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Epilepsy is a behavioral manifestation of a brain disturbance that causes synchronous firing of large numbers of neurons. Its incidence in the population and the severity of the disability in millions of individuals makes this a serious health problem worldwide. Diagnosis depends on a careful analysis of the person’s behavior and the pattern of neuronal activity in the brain, sometimes supplemented by brain imaging studies. In some individuals, there is no discernible physiological cause of the seizures and a diagnosis of psychogenic seizures will be made, often with the help of someone in the mental health field. Psychologists and social workers often play a role in the choice of treatments and their implementation. Another role of the mental health worker is to point the person with epilepsy or that person’s family to groups in the community and national groups that are dedicated to helping persons with this disorder.
Epilepsy Research Paper Outline
I. Overview
II. Prevalence and Incidence
III. Classification of Epileptic Seizures
IV. Impact on the Individual with Epilepsy
V. Causes and Diagnosis
VI. Nonepileptic Seizures
VII. Effects of Epilepsy on Cognition
VIII. Personality Changes and Behavioral Problems
IX. Treatment Options
X. Epilepsy Advocacy Groups
XI. Future Directions
I. Epilepsy Overview
Unlike most medical conditions, epilepsy is entirely defined by and diagnosed on the basis of publicly observable behavior: the epileptic seizure. Purists will argue that epilepsy is not a disease but rather the symptom of an underlying neurological condition. Usually the seizure disorder is accompanied by an obvious abnormality in the electroencephalogram (EEG), but the presence of abnormal brain waves in the EEG is not a necessary condition for diagnosis. The condition has serious consequences for schooling, psychosocial development, and employment. Life expectancy is also reduced for persons with epilepsy, with mortality rates 2 to 4 times those of matched nonepilepsy persons. Treatment options have increased markedly in the past 50 years, and strictly behavioral treatments have been developed. Opportunities for a normal life with epilepsy are greatly improved compared with even a generation ago, and persons with seizures and their families are being involved increasingly in choosing treatments.
The earliest known descriptions of epilepsy are in the writings of Hippocrates (ca. 460-ca. 377 BC), most notably in his essay titled “On the Sacred Disease,” so named because in ancient Greece, the inexplicable cause of seizures had led to the common belief that the person with epilepsy was periodically possessed by spirits (in more recent times, demons). Hippocrates denounced such interpretations as charlatanism and argued that this was a human disease with physical causes. In this interesting essay he also refers to the shame that is felt by those who have seizures, although it is clear from the following quote that he thought children would seek comfort whereas adults would choose to be alone during the seizure:
But such persons as are habituated to the disease know beforehand when they are about to be seized and flee from men [to a place] where as few persons as possible will see them falling . . . . This they do from shame of the affliction, and not from fear of the divinity, as many suppose. And little children at first fall down wherever they may happen to be, from inexperience. But when they have been often seized, and feel its approach beforehand, they flee to their mothers, or to any other person they are acquainted with, from terror and dread of the affliction, for being still infants they do not know yet what it is to be ashamed. (15)
Many extraordinary individuals have had epilepsy, and writers have for centuries suggested a connection between genius and epilepsy. Although some of the early cases may be better classed as having episodes of insanity (Aristotle, who compiled a list of ancient epileptics, agreed with the Hippocratic view of a close relationship between epilepsy and insanity, which at that time was attributed to black bile), the behaviors described often sound very much like seizures. Intriguingly, some have suggested that an aggressive drive needed for success may be caused by temporal lobe epilepsy. A partial listing of early famous persons with behaviors that may have been epileptic include Pythagoras (b580 BC), Socrates (b470 BC), Alexander the Great (b356 BC), Caesar (bl00 BC), Caligula (b12 AD), and Mohammed (b569). Some later figures for whom epilepsy is clearly established or very likely include Petrarch (b1304), Charles V of Spain (b1500), St. Teresa of Avila (b1515), Cardinal Richelieu (b1585), Louis XIII (b1601), Moliere (Childhood; b1622), Pascal (b1623), Peter the Great (b1672), Handel (b1685), Napoleon (b1769), Paganini (b1782), Byron (b1788), Shelley (b1792), Dickens (Childhood; b1812), Edward Lear (b1812), Dostoyevski (b1821), Flaubert (b1821), Nobel (b1833), William Morris (b1834), Swinburne (b1837), Tchaikovsky (b1840), de Maupassant (b1850), and van Gogh (b1853). Interestingly, epilepsy among Queen Victoria’s relatives in the late nineteenth century led to the founding of the internationally renowned National Hospital for the Paralysed and Epileptic at Queen Square, London (now known as The National Hospital for Neurology and Neurosurgery). It is probably obvious that these famous individuals are mentioned frequently in essays and articles for the general public in order to demythologize the illness and to provide inspiration for persons with epilepsy by showing that a normal or even extraordinary life can be led by persons with the disorder.
