EPILEPSY. HISTORICAL BACKGROUND
The human race has been familiar with epilepsy from time immemorial. The earliest writings of ancient civilisations and cultures mention the condition. The oldest known document on epilepsy is a clay tablet written in the Sumerian language and dated at 1067-1046 B.C., i.e., the reign of the Babylonian king Adad-apla-iddina. The tablet, which is covered in cunei-form inscriptions, is called antashubba which is Sumerian for "falling disease"; it is one of several dozen chapters of a handbook of human diseases.
|Fig. 1. The oldest known document on epilepsy written in cunei-form writing in the Sumerian language. |
The message contains descriptions based on insightful observations of the situations anticipating seizures; it also mentions the prognosticated course of epilepsy and many types of seizures which we now call tonic-clonic, absence or partial complex seizures.
The nature of epilepsy was the object of investigation of many ancient and mediaeval writers, e.g., Aristotle, Hippocrates, Galen, Averroes and Avicenna. Hippocrates identified several different forms of epilepsy, one of which he considered to be peculiar to women. Prompt marriage was his remedy. In these ancient writings we also find attempts to identify the causes and effective methods of treatment of epilepsy which, by then, was actually called epilepsy.
Throughout history, treatment of epilepsy in various cultures depended on the standards of development of medical knowledge and the philosophy of the day. Prevention of seizures was not only the business of the medics, it was also part of the work of the shamans and the exorcists. From the sixteenth century on, amidst the Greek and Latin texts we also find works by Polish writers, e.g., Szymon of Łowicz, J. Chróściejewski, S. Falmirz and M. Siennik (cf. Tomal 1994). Epileptologia gives a comprehensive bibliography of writings on epilepsy from the first publications in 1537 to present times.
|Fig. 2. A fragment of "Enchiridion medicinae, pro tyruculis huius artis quam compediosissime, per Simonem de Lovic" by Szymon of Łowicz (Florian Unger, Kraków 1537).
|| ||Fig. 3. The title page of Jan Chró¶ciejowski's "De morbis puerorum tractus" (Venice 1853)|
Traditionally, convulsive seizures, rich in symptoms and often associated with mysticism, magic and shamanism, attracted the most attention. In bygone days and in certain cultures people who had this kind of seizures were attributed exceptional power and they were the object of particular attention. In the Middle Ages people suffering from epilepsy were quite commonly believed to be obsessed or possessed by the devil, the Evil One. As the years went by, knowledge of the pathogenesis, diagnosis and particularly the treatment of epilepsy evolved considerably.
Our knowledge of epilepsy increased significantly in the last century but the most important diagnostic progress was not to be achieved until the mid-twentieth century and the greatest therapeutic progress only took place within the last twenty years. The dynamic development of knowledge on pathogenesis, diagnosis a particularly treatment of epilepsy recently led to the emergence of a new scientific discipline, epileptology.
MODERN IDEAS ON THE NATURE OF EPILEPSY
Every human behaviour disorder may be a sign of epilepsy if it is triggered by sudden focal or generalised specific cerebral dysfunction and if there is a tendency for these incidents to repeat. It is now accepted that two epileptic attacks without a tangible cause within a period of 1 to 2 years signifies epilepsy. However in certain conditions even one seizure is sufficient to diagnoze epilepsy and to start antiepileptic treatment.
The quantitative data indicate that epilepsy is now one of the most frequent neurological diseases. The prevalence of epilepsy is about 1000 per 100 thousand and we must assume that 1 percent of the Polish population, i.e., about 400 thousand people have epilepsy. Prevalence is highest in over sixty-five-year olds and is 2-3 percent. However, if we take the incidence of at least one epileptic attack in the whole life span, including attacks of febrile convulsions, then the risk rate for the population is about 6-8 percent. It is not easy to say whether a seizure will repeat and when and only a professional neurologist specialising in epilepsy (an epileptologist) can venture to make such predictions.
We now know that the epileptic seizure is the result of development in a particular part of the brain of an epileptic focus embracing a group of nerve cells which manifest periodical pathological hyperexcitability, a state which may interfere to various extents with the normal functioning of the human nervous system. This neuronal hyperexcitability is manifested in high frequency of bioelectrical discharge in a group of neurones called the epileptic focus. Depending on the localisation and spread of bioelectrical discharge of the epileptic focus we can have many different types of seizures (see below for classification). In addition to the most familiar type - with convulsions of the whole body and loss of consciousness and called generalised convulsive epileptic seizures - attacks may be limited to jerks of the hand, foot, palm or even a single finger. This type of seizure is called a simple partial motor seizure. Partial seizures may be incidents involving simply the performance of a series of automatic actions whose dysfunctional nature is hard for onlookers to assess. Epileptic seizures may also involve complete or partial loss of consciousness or simply disturbed sensory perception, e.g., experiencing tastes, smells, sounds or images in the absence of an actual environmental stimulus. Also, individual symptoms may enter various combinations giving a symptomatologically complex picture of the seizure. Subjective disorders are so complex and so heterogeneous that it is impossible to list them here. What the uninitiated should look out for is the sudden, brief and repetitive nature of any sensation or motor disorder appearing without an obvious reason. In every case the diagnosis must be confirmed by presence of appropriate changes in the electroencephalographic recording (EEG).
