Closing Comments

I have been asked to try to summarize what happened in this very interesting workshop. First, I should like to comment on the fact that I think it is an excellent idea to have a workshop on epilepsy and learning disability for the reasons that are familiar to us all. Epilepsy is very common in learning disability and needs skilled management. Learning disability, particularly the more subtle forms, is very common in epilepsy and may often go unrecognized. A brief personal overview of the presentations follows: Cornaggia provided a comprehensive introduction. He raised the problems of definitions and terminology. He discussed the concepts of permanent and state-dependent learning disability. He emphasized that a falling IQ does not necessarily mean loss of skills; it may simply mean that the skills have not been acquired at the expected rate. However, that in itself should cause concern and may be a result of the epilepsy. He gave a tantalizing introduction, suggesting that much more work was necessary, a theme that arose in many of the other talks. Sander, speaking on epidemiology, emphasized the importance of the correct diagnosis of epilepsy. It seems that >50% of those with diagnosis of epilepsy in primary care do not have the condition. We certainly know that some 20% of patients who are referred to specialist clinics do not have epilepsy. The classic work of Jeavons in 1983 illustrated this point. He reexamined >1,000 patients who had a diagnosis of epilepsy, many of whom were said to have intractable epilepsy. He found that 25% did not have epilepsy. With the wisdom of hindsight, it is perhaps not surprising that many of these patients were said to have had intractable epilepsy because, if the diagnosis of epilepsy was incorrect, then they would not be likely to respond to antiepileptic treatment. I do not think that we should use the term “intractable” epilepsy. We should prefer the terms “difficult-to-treat” or “refractory” epilepsy, because they suggest that we still have to take up the challenge of trying to treat it. Sander also referred to types of learning disability that can occur in association with epilepsy, differentiating between congenital and acquired causes. The latter may be acute, for example, meningitis or brain injury, or may occur as a form of more gradual deterioration in intellectual ability over time. He underlined the fact that there are some good data on aspects of learning disability in epilepsy but there are very limited data on specific learning disorders and epilepsy. Some of the very carefully designed population-based epidemiologic studies, such as those by Sillanpaa and Ross, have shown that learning disability and schooling problems are remarkably common, occurring in ∼50% of children with epilepsy. Beghi introduced the suggestion that a new international epidemiologic study should be performed. This is a very ambitious but worthwhile proposal. The aim would be to examine learning disorders and epilepsy. This presentation was followed by very fruitful discussions. In those discussions, it was agreed that the inclusion of a control group would be mandatory because this would overcome so many of the difficulties of intercultural and international differences. In each country, people with epilepsy would be compared with people from the same culture and background, making the results much more valid than they would be otherwise. Although this is an ambitious study, if no one makes the first move, then such an important study will never be performed. Holmes also discussed the question of falling IQ in people with epilepsy. He presented an alarming graph of how IQ may decline with time. He referred to the pioneering work of Meldrum, who showed that seizure activity itself could cause cell damage. It was not necessary to have hypoxia or ischemia, which can also cause cell damage; even in the absence of hypoxia or ischemia, the seizure itself, in an experimental situation, can cause cell damage. Holmes made the additional point, again referring to animal work, that it is not only cell damage that is important. Interruption of the circuits, particularly in the hippocampus, may be very damaging to function. Binnie gave a classic and expert presentation on subtle manifestations of epilepsy, particularly transitory cognitive impairment. The point he emphasized was that we really need to see epilepsy as a spectrum. It varies from the very obvious motor and sensory manifestations of epilepsy, which are readily recognized as seizures, to the very subtle manifestations, which most people do not recognize as seizure activity. If we do not look for the subtle manifestations, we may not find them. In some people, subtle manifestations of epilepsy may be impairing performance and quality of life to a major degree. Hermann discussed language function and epilepsy. He asked the important question: “What are the patterns of academic competence in temporal lobe epilepsy?” He again gave an alarming statistic, suggesting that perhaps ∼50% of adults with temporal lobe epilepsy underachieve. He asked us to assess language dysfunction, which he emphasized is very common in people with temporal lobe epilepsy. If we do not look for it, we may not find it, and if we do not find the deficit, we may not be serving the patient well. Elia gave a very fascinating presentation on some of the chromosomal abnormalities that can be associated with epilepsy. Angelman syndrome has a very characteristic phenotype, with typical facial appearance, behavior, and EEG. People with ring chromosome 20 have a particular problem: frequent and often difficult-to-treat status epilepticus. The fragile-X syndrome has been increasingly recognized in recent years. Only a proportion of people with fragile-X syndrome have epilepsy. This syndrome more commonly occurs in males, although females can be affected. He discussed the very important role of cortical malformations in causing epilepsy. These range from the extreme examples of lissencephaly, with the smooth cortex and very poor brain function, to less obvious syndromes that have been increasingly recognized, such as nodular heterotopia. Piazzini, presenting on depression and anxiety in people with epilepsy, reminded us of the very high rates of these disorders. There appears to be a strong link between depression and left temporal lobe epilepsy. Again, if we are not aware of depression in our patients, we may not treat it, and we may be failing to provide adequate standards of service. Thompson talked about cognitive testing in adults. She underlined the importance of identifying profiles of cognition. This is a very important point because it enables us to put strategies into place that can help these individuals enormously. Even if the deficits are permanent, the outcome of good management can be very positive indeed. She explained that the role of the psychologist covers a broad spectrum, from identifying and providing management strategies for specific profiles of cognition on the one hand, to dispelling prejudice about people with epilepsy and learning disability on the other. Epilepsy is sometimes equated with learning disability. We all know that this is often not the case. Some people with epilepsy have a very high level of cognitive ability. However, it is very important to recognize the pattern of learning disability when deficits do occur. She referred to the sad lack of