II. Prevalence and Incidence of Epilepsy
Estimates of the prevalence of epilepsy in the general population, that is, the ratio of individuals affected to those unaffected, have varied from 2 per 1000 to 50 per 1000. The variability reflects the different definitions of what constitutes epilepsy (e.g., Does the diagnosis require a minimum seizure frequency? Are febrile seizures included? How accurate is the diagnosis?). On average, the most accurate estimates suggest that the prevalence is around 5 per 1000, meaning that in a city of 1,000,000 there would be 5000 individuals with seizures. Thus in the United States, with a population of around 250,000,000, there are approximately 1.5 million individuals with a seizure disorder. Twenty percent of these will have intractable seizures (uncontrollable using current treatments). Over a lifetime, 1 in 20 individuals (5 % ) will have at least one seizure; of those that have a seizure, 12 % will develop chronic epilepsy (this suggests that the prevalence should be .12 x .05 or 6/1000 rather than 5/1000–the discrepancy probably reflects the increased death rate among persons with epilepsy, which is described in more detail in the next section.
The incidence of epilepsy (new cases in a particular time period) has been estimated to be about 0.5 to 1.0 per 1000 per year, meaning that in a group of 1,000,000 individuals there would be about 500 to 1000 new cases each year. Data from studies in Great Britain indicate that most seizure disorders begin in the age range of 0 to 4 years (.75/1000). The next most common ages of onset are 15 to 19 years (.53/1000), 10 to 14 years (.44/1000), and 5 to 9 years (.33/1000). All other age groups have incidence rates below .23/1000. Males and females appear to be equally affected; there are more left-handers with epilepsy than would be expected by chance.
III. Classification of Epileptic Seizures
In 1970, an international group established a classification scheme according to the type of seizure, consisting of four general classes, each with several subcategories, as shown in Table I.
Table I International Classification of Epileptic Seizures
- Partial seizures (seizures beginning locally)
- Partial seizures with elementary symptomatology (generally without impairment of consciousness)
- With motor symptoms (includes Jacksonian seizures)
- With special sensory or somatosensory symptoms
- With autonomic symptoms
- Compound forms
- Partial seizures with complex symptomology (generally with impairment of consciousness); temporal lobe or psychomotor seizures
- With impairment of consciousness only
- With cognitive symptomology
- With affective symptomology
- With “psychosensory” symptomatology
- With “psychomotor” symptomatology (automatisms)
- Compound forms
- Partial seizures secondarily generalized
- Generalized seizures (bilaterally symmetrical and without local onset)
- Absences (petit mal)
- Bilateral massive epileptic myoclonus
- Infantile spasms
- Clonic seizures
- Tonic seizures
- Tonic-clonic seizures (grand mal)
- Atonic seizures
- Akinetic seizures
III. Unilateral seizures (or predominantly)
- Unclassified epileptic seizures (owing to incomplete data)
IV. Impact on the Individual with Epilepsy
Seizures are not just unpleasant and embarrassing experiences for the person with seizures. Epilepsy entails a significant increase in morbidity and mortality. Particularly important for the mental health clinician is the increased risk for serious depression (around 5 % across all kinds of epilepsy and 3 to 4 times the national average; among persons with epilepsy, suicide is listed as cause of death in 7 to 22% of deaths). Studies since the turn of the century have consistently shown that the life expectancy of persons with epilepsy is shorter than in the rest of the population; the death rate of children with epilepsy 0 to 5 years of age is 1.3 times greater than that of the general population; from 5 to 24 years of age, the rate is 6.6 times greater; and from 24 to 25 years of age, the rate is 3.7 times greater. During the period when persons with epilepsy were typically institutionalized, as many as 50% of them died of causes either directly or indirectly associated with their seizures. Among those whose deaths were directly attributed to seizures, about 12% died of status epilepticus (a continuous and uncontrollable seizure); many others died of the accumulative effects of multiple injuries sustained during seizures.