TYPES OF EPILEPTIC SEIZURES
Two large types of epileptic seizures have been distinguished, each of which is further divided into smaller groups
Type one, primary generalised epileptic seizures; are seizures in which sudden epileptic discharges in the nerve cells appear simultaneously in both brain hemispheres, without a tangible focal origin of the discharge in one hemisphere. This type of seizures may have one of two forms: 1. primary generalised seizures, sometimes with biting of the tong or the inner cheek and/or involuntary urination; the seizure usually lasts from 1 to 3 minutes and always involves loss of consciousness or 2. loss of consciousness only for several to over a dozen seconds.
Type two comprises several dozen forms of seizures, all of which have focal onset. This means that each of these attacks begins in a specific group of nerve cells, the so-called epileptic focus. Localisation of this focus determines the clinical form of the seizure. We call this type of seizures partial seizures.
There are two forms of partial seizures: 1. partial simple and 2. partial complex. The arbitrary difference between them consists in assessment of the patient's state of consciousness at the time of the seizure. In partial simple seizures the patient is conscious whereas in partial complex seizures there is loss of consciousness. The degree of loss of consciousness may vary depending on the spread of the epileptic discharges.
Partial (focal) seizures may be preceded by an aura. There is no aura in primary generalised seizures. If the epileptic discharges spread over the entire brain, they may lead to a secondary generalised seizure, which usually has a tonic phase, and convulsions. The seizure may be accompanied by involuntary urination and/or tongue biting. Secondary generalised seizures may develop within one to several dozen seconds. This is why observation of the first seconds of the seizure is important for diagnostic purposes. After this period the secondary generalised convulsive seizure has the same dynamic as the primary generalised seizure and usually ends in the patient falling asleep. This sleep should not be disturbed.
As far as diagnosis is concerned, it is the epileptologist's job to 1. determine the type of seizure (seizure diagnostics); 2. determine the localisation of the epileptic focus (localisation diagnostics); 3. determine the etiology of the focus, i.e., the type of pathology harming the brain and thus leading to the development of the epileptic focus (etiological diagnostics).
If seizures repeat several times within e.g. one hour and consciousness is retained between seizures we call these cluster seizures. But if the patient does not regain consciousness between as few as two seizures we call this an epileptic state (status epilepticus). This is a life-threatening condition and requires immediate medical intervention.
TREATMENT OF EPILEPSY
Until recently only a few so-called basic antiepileptic drugs (AEDs) were available but new AEDs (so-called new generation AEDs) can now be bought in Polish pharmacies. All in all, we have about 15 AEDs. Properly selected, regularly taken, in appropriate doses, these drugs help epileptic patients, particularly those who do not improve with the basic AEDs. Nearly all new-generation AEDs are now available in Poland.
Most patients need just one AED to control their seizures but 30-35% need two drugs and a few percent even need three drugs to obtain seizure control. In such cases one must remember that drugs may interact leading to reduced or increased efficacy and toxicity. These patients are called drug-resistant or intractable. In half of this group, addition of a second, new drug may bring considerable improvement, i.e., reduction of the frequency and/or severity of seizures and hence better quality of life. For example, partial simple and partial complex seizures will not generalise to tonic-clonic seizures or simple seizures will not evolve into partial complex ones, or seizures will not last so long, will be less severe, with less loss of consciousness and the overall duration of the seizures will be shorter. This also means that the consequences of the seizures will not be so serious.
It takes great skill to choose the right drug and the right dose. It sometimes takes several months to select the best drug and dosage and in the most difficult cases it may even take more than a year. This is because epilepsy is so heterogeneous with respect to causes of seizures, clinical dynamics, co-morbidity and reactions to medication.
The fact that doctors can now administer a greater variety of new AEDs, with a broad spectre of mechanisms and fewer adverse effects, allows them to make more accurate choices of one or more drugs for a given type of epilepsy. We must also keep in mind that the antiepileptic drug is only part of the treatment; drug-resistant patients must also observe a special life style. It is the doctor's job to determine the patient's individual recommendations, potential and limitations.
