Epilepsia. 3l(Suppl. 4):S21-S25, 1990 Raven Press, Ltd.. New York 0 International League Against Epilepsy
Education and Epilepsy: Assessment and Remediation 0.Henriksen The National Centerfor Epilepsy, Sandvika, Norway
Summary: Learning difficulties in children with epilepsy
form discharges on cognitive function and also help to evaluate the effects of antiepileptic drugs. Objective assessment of subclinical epdeptiform activity makes it easier to treat the pathology identified by the EEG with optimal dosage of the most appropriate drug. A balance is required because epileptiform discharges and even occasional seizures may be less disabling than side effects from large doses of several drugs. Information to the school and the parents concerning the patient’s abilities and limitations may be as important as seizure control. Specialized teaching should be started early, when necessary. with the patient integrated into a normal school if possible. However, good functioning in a special school is prefmble to marginal functioning in a normal school. Key Wwds: EpilepsySeiizures-Children-Learning disability-Education.
may be caused by brain damage and should be investigated. In many cases, however, seizures and/or electroencephalographic (EEG) findings are the only signs of pathology. Frequency and type of seizures may be determining factors that should, if necessary, be evaluated by long-term EEG monitoring, preferably during school performance or in conjunction with neuropsychologicalassessment. This may prove that subclinical epileptiform discharges in the EEG can adversely affect the child’s performance. Secondary psychological problems in epilepsy patients, combined with side effects of antiepileptic drugs, may cause or heighten learning problems. Prophylactic control of seizures with one appropriate drug may alleviate learning problems. Computerized neuropsychological testing with simultaneous EEG recording may reveal the influence of epilepti-
Development Study found that only 67% of 64 children with epilepsy attended ordinary schools at the age of 1 1 years, compared to 58% at the age of 15 years. Attending an ordinary school, however, does not imply that there are no school problems. Holdsworth and Whitmore (1974) found that more than 5096 of 85 children with epilepsy were reported by their teachers as ‘‘just holding their own” in ordinary schools, at a below-average level, whereas one-sixth had fallen seriously behind. Although the average I.Q. in children with epilepsy may be lower than in a matched control group (Farwell et al., 1985), about one-sixth of children with epilepsy who have normal intelligence appear to be underachievers (Pazzaglia and Frank-Pazzaglia, 1976). In this study, the underachievement was attributed to depressive reactions triggered by classmates’ fear of seizures and by the hostility of their parents after a generalized tonic-clonic seizure had occurred in school. Given that the prevalence of epilepsy in children ranges fiom 4 to 8 per 1,O00 (Blom et al., 1970 most schools will have several pupils with epilepsy at any time. It is therefore important that teachers are in-
Among unselected patients with epilepsy, there are great variations in both mental and psychosocial functioning. Studies by Krohn (1961) and others show that approximately 60% of epilepsy patients function well in society, 25% are somewhat incapacitated by psychosocial problems but integrated into society, and the remaining 15% need continuous care, not necessarily because of epilepsy, but for additional handicaps such as cerebral palsy, mental retardation, or psychiatric illness. Although the majority of children with epilepsy attend ordinary schools, various studies (Cavazutti, 1980; Ross et al., 1980) show that learning disabilities and school problems are frequent. It is difficult to estimate the size of the problem because of the heterogeneity of the disorder and its causesand methodological differences in the studies performed. Hanison and Taylor ( 1976) found that 90-956 of children with epilepsy were attending ordinary schools, whereas Verity and Ross (1985) in the National Child
Address correspondence and reprint requests to Dr. 0. Henriksen at The National Center For Epilepsy, N-1301 Sandvika,
Norway.
s2 I
s22
0.HENRIKSEN
formed about the disorder and its management, if they are to help in the assessment and remediation of children with epilepsy.
ETIOLOGY Anyone who has experienced two or more spontaneous epileptic seizures is said to have epilepsy. AIthough the pathological substrate lies within the brain, specific causative lesions are not often demonstrable, even by modem techniques, and the etiopathogenesis is seldom fully understood. Thorough investigation should nevertheless be camed out to diagnose the cause of the epilepsy whenever possible. If an acute cause such as infection or a brain tumor is present, immediate antibiotic treatment, brain surgery, and/or radiation may be indicated. If the epilepsy is caused by a progressive disease, early diagnosis has great significance for further evaluation. prognosis, and treatment. Whether or not the cause of the epilepsy can be identified, it is important to establish the location and extent of any underlying lesion, as this may indicate the sort of specific leaming disabilities to expect and lead to more appropriate management. The most important diagnostic tools are a detailed medical history, full clinical and neurological examination, cerebrospinal fluid examination, brain imaging with computed tomographic scan or nuclear magnetic resonance, an electroencephalogram (EEG), and neurological assessment.