Still important but not directly life-threatening, childhood epilepsy also negatively affects educational achievement and, regardless of the age of onset, has an impact on the person’s eventual employment level. About one third of children with epilepsy receive special educational support, and IQ increases linearly as a function of age of onset (from 83 for adults whose seizures began in infancy to 102 for those with adult onset). Equally serious, and probably not unrelated to the person’s educational experiences, a person’s ability to find employment is greatly reduced by epilepsy. In a 1973 survey, almost one half of persons with epilepsy reported that they had been turned down for a job because of their epilepsy, and 30% reported that they had lost at least one job because of seizures. Various studies over this century have shown that the unemployment rate among working-age persons with epilepsy is between 2 and 7 times the rate of unemployment in the general population. In general, an employer has the right to ask a prospective employee if he or she has any medical condition that will interfere with successful carrying out of the duties, so the reduction in employment opportunities may not be entirely the product of a prejudice against epilepsy itself. Loss of the ability to drive a car is mentioned by most persons with epilepsy as a major loss (the period of being seizure-flee before driving again differs from place to place; e.g., in Michigan, it is 6 months, in Ontario, Canada, I year).
There have been reported cases of birth defects for women who use antiepileptic drugs (AEDs). In the overall population, the rate of birth defects is 2 to 3 %, and the rate is slightly higher (0.5 %) for women with epilepsy who are not taking medication. Women taking a single AED have a risk of about 6 to 7%, with some medications being more problematic than others. Taking several AEDs increases the risk even more. Unfortunately, seizure frequency may go up during pregnancy, so the need for AEDs may even increase. In some cases, the person’s physician may feel that the risks of pregnancy are too great for the mother and child, and recommend that pregnancy be avoided. Finally, some AEDs reduce the effectiveness of oral birth control pills. As discussed later, the genetics of epilepsy suggest that children may inherit a predisposition to epilepsy, but not epilepsy itself. This means, for example, that a head injury would more likely lead to a seizure disorder if the person has close relatives with epilepsy.
Persons with epilepsy frequently suffer consequences when bystanders do not know what to do when they see a seizure occur. It was once believed that a soft object should be inserted between the person’s teeth to prevent biting the tongue during a seizure. This is now highly discouraged. The proper response, if any, depends on the kind of seizure. In general, only a person having a generalized tonic-clonic seizure (grand mal) needs attention, and in this case the greatest help consists of remaining calm, helping the person gently to the floor, and loosening any tight clothing. Hot or sharp objects that could cause harm should be moved away. Placing a cushion or folded piece of clothing beneath the person’s head can reduce the chance of a head injury, and turning the head to one side so the saliva can escape is a good idea. It is useless to try to interrupt the seizure; when the seizure is over, the person may need to rest or sleep. Seizures usually stop within several minutes, but if the seizure continues for 10 minutes or more, or if seizures follow in succession without a period of complete recovery, then medical attention should be sought (this could signal the beginning of status epilepticus). Focal (simple partial) seizures do not require any action on the part of observers. A person having a complex partial seizure should not be restrained unless the person is placing him/herself in danger. As in the case of a generalized seizure, hot or sharp objects should be removed. Partial seizures sometimes progress to a generalized seizure (secondarily generalized), so further precautions may be necessary.
V. Causes and Diagnosis of Epilepsy
When classified according to the known or putative origin of the seizure disorder, there are three etiological categories.
- Symptomatic Epilepsy. Symptomatic epilepsy comprises cases in which the seizures are one of the clinical manifestations of a neurological disorder, such as tuberous sclerosis, Sturge-Weber syndrome, and cerebral degenerative diseases. Alcohol withdrawal seizures would also fit into this category.
- Secondary or Organic Epilepsy. This type of epilepsy results from nonspecific cerebral changes or damage that is permanent and nonprogressive, such as head trauma or perinatal anoxia (i.e., temporary lack of oxygen during a difficult delivery).
- Idiopathic Epilepsy. This epilepsy is also referred to as cryptogenic, essential, pure, primary, or true. These are cases in which the cause is unknown; genetic factors may be involved in the etiology. About 75 to 80% of all cases of epilepsy are of this type.