THE SOCIAL PERCEPTION OF EPILEPTIC PATIENTS
In their everyday lives epileptic patients struggle with many different objective and subjective problems relating to their condition. Research on patients' satisfaction with their treatment now pays more and more attention to mastery of ways of coping with various psycho-social barriers and prejudices. Increasing attention is being given in the medical literature to such psychological determinants of patient functioning as self-esteem, intellectual proficiency, self-sufficiency, efficacy and sense of independence. Like the clinical course of the disease, these issues contribute to quality and standard of life as experienced by the epileptic patient.
Although the research on social attitudes towards people with epilepsy conducted in developed countries suggests that social attitudes towards epilepsy are continually improving, day-to-day observation often fails to support these optimistic conclusions. Many epileptic patients continue to experience prejudiced treatment, even in economically and culturally developed societies. This leads to reduced self-esteem, stigmatisation and humiliation. And although it is good to know that people with physical disabilities, diabetes and other chronic diseases now evoke compassion and nurturing feelings, epilepsy continues to be a shameful, bizarre and anxiety-provoking condition for reasons which are not at all clear.
British researchers found that in Europe lack of tolerance is greatest in France, Greece and Germany. Poland, meanwhile, together with Spain and Denmark, is one of the most tolerant nations. The researchers also found that the country's economic standard does not have a significant effect on attitudes towards epilepsy. In many countries a long tradition of failure to nurture tolerance and lack of sufficient and accurate information about epilepsy seems to be the cause of undesirable attitudes. One way to overcome these erroneous stereotypes is to modify the inaccurate image of epileptic patients in social perception. There are still many myths and social stereotypes concerning both the causes of epilepsy and the day-to-day functioning of epileptic patients.
Negative attitudes in the community often eliminate epileptic patients from various spheres of life. Social intolerance aggravates feelings of shame and has a demobilising effect on the day-to-day functioning of people who suffer from epilepsy. Quality and standard of living are determined by goodness of fit between the patient's expectations and his or her realistic capacity to function normally. The more we know about the subjective psychological determinants of the patient's situation and its actual limitations, the more likely it is that we will be able to choose the best therapeutic intervention leading to improved well-being and satisfaction with life. Patients' social situation and their subjective appraisal of their capacity to realise life goals often weigh heavily on the progress of treatment. In more affluent and more culturally developed societies there are special education and information programs on epilepsy. The general public is also informed about the conditions of living of epileptic patients. The Polish Society of Epileptology and the Jerzy Majkowski Foundation of Epileptology actively support progress in the treatment of epilepsy and epilepsy research and education. The purpose of this activity is to encourage a reasonable and tolerant attitude towards epileptic patients.
REACTIONS WITHIN THE FAMILY
Epileptic attacks have a very considerable effect on family relations. Parents often disagree as to how their child should be treated or how to cope with the illness and this weighs directly on the sense of family cohesion. Sick children experience this situation acutely. They are often frightened or even terrified and tend to blame themselves for the family problems, thinking that their parents no longer love them. Siblings may be very embarrassed by the whole situation but they may also be jealous of the greater attention given to the sick brother or sister and feel that they are being treated unfairly.
The atmosphere at home has a very powerful effect on the patient's self-esteem. At first, the family resorts to denial. If the patient is free of seizures he or she may forget to take the prescribed medication or deliberately refuse to take it because the need to take medicine is clear evidence that something is wrong. But when seizures recur, denial gives way to anxiety fear of the present and the future.
The family climate is very important for the progress of pharmacotherapy but it weighs most heavily on level of satisfaction, self-esteem and self-sufficiency, all factors contributing to the patient's quality of life. Sociological research and the personal experience of doctors and other people involved in the treatment of epilepsy all indicate that families vary greatly both in their approach to the patient and to the disease itself. Generally speaking, social prejudice or intolerance are reflected in the attitudes of members of the family to the sick patient. Hence the building up of prejudice in the family is caused by pathology in social life. The family may respond in a variety of ways to a member's epilepsy: it may view it as a form of punishment, an embarrassing weakness, a blessing, an enemy, an action strategy, an inevitable loss or an original experience.
Needless to say, this classification, like any other classification, is rooted in generalisations and simplifications. Medical practitioners often confront attitudes which are various combinations of the types listed above. Also, attitudes within the family may evolve as treatment progresses. Sometimes different members of the family have different attitudes. From a practical point of view only the first, task-oriented type of family functioning is acceptable and it is usually a great help and source of support for the doctors, psychotherapists and other people involved in the treatment of epilepsy. Unfortunately such rational attitudes are less frequent in day-to-day clinical practice than the more irrational ones. One significant reason for the proliferation of such stable, dysfunctional approaches is the fact that the family is but one element of a larger, prejudiced, poorly informed society.