CLASSIFICATION OF EPILEPSY IN RELATION TO LEARNING SITUATION The various kinds of epileptic seizures and syndromes may affect learning ability in different ways. An abbreviated version of the International Classification of Epilepsies and Epileptic Syndromes (Commission, 1989) is helpful in subdividing both localized and generalized forms of epilepsy into those that are idiopathic (i.e., of unknown cause) or symptomatic (with a demonstrable brain lesion). In many cases of primary or idiopathic epilepsy, the age of onset, the evolution of seizure patterns, and the EEG findings enable us to classify the seizures and/or syndrome. This is important because it determines the choice of treatment and enables a prognosis to be made-both of which have a bearing on the child’s educational development. The prognosis in epilepsy ranges from full seizure remission after a few years to persistent seizures intractable to all known medication. Although some idiopathic epilepsies-such as benign partial epilepsy of childhood, or childhood absence epilepsy-have a Epilepsia. V d . JI. Suppl. 4. 1990
favorable prognosis, children with intractable epilepsy often require special education, and may even need institutional care because of progressive educational and psychosocial handicaps combined with a devastating seizure problem. Unfavorable develop ment frequently occurs in children suffering from symptomatic epileptic syndromes. Patients with demonstrable brain lesions and early onset of seizures usually fall into these categories. They often have multiple seizure types, which may include partial seizures with or without becoming secondarily generalized, generalized tonic-clonic seizures. myoclonic seizures, atonic seizures (drop attacks). and atypical absence seizures. Children who begin having generalized seizure (absence and/or generalized tonic-clonic seizures) at about 5-7 years of age without any demonstrable brain lesion usually have a good prognosis. Children with simple partial and complex partial seizures fall into different prognostic categories. They more oRen remain intractable and sometimes have substantial learning problems, which may be related to the site of the epileptic focus. Even though the brain lesion may not be radiologically demonstrable. patients with complex partial seizures and temporal lobe EEG foci tend to have more learning difficulties(Stores, 198 1): boys with left temporal foci often have impaired reading skills while those with foci in the nondominant hemisphere fail on practical performance. It should be stressed, however, that the distinction between the various prognostic groups is somewhat vague. Many patients fall between groups and cannot be categorized with certainty. Each child must therefore be evaluated individually.
ROLES OF EEC AND FUNCTIONAI. TESTING IN RE1,ATION TO LEARNING Besides being a tool for identifying epilepsy, the EEG is useful in locating the epileptic focus, classifying the seizures, quantifying the occurrence of paroxysmal discharges, and categorizing the epileptic syndrome. Most patients with epilepsy have interim1 discharges in the EEG but these are highly variable, not from patient to patient but over time in a given patient. The frequency of epileptic discharges, as well as manifest seizures, is influenced by time of day. stress. activity, wakefulness, drowsiness or sleep. and by testing and school activities. Therefore. an ordinarq 20-min EEG recording does not necessarily give a representative picture of the situation. With modem equipment, it has become more and more common to make long-term recordings. sometimes with simul-
ASSESSMENT A h’D RE.ZfEDI.4 T10h’ taneous video monitoring and telemetry. This. combined with a computerized neuropsychological test battery. makes it possible to demonstrate the relationship between epileptic discharges and various activities and to evaluate whether such discharges influence performance (Stores 1987: Binnie. 1988). Intenctal discharges or subclinical discharges may not be subclinical in the sense that they influence cognitive functioning and motor responses. In a child with frequent interictal discharges, disturbance of cognitive functioning and learning may therefore be expected. However, functional testing is required because the amount of epileptic activity in a standard EEG, where the patient is lying down with eyes closed, does not necessarily reflect the situation at school. EEG plus video monitoring and telemetry in conjunction with cognitive activities should be considered before drug therapy is prescribed in some cases. At the National Center for Epilepsy in Norway. over the past 5 years, we have been investigating patients using a computerized neuropsychological test battery with simultaneous video-EEG telemetry (Rugland et al., 1987). Some patients have been referred because of frequent epileptiform activity in the EEG, even though they have never had overt seizures. When generalized epileptiform discharges have been shown to affect patients’ performance, treatment with valproate has resulted in an improvement proportional to the reduction in epileptic discharges. The consequences of epileptiform discharges vary from patient to patient. Short (1-2 s) generalized discharges may occur without apparent effect on performance, whereas focal discharges of even shorter duration sometimes influence a patient’s response. However, these findings suggest that all epileptiform discharges disturb functioning to some extent if the test batteries are sufficiently sensitive. ATTENTION-DEFICIT DISORDERS AND EPILEPSY Attention-deficit disorder is occasionally encountered in patients with epilepsy. In a group of children selected because of frequent epileptiform discharges without clinical seizures, we have occasionally noticed that antiepileptic medication has a limited effect on cognitive functioning, probably due to intrinsic inattention. Tests have been designed to show the effect of amphetamines on the children. who are monitored with a computerized neuropsychological test battery that measures reaction time. The results are not affected by repeated testing, and placebo is used to demonstrate the effect of amphetamines as
S23