An enormous amount of work has been dedicated to uncovering the biochemical events that are responsible for an epileptic seizure. It is likely that seizures are caused by a lack of inhibition in the region of the epileptogenic focus rather than by an increase of excitation of the neurons in that area, but both factors may need to be present for the initiation of many if not most seizures. An important factor thought to be involved in the development and expansion of an epileptogenic seizure focus is the so-called “kindling” phenomenon. This was discovered by Goddard at McGill in the 1960s while testing Hebb’s theory of synaptic changes in learning and memory. Goddard found that if a restricted subcortical brain region of a rat was stimulated electrically over many days, an initially innocuous stimulus would eventually lead to seizures, presumably because of the establishment or strengthening of pathways that convey the excitation of the stimulus from a localized area to a more widespread region, and thereby eventually synchronous activity of the whole brain (a seizure). Although the causal connection is still controversial, the development of an epileptogenic focus in another part of the brain has been attributed to this kindling effect, with the “mirror focus” in the homologous position of the opposite hemisphere being a special candidate for this designation.
There are two distinct steps in the process of diagnosing epilepsy. First, the neurologist or general physician has to be convinced that the spells, which are almost always described secondhand by someone not well trained in observation, are characteristic of epilepsy and not some other illness, organic or otherwise. Abnormal EEGs are very helpful in confirming that the seizures probably have a physiological (organic) rather than psychogenic cause, but epileptiform brain activity (i.e., EEG abnormalities characteristic of epilepsy) can also be found in individuals without seizures, and some persons with epilepsy have an apparently normal EEG between seizures. In rare cases, the seizure focus is too deep in the brain to be discerned by scalp electrodes, and the EEG will remain normal even during a seizure. If the spells are determined to be epileptic in nature, their cause needs to be found as that will dictate the appropriate treatment (e.g., medication or surgery). Sometimes the seizure is the first manifestation of the presence of a brain tumor. In this case, the malignancy of the tumor will need to be determined so the benefits of surgery can be weighed. If the seizure occurs in childhood, both the child and the family will need to be educated about epilepsy and the importance of following a medication regimen. In some patient groups, the cessation of seizures under medication control for 2 to 5 years may mean that the medications can be safely stopped without a return to seizures (stopping the medication too soon can lead to a resumption of seizures, which may now be harder to stop, possibly because of kindling effects as described earlier).
VI. Nonepileptic Seizures
The vast majority of seizures have an organic basis, and mental health workers should assume that a patient or client with seizures has no control over their frequency, duration, or form. However, some seizures are nonorganic in origin and will need to be treated by nonmedical therapies. Nonepileptic seizures may be under conscious control (malingering), presumably because of secondary gain, or they may be outside the patient’s control (e.g., Munchausen syndrome) and difficult to understand in terms of a supposed “reward” for the behavior. When the seizure is believed to be nonorganic in origin, it has been called a pseudoseizure or hysterical seizure, although these terms are generally discouraged because their pejorative connotations may interfere with effective communication with the individual and his or her family. One should also be aware of the fact that the majority of nonepileptic seizures occur in individuals who also have organically determined seizures (this probably explains why they are so good at mimicking the seizure behavior).
Withdrawal symptoms from alcohol dependency may occur for several days after abstinence. It is thought that postsynaptic supersensitivity is caused by alcohol’s prolonged inhibition of a particular transmitter system in the brain (the glutamatergic system). With alcohol withdrawal, approximately 15% of individuals will experience seizures, which can be successfully treated or prevented with anticonvulsants and/or sedatives (e.g., diazepam [Valium] or chlordiazepoxide [Librium]). The seizures in such cases are not expected to continue beyond the acute withdrawal period. Of course, individuals with a history of frequent falls during intoxication are at increased risk of seizures from brain damage, and in such cases, seizures may occur as a consequence even without the added effect of withdrawal. Recurrent hospitalization and detoxification appears to lead to increased risk for seizures at a subsequent detoxification, suggesting a kindling effect.
