Pediatric epilepsy is a common, chronic, and challenging physical illness for children and their families. This article provides a medical overview and discusses the cognitive functioning and psychosocial adjustment as well as the psychiatric management for children and adolescents with pediatric epilepsy. The management of these children involves establishing a collaborative health care approach, evaluating academic functioning, considering psychotherapy, and managing psychopharmacologic treatment. A thorough understanding of the biopsychosocial concerns in pediatric epilepsy can enable medical providers and mental health clinicians to promote resiliency and adaptation in children and their families facing troubling seizure disorders.
Pediatric epilepsy is a common, chronic, and challenging physical illness for children and their families. Approximately 45,000 children younger than 15 years develop a new seizure each year, with a median age of onset between ages 5 and 6 years. By 20 years of age, 1% of the US population can be expected to have developed epilepsy. In 7 of every 10 cases, there is no known cause, leading to a diagnosis of idiopathic epilepsy. A secondary or symptomatic epilepsy diagnosis is given when there is a known cause for the seizures (eg, anoxic damage, physical trauma, infection, or tumor). Finally, cryptogenic or probably symptomatic epilepsy implies that an underlying cause for epilepsy exists but cannot be identified.
Childhood epilepsy can place substantial cognitive and psychosocial burdens on young patients and their families. Research on the psychiatric concerns of pediatric epilepsy has grown exponentially over the years (eg, PubMed search on 11/14/2009 found 134 articles published by 1980, 1182 articles between 1981–2000, and 2013 articles from 2001–2009). Focusing primarily on literature from the last 2 decades, this article provides a medical overview along with reviews of cognitive functioning, psychosocial adjustment, and psychiatric management of children and adolescents with pediatric epilepsy. A thorough understanding of these interrelated domains can enable providers to promote resiliency and adaptation in the patients and families facing pediatric epilepsy.
Medical overview
Seizures are changes in motor function, sensation, and/or consciousness that are the consequences of abnormal synchronized electrical discharges in the brain. In 2001, the Commission on Epidemiology of the International League Against Epilepsy (ILAE) published a diagnostic scheme based on the following 5 axes: (1) behavior during the seizure, (2) types of seizures, (3) detailing specific syndromes, (4) causes, and (5) resultant impairments. Box 1 provides an overview of the ILAE diagnostic schema; for a detailed review of seizure types the reader is referred to Friedman and Sharieff.
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Axis I: ictal phenomenology
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Description of the seizure event
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Axis II: seizure type
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Self-limited
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Generalized: begins deep in the brain and reaches all parts at the same time (eg, tonic-clonic, clonic, absence, spasms, neocortical temporal, myoclonic seizures)
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Focal: begins in one region of the cortex (eg, focal sensory or motor seizures)
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Simple: consciousness is preserved
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Complex: consciousness is impaired
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Continuous
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Generalized status epilepticus
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Focal status epilepticus
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Reflect: ictal events participated by sensory stimuli (visual, thinking, music, eating, reading, startle, hot water)
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Axis III: syndrome
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Complex of signs and symptoms that define a unique epilepsy condition (eg, febrile seizures, alcohol withdrawal, progressive myoclonic epilepsy, childhood absence, Lennox-Gastaut syndrome, familial temporal lobe, limbic or neocortical epilepsies, or reflex epilepsies)
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Axis IV: cause
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Symptomatic: resulting from structural brain lesions (secondary epilepsy)
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Probably symptomatic: believed to be symptomatic but no cause identified (cryptogenic)
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Idiopathic: primary epilepsy with no identifiable structural brain lesion, presumed to be genetic
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Axis V: impairment
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Description of disability caused by the epileptic condition
Approximately 70% of children with epilepsy become seizure free on antiepileptic drugs (AEDs) within a few years of diagnosis and subsequently can safely discontinue their medications without recurrence of seizures. Seizure remission is associated with a defined cause of seizure, absence of an epilepsy syndrome, onset before 10 years of age, a low seizure frequency, and early treatment response. In a prospective study of childhood-onset epilepsy, only about 10% of the sample met the criteria for intractable epilepsy (defined as having failed 2 or more AEDs and having had 1 or more seizures per month during a period of 18 months).
There is a multifactorial approach to treatment, which is based on patient’s age; type of seizure; risks of recurrence and potential for injury; psychosocial implications; other predisposing medical issues; or effects of treatment, such as toxicity, expense, and cognitive and emotional well-being correlates. Generally, AED monotherapy is more efficacious and results in fewer and less-significant side effects. Polytherapy generally occurs after the failure of more than 1 individual AED trial. For those children with disabling uncontrolled epilepsy and localized regions of seizure activity, surgery has become a treatment option. Temporal or extratemporal resections or corpus callosotomies have been found to significantly decrease seizures in 50% to 90% of such children.