well as to facilitate titration of an optimal stimulant dose. SECONDARY PSYCHOSOCIAL PROBLEMS Psychosocial problems are common among children with epilepsy. They may be caused partly by school problems and partly by the seizures. Conversely, psychosocial problems may increase seizure frequency and school problems. Certain children are therefore caught up in a vicious circle, with seizures leading to learning difficulties, and then to school problems, psychosocial problems, and more seizures. The problems are magnified if the child also has attention-deficit disorder. To complicate the picture, many patients with epilepsy experience side effects from antiepileptic medication unless the seizures are controlled with a small dose of a single drug. High doses and more than one drug increase the risk of side effects. Several studies (Thompson and Trimble, 1982; Tomlinson et al., 1982; Rugland et al.. 1987) have shown how this combination may adversely influence cognitive function and learning abilities. Many children with an intractable epilepsy therefore have to cope not only with seizures and subclinical epileptiform activity in the EEG but also with brain damage and drug side effects and psychosocial problems-a truly vicious cycle. PSYCHOLOGICAL AND EDUCATIONAL ASSESSMENT Each of the factors mentioned above is of potential importance to a child with epilepsy. A proper assessment of this complex situation may therefore be very difficult, requiring the collaboration of a team consisting of a physician (epileptologist), a psychologist. and a teacher. Typically, such teams exist only at specialized epilepsy centers or clinics. Children with a difficult seizure problem and learning difficulties should therefore be referred to a center of this type to insure the best possible assessment. Although most children with epilepsy benefit from medication, excessive medication may cause learning difficulties, even in a well-functioning child with benign absence seizures. REMEDIATION When treating a child with epilepsy, the doctor’s first duty is to look for the cause and alleviate it if possible. If the patient still has seizures, the epilepsy must be classified so that treatment with appropriate antiepileptic drugs can be started, always at the lowest possible dose. The doctor must always be on the
0.HENRIKSEN
S24
lookout for side effects, especially if high dosages are needed to control the seizures. In such cases, neuropsychological evaluation is indicated. Sometimes the addition of a second or even a third drug may control the seizures, but in these situations it is necessary to insure that side effects do not replace the seizures. Subtle changes in alertness and cognition caused by medication may be difficult to detect, especially if the child originally suffered from frequent brief seizures. These symptoms may be demonstrated by a computerized test battery that can be administered repeatedly and which is therefore a fine tool for optimizing the dose of medication. It may sometimes be preferable for the patient to have some seizures with a moderate dosage of a single drug rather than be seizure free with massive dosages of several drugs. Very few clinicians have access to a computerized test battery and may therefore have to rely on close follow-up and cooperation between the physician, psychologist, teacher, and parents. Such teamwork may disclose adverse drug effects that might otherwise remain undiagnosed and affect the child’s learning abilities for Y.-
Whether or not to treat is a complex question. Before starting treatment, the potential side effects of antiepileptic medication should be considered. On the other hand, the earlier epilepsy is controlled, the better the prognosis. If subclinical epileptic activity shown by the EEG is treated, the treatment must be monitored with a computerized neuropsychological test battery. Treatment should be continued only if benefits of reduced epileptiform activity can be Jemonstrated and if adverse cognitive effects of the drug can be avoided.
INFORMATION TO SCHOOLS AND PARENTS Thorough information is necessary to prevent or alleviate psychosocial problems (Henriksen, 1985). Communication begins with the patients and their parents, but teachers and peers should not be forgotten. Watching a friend in school having a generalized tonic-clonic seizure is a frightening experience that can have an unfavorable effect on the attitude of peers and their parents. This can be prevented if everyone is well informed beforehand. The label “epilepsy” may have a masking effect, causing both the patient and the teacher to blame all of the child’s problems on the epilepsy. Parents, in particular, may have difficulties in realizing and accepting that problems other than epilepsy are present. If a child is mentally retarded and needs special
schooling, prompt and extensive counseling is often required. The teacher and psychologist, in collaboration, are responsible for diagnosing specific learning disabilities as early as possible and organizing special education when needed. A child with epilepsy can have any form of learning disorder, and a variety of remedial measures may need to be considered (Strang, 1987). Neuropsychological evaluation should be comprehensive and interpreted from a broad perspective, taking into account the child’s ability in relation to his/her home, the community, and the school demands. This assessment forms the bask for appropriate remedial education. If these problems are successfully diagnosed and treated, the child can be motivated to learn, self-esteem improves, and the psychosocial problems diminish. Although integration of handicapped children into ordinary schools is preferred, good functioning in a special school may be better than marginal functioning in an ordinary school. Environmental demands need to be balanced with the child‘s ability, in order to optimize self-esteem, learning potential, and psychosocial functioning. A mentally retarded child may thus be happier at a special school where peers have similar problems. Unrealistic aspirations for a child only serve to reduce self-esteem, and may depress learning, increase seizure frequency, and lead to social isolation. Appropriate psychosocial treatment can reverse a vicious cycle of seizures, learning difficulties, and psychosocial problems. An evaluation done in 1986 in the Children’s Department at the Norwegian National Center for Epilepsy showed that eight children became seizure free because of psychological and educational remediation, while medication remained unchanged (Skaare, 1989). Whether a child with epilepsy has a high I.Q. or is mentally retarded, much can be gained through early diagnosis, adequate treatment, and thorough information to all concerned.