The insufficient knowledge on the specific dynamics of various forms of epilepsy unnecessarily increases the limitations which patients experience in their lives. Not only teachers and superiors but also relatives often manifest such improper attitudes. Parents feel responsible for their child's illness and often behave as if they wanted to make up to the child and provide more care and nurture than they give to their other children. They often isolate the sick child from activities in which its peers willingly engage such as climbing trees, swimming, cycling etc. All too often epileptic children and adolescents are excused from gym classes "for their own good" when a reasonable amount of physical exercise could be a valuable form of relaxation and active recreation (we wrote about sports in EPI no. 3/2002). Such a protective or even overprotective approach may be justified to a certain extent in very severe cases of epilepsy but it is counterproductive and psychologically destructive in cases where seizures are well controlled by means of antiepileptic drugs. The reason for these maladaptive attitudes is fear that activity may prove seizures or cause many injuries. One cannot rule out this risk completely but the probability is really very low. Such adverse events are caused much more frequently by failure to take the prescribed medication.
Another problem which families of epileptic patients confront is parental guilt. Such attitudes usually lead to the development of overprotection which restricts the child's freedom. In such cases children often develop a syndrome called learned helplessness or adopt the "sick child" role and are isolated from their peers. Parental guilt may sometimes evoke an opposite response. The parents may deny that the child is ill and hence deprive it of sense of security and support. Because there are so many forms of epilepsy and because the illness has such a variety of dynamics, responses to medication and prognoses, and because attitudes towards epileptic patients differ so widely, it is necessary to find the correct individual balance between restrictions and encouragement or permissiveness.
EPILEPSY AND PREGNANCY
We must make it very clear that the most important factor in the reduction of the risk of complications in pregnancy and childbirth is the earliest possible provision of care to epileptic women (preferably even before they conceive) by a professional team consisting of a neurologist-epileptologist and a gynaecologist-obstetrician. It is also very important that epileptic women follow doctor's instructions very carefully prior to conception or when they are pregnant. Nowadays about 94% of women with epilepsy who are taking antiepileptic drugs give birth to healthy offsprings. The problem of congenital anomalies applies to only a small percentage of foetuses submitted to the teratogenic side effects of antiepilptic drugs in the early stage of organogenesis. Usually adverse effects of drugs are found in cases when more than one AED was being taken and (or) the doses were too large. Therefore, to optimise treatment, doctors should try to reduce the number and doses of AEDs to the essential minimum. In such cases drugs with prolonged action (retard, chrono), now available in Poland, may be very useful. By maintaining a stable concentration of the drug in the serum we avoid the problem of peaking concentrations and can therefore reduce its adverse effects.
The risk of foetal defects increases with the increase in number of AEDs from a two-fold increase for 2 drugs to a 10-fold increase for 4 drugs. One must also bear in mind that 2-3% of healthy women give birth to offspring with congenital defects. .
Epileptic women have many queries relating to pregnancy, childbirth and the health of their offspring. They may turn to the Polish National Epilepsy Centre for Pregnant Women (OCPK) which is affiliated with the Foundation of Epileptology and co-operates with the European Drug and Pregnancy Register (EURAP) where they will receive comprehensive answers to their questions. Every doctor may submit information on his or her patients to OCPK and seek information there. Program participants may receive state of the art professional consultation including EEG and control of AED concentration free of charge. There are also other centres specialising in guidance of pregnant epileptic women in Poland. In Warsaw there is the Epilepsy Clinic for Pregnant Women at the Department of Neurology and Epileptology of the Medical Centre for Postgraduate Education. The Clinic co-operates with Department of Gynaecology and Obstetrics at the Medical Centre for Postgraduate Education. There is also a clinic in Łódź at the Polish Mother's Health Centre and similar clinics in Gdańsk and Poznań. The problems of pregnancy, motherhood and epilepsy were discussed in EPI 4/2002.
COPING WITH EPILEPSY
Coping with epilepsy means knowing how to draw upon one's own resources to overcome the limitations and obstacles resulting from epilepsy and confronted in everyday life. These limitations are caused both by the nature of the disease itself and by its consequences and coping potential is determined by individual resiliency, inventiveness, individual resources and ability to use environmental support. The individual potential for successful adjustment to epilepsy largely depends on the temporal-limbic and sub-thalamic regions of the brain. We must stress that both epilepsy and its treatment may have a considerable effect on these brain structures and therefore the patient's condition and coping strategies may be directly or indirectly negatively affected.
External sources of support such as family, friends and acquaintances as well as governmental and non-governmental organisations may be extremely helpful for the chronically ill. If limitations are too great or potential support is not forthcoming, then coping strategies must be modified accordingly.