VII. Effects of Epilepsy on Cognition
The performance of epilepsy patients on cognitive tests has been of great help in exploring the brain organization involved in perception, thinking, and reasoning. Memory functions of patients who have a seizure focus in the temporal lobe (or who have had an excision of part of their temporal lobe) have been particularly informative in breaking down memory functions into their component parts. A small number of patients have focal motor seizures that leave consciousness and cognitive abilities intact, but the vast majority of persons with epilepsy lose consciousness and are subsequently amnesic for the events that occurred during the episode. However, unlike the case of most psychiatric illnesses, these effects are discrete in time and the person can be entirely normal between seizures. Of significance for the mental health professional is that there can be lasting behavioral effects of the seizures (especially episodes of continuous seizure activity) and/or the effect of the brain damage that is the cause of the seizures in the first place. Factors to be considered when deciding whether a person is showing effects of a time-related decline in abilities include age of onset, the site of the focus or foci, treatment side effects (medication, surgery), effects of injuries sustained during a seizure (especially head injuries), and effects of status epilepticus or prolonged seizures with inefficient breathing. Age of onset has obvious effects on the acquisition of knowledge and on interpersonal relationships. Sometimes the effects of a seizure focus are direct (lowered attentional abilities), sometimes indirect (missing school for a schoolage child). Overall, significant brain reorganization is much more likely if the brain damage and seizures onset occurs before the age of 6 years than after that age, with puberty providing a likely upper limit for the period of useful plasticity.
VIII. Personality Changes and Behavioral Problems
There has been much written about the so-called “epileptic personality,” some sensible and some bordering on the ridiculous. Lennox and Lennox in their 1960 book, Epilepsy and Related Disorders, point out that many of the personality characteristics supposedly associated with epilepsy are actually frequently seen in many institutionalized patients suffering from a variety of debilitating chronic physical illnesses. Therefore they cautioned against an overarching assumption that the epilepsy itself may cause a personality disorder. Nevertheless, the particular characteristics of epilepsy (sudden loss of consciousness and other bodily functions, uncertainty about when a seizure will occur and what danger it may engender, the attendant problems dealing with family and friends, etc.) make it likely that individuals with seizures will be at increased risk for psychological problems. Joseph A. Schwartz, a psychiatrist who often works with epilepsy patients, has found that consultation requests by neurologists mention personality disorders about 10 times more often for epilepsy patients than for patients with other neurological disorders. He has proposed that the problems experienced by persons with epilepsy be referred to as “social apraxia” in order to remove the moral opprobrium usually associated with the term personality disorder.
The auras themselves may resemble a psychotic experience (25% of auras involve a feeling or sensation; 15 % involve a change in mood). It is not uncommon to become fearful before, during, and after seizures. These emotional concomitants are naturally associated primarily with temporal lobe seizure foci (because of the connection with the limbic system), with mood disturbances being more associated with left-sided foci than with a right-hemisphere foci (approximately 65% vs. 45%). Interestingly, depressed mood is sometimes alleviated after a seizure in these patients, much as electroconvulsive shock has been shown to be helpful in cases of severe intractable depression. Rarely, patients will experience a delayed psychotic episode 12 to 48 hours after a seizure.
For these reasons and others, personality testing is often requested and may be used to help determine the origins of the various facets of the person’s problem (especially in cases of possible nonorganic origins). Psychological assessments of epilepsy patients will naturally be tailored to reflect the needs of the patient and referral question. If the patient is being considered for epilepsy surgery, the assessment will include a full neuropsychological battery, with a focus on memory functions, but also assessing frontal, parietal, and occipital functions (prognosis for successful outcome is greatly improved if the neuropsychological findings point to a disturbance of functions of a single region of the brain). For psychosocial assessments, a commonly accepted instrument is Dodrill’s 1977 Washington Psychosocial Seizure Inventory, a yes/no questionnaire of 132 questions about the epilepsy patient’s feelings and attitudes concerning his or her seizure disorder (e.g., Do your seizures keep you from driving? Are you generally free from depression? Are you concerned people won’t like you or want you around after a seizure?). There are 29 “Critical Items” that may suggest the need for further discussion or action (e.g., Do you often wish you were dead? Do you need vocational counseling?) and eight clinical scales assessing seven psychosocial areas and an overall index of adjustment:
- Family Background (questions about relationships with family members, happiness, and security in the home, etc.)
- Emotional Adjustment (questions about depression, feelings of hopelessness, fatigue, worry, etc.)