REFERENCES Binnie CD. Seizures, EEG discharges and cognition. In: Trimble MR, Reynolds EH, eds. Epilepsy. behaviour and cognitive funcrion. Chichester: John Wiley & Sons 1988:45-9. Blom S, Heijbel J, Bergfors ffi. Incidence of epilepsy in children. Epilepsia I978;19343-50. Cavazutti GB. Epidemiology of different types of epilepsy in xhool-age children of Mcdena, Italy. Epilepsia 19802 157-62. Commission on Classification and Terminology of the Intemational League Against E p i l w . RoDosal for revised classification of epilep&es and epileptic syndromes. Epilrpsro 1989;30:389-99. Fatwell JR, Dodrill CB,Batzel LW. Neumpsychological abilities of children with epilepsy. Epikpsia 1985:26:395-400.
-’tt-
ASSESSMENT AND REMEDIATION Harrison RM, Taylor DC.Childhood seizures: a 25 year follow up. Social and medical prognosis. Loncet 1976;1:948-5I . Henriksen 0.The importance of information of patients and relatives. In: Dam M, Gram L, Pedersen B, 0 m m H, eds. Modern approach to antiepileptic drug treatment. Copenhagen: The Danish Epilepsy Society, 1985:55-60. Holdsworth L, Whitmore K. A study of children with epilepsy attending ordinary schools. I: Their seizure patterns, progress and behaviour in school. Dev Med Child Neurol 1974;16:74658. Krohn W. A study of epilepsy in Northern Norway; its frequency and character. Acta Psychiatr Neurol Scand I961;36(suppl 150):25-225. Pazzaglia P, Frank-Pazzaglia L. Record in grade school of pupils with epilepsy: an epidemiological study. Epilepsia 1976;
I7:361-6. Ross EM, Peckham CS, West PB, Butler NR. Epilepsy in childhood: findings from the National Child Development Study. Br Med J 1980;1:207-10. Rugland AL, Bjernaes H, Henriksen 0, LByning A. The develop ment of computerized tests as a routine p r d u r e in clinical EEG practice for the evaluation of cognitive changes in patients with epilepsy. 17th Epilepsy International Congress, 1987,A b stract, p. 102.
w-
S25
Skaare R. Effekt av tverrfaglig behandlingsopplegg sett fix pedagogisk synsvinkel. Abstract from 7. Skandinaviska Epilepsikongressen in T y l h n d , April 20-22. 1989. Stores G. Learning and emotional problems in childm with epilepsy. In: Reynolds EH, Trimble MR, eds. Epilepsy andpsychiatry. London: Churchill Livingstone, 1981:293-3 10. Stores G. Effect on learning of "subclinical" seizure discharge. In: Aldenkamp AP, Alpherts WCJ, Meinardi H, Stores G, eds. Education and epilepsy. Lisse/Berwyn: Swets & Zeitlinger,
1987:14-20. Strang JD. Educational and related treatment considerations concerning the child with epilepsy: a developmental neuropsychological approach. In: Aldenkamp AP, Alpherts WU, Meinardi H, Stores G, eds.Education and epilepsy. Lisse/Berwyn: Swets & Zeitlinger, 1987:118-34. Thompson PJ, Trimble MR. Antimnvulsant drugs and cognitive functions. Epilepsia 1982;23:531-44. Tomlinson LL, Andrewes DG, Memfield E, Reynolds EH. The effects of antiepileptic drugs on cognitive and motor functions. Br J Clin Pracl 1982;18(suppl):177-83. Verity CM, Ross EM. Longitudinal studies of children's epilepsy. In: Ross E, Reynolds E, eds. Paediatricperspectiveson epilepsy. Chichestex John Wiley & Sons Ltd., 1985:133-9.