age-appropriate scales for adults with learning disabilities. There really is a need for psychologists to meet around the table and design good scales that have wide application across the age range and across the ability range. Vargha-Kadhem introduced the fascinating phenomenon of developmental amnesia. This occurs in children who acquire bilateral hippocampus sclerosis through hypoxic–ischemic damage, either early or later in life. Neuroimaging has demonstrated that the hippocampus can shrink down to almost a line on both sides. These children have major problems with delayed recall. Their immediate recall is preserved. She again underlined one of the recurring and most encouraging themes of the conference, the fact that even if a person has a permanent deficit, we can still intervene in a very positive way. There is a very simple and effective intervention with these children, which is repetition. With repetition, the delayed recall, which is very poor, can be improved to almost normal levels. Baker talked about neuropsychological assessments before and after surgery. He also referred to the lack of standardization and the lack of suitable tests for people with learning disability. In the discussion, again the need for international cooperation was emphasized. Even for centers that carry out neurosurgery on large numbers of patients, when the results are stratified for all the variables, there are usually quite small numbers in each of the various subcategories. The best way of finding the answers to some very fundamental questions would be by international collaboration. At present, even for the most commonly performed operations, such as temporal lobectomy, all we can say to patients is, “As a result of surgery, your learning may stay the same, improve, or become worse.” Although we have some indicators that might help to predict the category into which they might fall, we still do not have very good measures of prediction. In one of the most experienced neurosurgical units, in Montreal, 90% of the patients are free of seizures after temporal lobectomy. However, our colleagues still tell us that they do not know why the other 10% are not seizure free. That is the challenge. Hermann has pointed out that there is an important group of patients who seem to have seizures of temporal lobe origin but do not have hippocampal sclerosis on the same side. Many of these patients have quite severe impairments, particularly verbal memory impairments, after the surgery. Furthermore, the seizures are not controlled. Perhaps this is one of the predictors that would suggest poor prognosis. Kerr reviewed evidence-based prescribing in people with epilepsy and learning disability. It is quite shocking to note that there are almost no studies at all on evidence-based prescribing in this group. Again, there is a challenge for us to carry out well-designed studies. Vargha-Kadhem also discussed some specific examples of the outcome of epilepsy neurosurgery. She gave several dramatic examples of a good response to hemispherectomy and also a challenge for us to think of those children who did not perform well after hemispherectomy. This is a life-transforming operation for suitable candidates, but perhaps those with unsuspected bilateral damage may sometimes not have such a good outcome. Her message again was that there is a lack of good standardized preoperative and postoperative tests that provide valid and predictive measures. However, she emphasized that the good-prognosis category of children have very early lesions. In some cases these may be prenatal lesions. There is often a very long delay between making the diagnosis of medically refractory seizures and offering the child neurosurgery. This is probably one of the reasons for the poor results that have been seen in the past. Kerr referred to the importance of multidisciplinary/interagency services and made a number of key points. He underlined that the philosophy of the service is very important. There should be a systematic approach. People with learning disability should have the right to access services and interventions just as anyone else should have. However, there is an added need to have multidisciplinary and multiagency services for people with learning disability. It is also important to include the family in planning and management. The quality of care and social inclusion were other very important aspects in the total package of care. There is the continuing dilemma of respecting the rights of the individual on the one hand but avoiding unreasonable risks on the other. Carrying out a good professional risk assessment is an important element in this aspect of the management of the individual. The term “unreasonable risk” should be emphasized in this context. We all have risks in our lives. The aim is not to limit quality of life excessively by trying to achieve the futile goal of avoiding all risks. The aim is to enhance quality of life while avoiding unreasonable risks. What are the main themes that have been considered at this conference? This meeting has involved an international group of professionals who have an active interest in epilepsy and learning disability. The result has been not only a number of excellent presentations but also some very stimulating discussions. These might help to move our knowledge forward and to act as a stimulus to further research. The overwhelming theme has been the need to be systematic and methodical in approach, both in clinical practice and in research. The fundamental importance of taking a good history, including the history from a good informant who can describe the seizures, was emphasized again. Often bad service to the patient starts there, because people have not undertaken the most basic exercise of obtaining a good history with a clear description of the seizures and have not tried to understand the impact of both the epilepsy and the learning disability on the life of the family. Assessment of the patient should include psychological and psychometric assessment, as well as the appropriate investigations. This leads to a second important theme of the conference, which was that tailored intervention can have a major impact on the life of the individual. Specific strategies to overcome memory difficulties or to compensate for uneven cognitive profiles can be of great benefit. Intervening to improve the situation may depend on the outcome of appropriate EEG investigations. When there is a suspicion that someone may have lost skills, then EEG examination, including overnight EEG if necessary, may be of great relevance. The recognition and treatment of transitory cognitive impairment also falls into the category of very valuable intervention. The third theme was the shocking lack of evidence-based studies, both in the medication that we use to treat our patients and in the models of services we provide. This continues to present a major challenge. I hope that this workshop has been a tremendous impetus and a catalyst to all of us to think of what research we need to perform. If we do not have a good evidence base, we are not really at an adequate starting point. I hope that all of us will be inspired to think of sound research protocols that will help us to deliver systematic, effective services to our patients. If the workshop achieves that, we shall have achieved something very worthwhile indeed.

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