- Interpersonal Adjustment (questions about the patient’s relationships with people outside the family)
- Vocational Adjustment (questions about problems at work)
- Financial Status (questions about the patient’s financial resources and feelings of financial security)
- Adjustment to Seizures (questions about attitude toward the seizures, dread of the seizures, etc.)
- Medicine and Medical Management (questions about the patient’s perception of his or her doctors, compliance with medication regimen, etc.)
- Overall Psychosocial Functioning
After scoring the responses, the clinician plots the scores on each of the clinical scales, noting where the major concerns are focused and taking appropriate action if indicated.
IX. Epilepsy Treatment Options
In the fifth century BC, when Hippocrates wrote “The Sacred Disease,” epilepsy was treated by purifications, incantations, and magical spells. Certain foods were forbidden, as were particular kinds of clothing (e.g., goat skin) and fabric colors. Hippocrates thought such “treatments” were fraudulent and argued convincingly (largely on philosophical grounds) that the disease should be treated as any other physical illnesses. He concluded that the disease was hereditary (as discussed earlier, a predisposing hereditary component is in fact present in some cases). Hippocrates’ explanation for the onset of epilepsy seems absurd today, being based as it was on his theories on the effect of phlegm as it moves within the body and the brain (the weather, southerly winds in particular, also played a role in Hippocrates’ explanation, mainly insofar as they affect the production of mucus). His prescribed treatment was vague and appears internally inconsistent:
And in this disease as in all others, he [the physician] must strive not to feed the disease, but endeavor to wear it out by administering whatever is most opposed to each disease, and not that which favors and is allied to it. For by that which is allied to it, it gains vigor and increases, but it wears out and disappears under the use of that which is opposed to it. But whoever is acquainted with such a change in men, and can render a man humid and dry, hot and cold by regimen, could also cure this disease, if he recognizes the proper season for administering his remedies, without minding purifications, spells, and all other illiberal practices of a like kind. (21)
It is likely that further relevant details of treatment were passed from physician to student during medical apprenticeship (some have even suggested that the Hippocratic writings were meant only for lay people and were therefore purposefully uninformative, with treatment secrets reserved for one-on-one training). The prescription of treatments that are considered useless today continued well into this century. For reasons that are no longer clear, at the turn of the century even reputable physicians tried the so-called Corsican treatment, cauterization of the ears. Institutionalization of persons with epilepsy was common until the 1940s and they made up as much as 25% of the residents in institutional settings. This was at least in part the consequence of prejudice against the person with epilepsy, both by physicians and by the general public.
A. Medication (Drug) Therapies
Anticonvulsant medications are numerous and trials of new medications are common. Early attempts to find effective treatments do not sound very scientific by today’s standards (Galen preferred mistletoe, reasoning that it is a plant that grows on oak and therefore resistant to falling, thus useful for the “falling disease,” epilepsy). Bromides (sedatives made of compounds of bromine and another element, such as potassium) gained popularity in the second half of the nineteenth century and helped approximately half the patients, although side effects of long-term use (mental torpor) were unacceptable. The popularity of this drug was so great that around the turn of the century, over a ton of bromides were being delivered yearly to the main hospital treating epilepsy in London, England. In 1912, phenobarbital was found to control seizures better than any substance before it, and many of the current effective medications are variations on the molecular structure of this substance. All anticonvulsants will cause side effects if the dose is high enough, but none of these medications should have unacceptable side effects in the vast majority of patients if the serum levels are kept within the therapeutic range. Around 4% of pediatric patients will need to switch to another anticonvulsant because of the severity of the side effects of the medication.
B. Surgery
Surgical removal of brain tissue or the cutting of intrahemispheric commissures (corpus callosotomy) to eliminate a seizure disorder may seem an extraordinary treatment, but as it has become clear that the origin of the seizure lies in the brain, it has in some cases become the most viable option. Because in most cases epilepsy surgery is elective (i.e., not necessary to preserve life), psychological and neuropsychological findings assume an unusual importance in the decision-making process. Psychologists, social workers, and others in mental health provide essential input into the decision about whether or not to proceed to surgery. For surgery to be considered, the following six conditions should be met:
- The seizures are uncontrollable even with high doses of anticonvulsants. Or if the seizures are controllable with high doses of medication, the side effects at these levels are unacceptable or dangerous. A watershed decision is made at this step to establish candidacy for surgery. Even if condition 1 is satisfied, some patients and their families may not want to proceed further toward brain surgery because of the possible dangers of surgery or in the hope that an effective medication will be developed in the near future.