Epilcpsra Vd.31, Supd 4. 1990
Education and Epilepsy: Assessment and Remediation 0.Henriksen The National Centerfor Epilepsy, Sandvika, Norway
Summary: Learning difficulties in children with epilepsy
form discharges on cognitive function and also help to evaluate the effects of antiepileptic drugs. Objective assessment of subclinical epdeptiform activity makes it easier to treat the pathology identified by the EEG with optimal dosage of the most appropriate drug. A balance is required because epileptiform discharges and even occasional seizures may be less disabling than side effects from large doses of several drugs. Information to the school and the parents concerning the patient’s abilities and limitations may be as important as seizure control. Specialized teaching should be started early, when necessary. with the patient integrated into a normal school if possible. However, good functioning in a special school is prefmble to marginal functioning in a normal school. Key Wwds: EpilepsySeiizures-Children-Learning disability-Education.
may be caused by brain damage and should be investigated. In many cases, however, seizures and/or electroencephalographic (EEG) findings are the only signs of pathology. Frequency and type of seizures may be determining factors that should, if necessary, be evaluated by long-term EEG monitoring, preferably during school performance or in conjunction with neuropsychologicalassessment. This may prove that subclinical epileptiform discharges in the EEG can adversely affect the child’s performance. Secondary psychological problems in epilepsy patients, combined with side effects of antiepileptic drugs, may cause or heighten learning problems. Prophylactic control of seizures with one appropriate drug may alleviate learning problems. Computerized neuropsychological testing with simultaneous EEG recording may reveal the influence of epilepti-
Development Study found that only 67% of 64 children with epilepsy attended ordinary schools at the age of 1 1 years, compared to 58% at the age of 15 years. Attending an ordinary school, however, does not imply that there are no school problems. Holdsworth and Whitmore (1974) found that more than 5096 of 85 children with epilepsy were reported by their teachers as ‘‘just holding their own” in ordinary schools, at a below-average level, whereas one-sixth had fallen seriously behind. Although the average I.Q. in children with epilepsy may be lower than in a matched control group (Farwell et al., 1985), about one-sixth of children with epilepsy who have normal intelligence appear to be underachievers (Pazzaglia and Frank-Pazzaglia, 1976). In this study, the underachievement was attributed to depressive reactions triggered by classmates’ fear of seizures and by the hostility of their parents after a generalized tonic-clonic seizure had occurred in school. Given that the prevalence of epilepsy in children ranges fiom 4 to 8 per 1,O00 (Blom et al., 1970 most schools will have several pupils with epilepsy at any time. It is therefore important that teachers are in-
Among unselected patients with epilepsy, there are great variations in both mental and psychosocial functioning. Studies by Krohn (1961) and others show that approximately 60% of epilepsy patients function well in society, 25% are somewhat incapacitated by psychosocial problems but integrated into society, and the remaining 15% need continuous care, not necessarily because of epilepsy, but for additional handicaps such as cerebral palsy, mental retardation, or psychiatric illness. Although the majority of children with epilepsy attend ordinary schools, various studies (Cavazutti, 1980; Ross et al., 1980) show that learning disabilities and school problems are frequent. It is difficult to estimate the size of the problem because of the heterogeneity of the disorder and its causesand methodological differences in the studies performed. Hanison and Taylor ( 1976) found that 90-956 of children with epilepsy were attending ordinary schools, whereas Verity and Ross (1985) in the National Child
Address correspondence and reprint requests to Dr. 0. Henriksen at The National Center For Epilepsy, N-1301 Sandvika,
Norway.
s2 I
s22
0.HENRIKSEN
formed about the disorder and its management, if they are to help in the assessment and remediation of children with epilepsy.
ETIOLOGY Anyone who has experienced two or more spontaneous epileptic seizures is said to have epilepsy. AIthough the pathological substrate lies within the brain, specific causative lesions are not often demonstrable, even by modem techniques, and the etiopathogenesis is seldom fully understood. Thorough investigation should nevertheless be camed out to diagnose the cause of the epilepsy whenever possible. If an acute cause such as infection or a brain tumor is present, immediate antibiotic treatment, brain surgery, and/or radiation may be indicated. If the epilepsy is caused by a progressive disease, early diagnosis has great significance for further evaluation. prognosis, and treatment. Whether or not the cause of the epilepsy can be identified, it is important to establish the location and extent of any underlying lesion, as this may indicate the sort of specific leaming disabilities to expect and lead to more appropriate management. The most important diagnostic tools are a detailed medical history, full clinical and neurological examination, cerebrospinal fluid examination, brain imaging with computed tomographic scan or nuclear magnetic resonance, an electroencephalogram (EEG), and neurological assessment.
