient came to the National hospital in 1909 when she was 16 with a history of fits for seven years recently coming once a week, she was put on medicine and the fits stopped in about 2?3 years, and in 1916 when she was 23, she had been on medicine for four years since her last fit, and decided not to come again. Two years later she was back again with a recurrence and was again put on medicine and this time it was seven years before the fits stopped again; then in 1922 she again became free and remained attending till 1936; recently, however, she came back again having had another fit?the first for 15 years. I feel sure that if only she had continued her medicine regularly she would have avoided not only the fit now but also the previous seven years of fits. This is a principle that it is difficult to teach patients and one of the doctors at hospital used to have a saying that the lucky ones are those that have a fit the first time they miss their medicine, because then they learn their lesson and are happy to go on taking medicine for the rest of their lives. There is also a popular prejudice that long continued medication like this will lead to some unknown damage, but there is a patient at hospital who first attended in 1879 and several who began during the 1880 decade, and who have taken medicine three times a day ever since without any obvious ill effect.
The idea in treatment then is to start as soon as possible and go on as long as possible, and the logical conclusion of this is the rule given above. Prognosis
I do not want it to be thought from these remarks on treatment that it is possible to control completely every case of epilepsy, yet I do think that the outlook is a good deal better than is usually realised, and no case is so bad that there is not a possibility of controlling the fits completely in time. My impressions are that they can be controlled in about 50 per cent of cases, and that if treatment can be started early, the proportion may be higher than this; in the remaining cases treatment can nearly always effect some improvement.
Apart from this, the dangers that an epileptic runs are of three sorts, the chief being that of injuring himself in a fit, usually through falling or occasionally through the muscular contractions of the fit itself. Next, those cases which have ” status epilepticus ” are in danger until this is controlled, and lastly, long continued fits may lead on to mental degeneration. On the whole, however, patients seem to survive their fits remarkably well, and any of these dangers is of surprisingly rare occurrence, but the economic difficulties met with are numerous, and the chance of leading a normal life is made very difficult.
Epilepsy and Mental Deficiency
I feel that you will be wondering when ] am going to talk about the relationship of epilepsy and mental deficiency.
The subject of the relationship of epilepsy and mental deficiency can be broadly divided into three classes?first, cases in which the epilepsy and mental defect are both due to some underlying cause. Here we have such conditions as : congenital syphilis?which may cause the one, the other, or both?and tuberose sclerosis; it may also happen that after some of the childish illnesses, such as measles or whooping cough, there is an inflammation of the brain which may lead both to fits and mental deterioration. The importance of these cases is that they should be recognised, as they may require treatment apart from that merely for the control of their epilepsy.
The second class is that in which epilepsy itself may lead to mental defects; this seems to happen particularly in cases which have numerous minor attacks, whether because this form of epilepsy is more difficult to treat, or whether from some other cause, is not known. It is partly because of these cases that it is so important to start the treatment of epilepsy early and to carry it out thoroughly, as it is not possible to predict which cases are going to go this way; and once they have begun to deteriorate, treatment is more difficult; it is, however, even then by no means hopeless, but if the fits can be stopped some of these patients make a most astonishing return to the normal state.
Lastly we have the cases?perhaps this is the largest group of all?of patients who start by being mentally defective and then later develop epilepsy. Here again the epilepsy is an adverse factor, from two points of view?first because it may lead to further mental deterioration, and secondly because it may lead in the case of children to exclusion from the Special Schools; treatment in these patients also, therefore, comes to be of importance.
There is another factor only too commonly associated with epilepsy, at least in London. A normal, epileptic child stands a considerable risk of being excluded from School. 1 do not know what the attitude of the school authorities here is, but in London 1 am fairly frequently meeting cases of this sort, and the result only too often is that by the time the fits are controlled the child is over school age and has had no education; thus the fact that he has ceased to suffer from epilepsy does not help him very much in his subsequent career.
This is just part of the popular prejudice against epilepsy, which is quit:out of proportion to the gravity of the disease, and the enquiry initiated by the Central Association for Mental Welfare into the conditions of life of epileptics, is to be cordially welcomed for there are many ways in which their lot could be improved. Perhaps the two ways in which help is most needed are with regard to schooling and occupation. One sees so many cases that are only having an occasional fit and are otherwise well and healthy, and who yet find it quite impossible to keep a job. There are, of course, a certain number of occupations that are unsuitable, but the suitable ones are much more numerous than most people will realise. I know of several patients who are working quite successfully as shop assistants, clerks, or in domestic work, with the rare employer who is reasonable. Usually, however, the trouble is that if they say they are epileptic they will not get the job, and if they conceal it, the first time they have a fit they are dismissed. From the hospital point of view there is also an additional complication, for employed patients are often not able to get the time off to fetch their medicine.
If one could educate the public to realise that epilepsy is not really such a dread disease, and that the majority of people with it are perfectly normal in between the fits, the lot of epileptics could be greatly improved and the need for social work on their behalf is a considerable one.
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