- The seizures are intolerable to the person experiencing them (sometimes this means that the person may be unemployable or the seizures may be dangerous or extremely unpleasant).
- All or most of the seizures arise from a single focal brain region (i.e., removal of the focus–usually scar tissue within the gray matter of the brain–is likely to eliminate or reduce the seizures because there is no other potential source of seizures).
- This seizure focus is accessible (i.e., it is in a part of the brain that can be exposed during surgery without undue risk to the patient).
- The tissue that would need to be excised can be removed without intolerable consequences for the person with epilepsy (i.e., surgery would not lead to severe loss of cognitive abilities, such as speech or memory).
- The individual has the internal psychological resources and a support network in place to help with postsurgery recovery and a successful transition to nonepilepsy status.
When the first two conditions are met, the patient begins a lengthy process to establish the remaining four conditions. Psychologists (usually neuropsychologists) are involved in conditions 3, 5, and 6. Condition 4 is the province of the neurosurgeon or neurologist. Condition 6 usually requires contributions from a social worker as well as the psychologist and nurse clinician working with the individual. On occasion, a patient will be told that before he or she can progress further toward surgery, an improvement in mood or a strengthening of the support network is needed. Different epilepsy surgery teams may differ in their threshold for this kind of problem.
Condition 3 usually includes a neuropsychological evaluation designed to pinpoint which brain areas are functioning at levels below expectation. This is important because if the pattern of cognitive strengths and weaknesses indicates bilateral involvement or damage to areas outside the region thought to harbor the epileptogenic focus, the prognosis for seizure control after surgery is worse. In the case of temporal lobe epilepsy, the memory functions for verbal and pictorial materials are tested (tapping speech- and nonspeech-hemisphere functions, respectively). Other examinations are usually done at the same time: EEG localization, brain scans, including computerized tomography (CT) or magnetic resonance imaging (MRI), and metabolic positron emission tomography (PET) or single-photon emission computerized tomography (SPECT). The ideal candidate for surgery has findings from all of these examinations that point to the same area of the brain as the probable origin of the seizures. Magnetoencephalography (MEG) is being developed as an alternative to EEG; it is reported to have a localizing accuracy of approximately 8 mm, compared with 10 mm for EEG. The test measures the magnetic field generated by cortical activity by using extremely sensitive detectors (strength of the field is about one billionth that of the earth’s gravitational field).
If conditions 3 and 4 are satisfactorily answered, condition 5 is evaluated by using one or both of the following methods: (1) the Intracarotid Amobarbital Procedure (IAP; sometimes called the Wada Test, after Juhn Wada, the neurologist who introduced the technique at the Montreal Neurological Institute in the 1950s), or (2) speech mapping if the hemisphere to be operated on makes a contribution to normal language production and comprehension.
1. The Intracarotid Amobarbital Procedure
This test is designed to determine the hemispheric organization of speech abilities and to prevent postsurgery amnesia by eliminating candidates whose memory abilities depend on the tissue in the area to be respected. Typically, a small amount of sodium amobarbital (Amytal) is injected into the internal carotid artery, which serves most of the cerebral hemisphere on the same side. After the injection, the neuropsychologist has approximately 5 minutes to test cognitive functions of the nonaffected hemisphere. Language abilities are tested and new information is presented to determine whether memory mechanisms of the noninjected hemisphere are capable of forming new memories. If the person can speak and learn new information after the injection, the noninjected hemisphere is considered able to support language and memory functions.
2. Speech Mapping
If the epileptogenic focus is near regions of the brain that are required for language, the area near the proposed surgical removal needs to be mapped (mapping may also be needed for motor and sensory functions if the removal is near the primary sensory or motor cortices). Electrical stimulation is applied to the brain surface, usually during the surgery in the awake patient, although in some cases it may be preferable to stimulate the region using an implanted electrode array over an extended period before surgery. In the case of language functions, an interruption of speaking or comprehension during stimulation indicates that the underlying cortex should be left intact.