CLASSIFICATION OF EPILEPSY IN RELATION TO LEARNING SITUATION The various kinds of epileptic seizures and syndromes may affect learning ability in different ways. An abbreviated version of the International Classification of Epilepsies and Epileptic Syndromes (Commission, 1989) is helpful in subdividing both localized and generalized forms of epilepsy into those that are idiopathic (i.e., of unknown cause) or symptomatic (with a demonstrable brain lesion). In many cases of primary or idiopathic epilepsy, the age of onset, the evolution of seizure patterns, and the EEG findings enable us to classify the seizures and/or syndrome. This is important because it determines the choice of treatment and enables a prognosis to be made-both of which have a bearing on the child’s educational development. The prognosis in epilepsy ranges from full seizure remission after a few years to persistent seizures intractable to all known medication. Although some idiopathic epilepsies-such as benign partial epilepsy of childhood, or childhood absence epilepsy-have a Epilepsia. V d . JI. Suppl. 4. 1990
favorable prognosis, children with intractable epilepsy often require special education, and may even need institutional care because of progressive educational and psychosocial handicaps combined with a devastating seizure problem. Unfavorable develop ment frequently occurs in children suffering from symptomatic epileptic syndromes. Patients with demonstrable brain lesions and early onset of seizures usually fall into these categories. They often have multiple seizure types, which may include partial seizures with or without becoming secondarily generalized, generalized tonic-clonic seizures. myoclonic seizures, atonic seizures (drop attacks). and atypical absence seizures. Children who begin having generalized seizure (absence and/or generalized tonic-clonic seizures) at about 5-7 years of age without any demonstrable brain lesion usually have a good prognosis. Children with simple partial and complex partial seizures fall into different prognostic categories. They more oRen remain intractable and sometimes have substantial learning problems, which may be related to the site of the epileptic focus. Even though the brain lesion may not be radiologically demonstrable. patients with complex partial seizures and temporal lobe EEG foci tend to have more learning difficulties(Stores, 198 1): boys with left temporal foci often have impaired reading skills while those with foci in the nondominant hemisphere fail on practical performance. It should be stressed, however, that the distinction between the various prognostic groups is somewhat vague. Many patients fall between groups and cannot be categorized with certainty. Each child must therefore be evaluated individually.
ROLES OF EEC AND FUNCTIONAI. TESTING IN RE1,ATION TO LEARNING Besides being a tool for identifying epilepsy, the EEG is useful in locating the epileptic focus, classifying the seizures, quantifying the occurrence of paroxysmal discharges, and categorizing the epileptic syndrome. Most patients with epilepsy have interim1 discharges in the EEG but these are highly variable, not from patient to patient but over time in a given patient. The frequency of epileptic discharges, as well as manifest seizures, is influenced by time of day. stress. activity, wakefulness, drowsiness or sleep. and by testing and school activities. Therefore. an ordinarq 20-min EEG recording does not necessarily give a representative picture of the situation. With modem equipment, it has become more and more common to make long-term recordings. sometimes with simul-
ASSESSMENT A h’D RE.ZfEDI.4 T10h’ taneous video monitoring and telemetry. This. combined with a computerized neuropsychological test battery. makes it possible to demonstrate the relationship between epileptic discharges and various activities and to evaluate whether such discharges influence performance (Stores 1987: Binnie. 1988). Intenctal discharges or subclinical discharges may not be subclinical in the sense that they influence cognitive functioning and motor responses. In a child with frequent interictal discharges, disturbance of cognitive functioning and learning may therefore be expected. However, functional testing is required because the amount of epileptic activity in a standard EEG, where the patient is lying down with eyes closed, does not necessarily reflect the situation at school. EEG plus video monitoring and telemetry in conjunction with cognitive activities should be considered before drug therapy is prescribed in some cases. At the National Center for Epilepsy in Norway. over the past 5 years, we have been investigating patients using a computerized neuropsychological test battery with simultaneous video-EEG telemetry (Rugland et al., 1987). Some patients have been referred because of frequent epileptiform activity in the EEG, even though they have never had overt seizures. When generalized epileptiform discharges have been shown to affect patients’ performance, treatment with valproate has resulted in an improvement proportional to the reduction in epileptic discharges. The consequences of epileptiform discharges vary from patient to patient. Short (1-2 s) generalized discharges may occur without apparent effect on performance, whereas focal discharges of even shorter duration sometimes influence a patient’s response. However, these findings suggest that all epileptiform discharges disturb functioning to some extent if the test batteries are sufficiently sensitive. ATTENTION-DEFICIT DISORDERS AND EPILEPSY Attention-deficit disorder is occasionally encountered in patients with epilepsy. In a group of children selected because of frequent epileptiform discharges without clinical seizures, we have occasionally noticed that antiepileptic medication has a limited effect on cognitive functioning, probably due to intrinsic inattention. Tests have been designed to show the effect of amphetamines on the children. who are monitored with a computerized neuropsychological test battery that measures reaction time. The results are not affected by repeated testing, and placebo is used to demonstrate the effect of amphetamines as
S23
well as to facilitate titration of an optimal stimulant dose. SECONDARY PSYCHOSOCIAL PROBLEMS Psychosocial problems are common among children with epilepsy. They may be caused partly by school problems and partly by the seizures. Conversely, psychosocial problems may increase seizure frequency and school problems. Certain children are therefore caught up in a vicious circle, with seizures leading to learning difficulties, and then to school problems, psychosocial problems, and more seizures. The problems are magnified if the child also has attention-deficit disorder. To complicate the picture, many patients with epilepsy experience side effects from antiepileptic medication unless the seizures are controlled with a small dose of a single drug. High doses and more than one drug increase the risk of side effects. Several studies (Thompson and Trimble, 1982; Tomlinson et al., 1982; Rugland et al.. 1987) have shown how this combination may adversely influence cognitive function and learning abilities. Many children with an intractable epilepsy therefore have to cope not only with seizures and subclinical epileptiform activity in the EEG but also with brain damage and drug side effects and psychosocial problems-a truly vicious cycle. PSYCHOLOGICAL AND EDUCATIONAL ASSESSMENT Each of the factors mentioned above is of potential importance to a child with epilepsy. A proper assessment of this complex situation may therefore be very difficult, requiring the collaboration of a team consisting of a physician (epileptologist), a psychologist. and a teacher. Typically, such teams exist only at specialized epilepsy centers or clinics. Children with a difficult seizure problem and learning difficulties should therefore be referred to a center of this type to insure the best possible assessment. Although most children with epilepsy benefit from medication, excessive medication may cause learning difficulties, even in a well-functioning child with benign absence seizures. REMEDIATION When treating a child with epilepsy, the doctor’s first duty is to look for the cause and alleviate it if possible. If the patient still has seizures, the epilepsy must be classified so that treatment with appropriate antiepileptic drugs can be started, always at the lowest possible dose. The doctor must always be on the
0.HENRIKSEN
S24
lookout for side effects, especially if high dosages are needed to control the seizures. In such cases, neuropsychological evaluation is indicated. Sometimes the addition of a second or even a third drug may control the seizures, but in these situations it is necessary to insure that side effects do not replace the seizures. Subtle changes in alertness and cognition caused by medication may be difficult to detect, especially if the child originally suffered from frequent brief seizures. These symptoms may be demonstrated by a computerized test battery that can be administered repeatedly and which is therefore a fine tool for optimizing the dose of medication. It may sometimes be preferable for the patient to have some seizures with a moderate dosage of a single drug rather than be seizure free with massive dosages of several drugs. Very few clinicians have access to a computerized test battery and may therefore have to rely on close follow-up and cooperation between the physician, psychologist, teacher, and parents. Such teamwork may disclose adverse drug effects that might otherwise remain undiagnosed and affect the child’s learning abilities for Y.-
Whether or not to treat is a complex question. Before starting treatment, the potential side effects of antiepileptic medication should be considered. On the other hand, the earlier epilepsy is controlled, the better the prognosis. If subclinical epileptic activity shown by the EEG is treated, the treatment must be monitored with a computerized neuropsychological test battery. Treatment should be continued only if benefits of reduced epileptiform activity can be Jemonstrated and if adverse cognitive effects of the drug can be avoided.
INFORMATION TO SCHOOLS AND PARENTS Thorough information is necessary to prevent or alleviate psychosocial problems (Henriksen, 1985). Communication begins with the patients and their parents, but teachers and peers should not be forgotten. Watching a friend in school having a generalized tonic-clonic seizure is a frightening experience that can have an unfavorable effect on the attitude of peers and their parents. This can be prevented if everyone is well informed beforehand. The label “epilepsy” may have a masking effect, causing both the patient and the teacher to blame all of the child’s problems on the epilepsy. Parents, in particular, may have difficulties in realizing and accepting that problems other than epilepsy are present. If a child is mentally retarded and needs special
schooling, prompt and extensive counseling is often required. The teacher and psychologist, in collaboration, are responsible for diagnosing specific learning disabilities as early as possible and organizing special education when needed. A child with epilepsy can have any form of learning disorder, and a variety of remedial measures may need to be considered (Strang, 1987). Neuropsychological evaluation should be comprehensive and interpreted from a broad perspective, taking into account the child’s ability in relation to his/her home, the community, and the school demands. This assessment forms the bask for appropriate remedial education. If these problems are successfully diagnosed and treated, the child can be motivated to learn, self-esteem improves, and the psychosocial problems diminish. Although integration of handicapped children into ordinary schools is preferred, good functioning in a special school may be better than marginal functioning in an ordinary school. Environmental demands need to be balanced with the child‘s ability, in order to optimize self-esteem, learning potential, and psychosocial functioning. A mentally retarded child may thus be happier at a special school where peers have similar problems. Unrealistic aspirations for a child only serve to reduce self-esteem, and may depress learning, increase seizure frequency, and lead to social isolation. Appropriate psychosocial treatment can reverse a vicious cycle of seizures, learning difficulties, and psychosocial problems. An evaluation done in 1986 in the Children’s Department at the Norwegian National Center for Epilepsy showed that eight children became seizure free because of psychological and educational remediation, while medication remained unchanged (Skaare, 1989). Whether a child with epilepsy has a high I.Q. or is mentally retarded, much can be gained through early diagnosis, adequate treatment, and thorough information to all concerned.