C. Nonmedical Therapies
Physicians rely primarily on medication or surgery to treat their epilepsy patients, but nonmedical treatments of seizure disorders have played at least a peripheral role throughout the history of the illness. On the most superficial level, patients themselves often feel that something in their environment or a modification of their behavior can affect the frequency, duration, or severity of their seizures, both positively and negatively. This knowledge, either because it is accurate or through a superstitious process, leads to modifications in behavior. Rarely, patients actually like the feeling of the aura or seizure and so find ways to trigger one. Visual stimulation, such as passing the fingers back and forth in front of the eyes, can elicit seizures, and flashing lights (photic driving) are used as part of the diagnostic workup, because they can frequently produce abnormalities in the EEG or actual seizures. Patients with visually evoked seizures learn to avoid visual conditions with flashing lights (e.g., strobe-illuminated dance halls) or repetitive moving patterns (e.g., a picket fence). Some people have their seizures elicited by any sudden stimulus that causes a mild startle reaction. Although it is difficult for a person to eliminate entirely the possibility of being startled, those around the patient can learn to reduce unexpected loud noises or sudden movements.
Other non-medication/non-surgical treatments include:
- Behavioral approaches, often used adjunctively. These treatments work best when the activities of the neurologist treating the patient are carried out in concert with the behavioral work. Three types are most common: (1) behavior modification, reward management or reinforcement-based strategies; (2) therapy, self-control or cognitive and psychodynamic-based strategies; and (3) conditioning, psychophysiological methods (desensitization and classical conditioning extinction, of greatest use in reflex epilepsy).
- Biofeedback using the EEG (e.g., individuals with seizures having a motor component may be able to learn to normalize the cortical neural firing patterns and thereby dramatically reduce seizure frequency); biofeedback using exhaled CO2 (hyperventilation, which will cause a seizure in susceptible individuals, leads to a decrease in the CO2 in exhaled breath; learning to increase CO2 levels by means of biofeedback training may act in the opposite direction and decrease the frequency of seizures).
- Special diet. One of these is the ketogenic diet, which is high in lipids (fats) and low in proteins and carbohydrates. This causes a condition in the body called ketosis, which appears to cause a rise in the threshold for seizures. This diet needs to be supervised closely by the person’s physician. Fasting in the short term has the same effect.
- Unconventional and as yet poorly studied treatments include the application of magnetic fields to the scalp over the seizure focus (applying to the brain a magnetic field within the same characteristics as seen when recording over the epileptogenic focus).
X. Future Directions
At the time of this writing, efforts are underway in a number of domains of importance to mental health workers concerned with epilepsy. Persons with epilepsy, after a long history of being fairly passive in their treatment, are beginning to be asked to play a more active or even a central role in the management of their illness. Toward this end, they and their family members are being asked about the impact of their illness with established questionnaires such a the WPSI and the Quality of Life in Epilepsy Inventory; similarly, families are being given a larger role when the physician considers the advisability of discontinuing antiepileptic medication after an interval without a seizure (usually more than 2 years). In cases of epilepsy, as well as other illnesses in which the person’s behavior may modulate the progression of the disease process, the increasingly active role that members of the public are playing in determining the nature of their medical care is a positive one that should be encouraged by mental health professionals.
Bibliography:
- Dam, M. & Gram, L. (Eds.). (1991). Comprehensive epileptology. New York: Raven.
- Epilepsy Foundation of America (1975). Basic statistics on the epilepsies. Philadelphia: Davis.
- Hippocrates (1964). The sacred disease. In The theory and practice of medicine [reprint of Francis Adams (1849)translation] pp. 356-357. New York: Philosophical Library.
- Lennox, W. G. (1960). Epilepsy and related disorders (with the collaboration of Margaret Lennox). Boston: Little, Brown.
- Mostofsky, D. I., & Loyning, Y. (1993). The neurobehavioral treatment of epilepsy. Hillsdale, NJ: Lawrence Erlbaum.
- Sackellares, J. C., & Berent, S. (Eds.). (1996). Psychological disturbances in epilepsy. Boston: Butterworth-Heinemann.
- Sands, H. (1983) Epilepsy: A handbook for the mental health professional. New York: Bruner/Mazel.
- Temkin, O. (1971). The falling sickness: A history of epilepsy from the Greeks to the beginnings of modern neurology (2nd ed.). Baltimore: Johns Hopkins.
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