REFERENCES Binnie CD. Seizures, EEG discharges and cognition. In: Trimble MR, Reynolds EH, eds. Epilepsy. behaviour and cognitive funcrion. Chichester: John Wiley & Sons 1988:45-9. Blom S, Heijbel J, Bergfors ffi. Incidence of epilepsy in children. Epilepsia I978;19343-50. Cavazutti GB. Epidemiology of different types of epilepsy in xhool-age children of Mcdena, Italy. Epilepsia 19802 157-62. Commission on Classification and Terminology of the Intemational League Against E p i l w . RoDosal for revised classification of epilep&es and epileptic syndromes. Epilrpsro 1989;30:389-99. Fatwell JR, Dodrill CB,Batzel LW. Neumpsychological abilities of children with epilepsy. Epikpsia 1985:26:395-400.
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ASSESSMENT AND REMEDIATION Harrison RM, Taylor DC.Childhood seizures: a 25 year follow up. Social and medical prognosis. Loncet 1976;1:948-5I . Henriksen 0.The importance of information of patients and relatives. In: Dam M, Gram L, Pedersen B, 0 m m H, eds. Modern approach to antiepileptic drug treatment. Copenhagen: The Danish Epilepsy Society, 1985:55-60. Holdsworth L, Whitmore K. A study of children with epilepsy attending ordinary schools. I: Their seizure patterns, progress and behaviour in school. Dev Med Child Neurol 1974;16:74658. Krohn W. A study of epilepsy in Northern Norway; its frequency and character. Acta Psychiatr Neurol Scand I961;36(suppl 150):25-225. Pazzaglia P, Frank-Pazzaglia L. Record in grade school of pupils with epilepsy: an epidemiological study. Epilepsia 1976;
I7:361-6. Ross EM, Peckham CS, West PB, Butler NR. Epilepsy in childhood: findings from the National Child Development Study. Br Med J 1980;1:207-10. Rugland AL, Bjernaes H, Henriksen 0, LByning A. The develop ment of computerized tests as a routine p r d u r e in clinical EEG practice for the evaluation of cognitive changes in patients with epilepsy. 17th Epilepsy International Congress, 1987,A b stract, p. 102.
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Skaare R. Effekt av tverrfaglig behandlingsopplegg sett fix pedagogisk synsvinkel. Abstract from 7. Skandinaviska Epilepsikongressen in T y l h n d , April 20-22. 1989. Stores G. Learning and emotional problems in childm with epilepsy. In: Reynolds EH, Trimble MR, eds. Epilepsy andpsychiatry. London: Churchill Livingstone, 1981:293-3 10. Stores G. Effect on learning of "subclinical" seizure discharge. In: Aldenkamp AP, Alpherts WCJ, Meinardi H, Stores G, eds. Education and epilepsy. Lisse/Berwyn: Swets & Zeitlinger,
1987:14-20. Strang JD. Educational and related treatment considerations concerning the child with epilepsy: a developmental neuropsychological approach. In: Aldenkamp AP, Alpherts WU, Meinardi H, Stores G, eds.Education and epilepsy. Lisse/Berwyn: Swets & Zeitlinger, 1987:118-34. Thompson PJ, Trimble MR. Antimnvulsant drugs and cognitive functions. Epilepsia 1982;23:531-44. Tomlinson LL, Andrewes DG, Memfield E, Reynolds EH. The effects of antiepileptic drugs on cognitive and motor functions. Br J Clin Pracl 1982;18(suppl):177-83. Verity CM, Ross EM. Longitudinal studies of children's epilepsy. In: Ross E, Reynolds E, eds. Paediatricperspectiveson epilepsy. Chichestex John Wiley & Sons Ltd., 1985:133-9.
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