Additional treatment options have been considered for the 20% to 30% of those who do not respond to AEDs, who are not surgical candidates, and/or who have failed surgery. Vagal nerve stimulation, an approved therapy down to age 12 years, has been found to lead to improvement in patients’ health-related quality of life. Similar positive effects have also been found with a ketogenic diet in which 80% of the total daily caloric intake is fat.
Cognitive functioning
Pediatric epilepsy is often associated with significant cognitive comorbidity. Cognitive functioning varies in youngsters with epilepsy because of varying interactions between the underlying cause of epilepsy (eg, physiologic disturbance, genetic defect), direct effects of seizure activity on brain structure and function, and/or the medications used to treat seizures. The interactions between these factors help explain the difficulty in obtaining a single unified picture of the neuropsychological strengths and vulnerabilities for the pediatric epilepsy population as a whole.
Similar to previous pediatric population studies, Berg and colleagues found that 73.6% of children had normal intellectual functioning, whereas 26.4% fell in a range of cognitive functioning suggestive of subnormal abilities. They found that the level of cognitive functioning was strongly associated with the underlying cause and type of epilepsy (ie, remote symptomatic cause), age at onset (worse outcome if child was younger than 5 years at the time of diagnosis), and medication status (worse outcome if child was still taking medication 5 years postdiagnosis). Furthermore, each of these factors was independently associated with a moderate increase in the risk of falling in the below-normal cognitive range, with a higher degree of impairment for children who experienced more than 1 factor.
Different neuropsychological profiles have emerged depending on the type of epilepsy, the specific focus of the seizures, or the type of treatment. The rate of co-occurring attention-deficit/hyperactivity disorder (ADHD) is increased more than that expected by chance, likely as a result of the bidirectional relationship between those diagnoses. The rate of executive functioning struggles seems increased as evidenced by deficits in processing speed, working memory, planning, organization, and/or mental flexibility. Visual spatial functions and concept formation/abstract reasoning are also affected, as are language abilities and motor skills.
Efforts to define cognitive functioning in children with idiopathic absence seizures, complex partial seizures, and generalized tonic-clonic seizures found an overall low average IQ as well as lower verbal skills, visual memory, and visual attention skills in these patients. Hemisphere-specific disruptions of cognitive skills have been found in temporal lobe epilepsy, with left-sided lesions showing lower performance on verbal memory tasks and right-sided lesions having decreased visual memory functioning. Children with cryptogenic localized epilepsy were found to display reduced psychomotor speed, poor alertness, slower central information-processing speed, and poorer visual long-term memory. Additional details on the neuropsychological profiles of children with epileptic syndromes may be obtained from the works of MacAllister and Schaffer and Hamiwka and Wirrell.
Effect of AEDs
Worsening scores in arithmetic abilities for children on topiramate and greater improvement for the maze task for children treated with carbamazepine have been found. In another study, no differences were found in the cognitive scores for children who were newly diagnosed with partial seizures and treated with either oxcarbazepine or carbamazepine/valproate during a 6-month treatment period. In a comparison study, the discontinuation of phenobarbital improved the total IQ score in the case group compared with the control group, mostly in nonverbal performance skills, with verbal skills remaining almost unchanged. More information on studies that are focused on the cognitive side effects of AEDs in children can be obtained from Loring and Meador and Aldenkamp and colleagues.
Academic achievement
Children with epilepsy are more likely to experience school problems, with lower academic achievement. The type of epilepsy (eg, localized and symptomatic generalized epilepsies) has been found to be the dominant factor that explains educational underachievement. Specific neuropsychological vulnerabilities in verbal skills, memory, and executive functioning are strongly related to reading, math, and writing problems. However, it is worth noting that studies show that, in addition to specific cognitive deficits, academic struggles are also moderated by demographic and psychosocial variables. For instance, family environment characterized by support and organization significantly moderated the effect of cognitive deficits on writing and reading in children with epilepsy.
Cognitive functioning
Pediatric epilepsy is often associated with significant cognitive comorbidity. Cognitive functioning varies in youngsters with epilepsy because of varying interactions between the underlying cause of epilepsy (eg, physiologic disturbance, genetic defect), direct effects of seizure activity on brain structure and function, and/or the medications used to treat seizures. The interactions between these factors help explain the difficulty in obtaining a single unified picture of the neuropsychological strengths and vulnerabilities for the pediatric epilepsy population as a whole.
Similar to previous pediatric population studies, Berg and colleagues found that 73.6% of children had normal intellectual functioning, whereas 26.4% fell in a range of cognitive functioning suggestive of subnormal abilities. They found that the level of cognitive functioning was strongly associated with the underlying cause and type of epilepsy (ie, remote symptomatic cause), age at onset (worse outcome if child was younger than 5 years at the time of diagnosis), and medication status (worse outcome if child was still taking medication 5 years postdiagnosis). Furthermore, each of these factors was independently associated with a moderate increase in the risk of falling in the below-normal cognitive range, with a higher degree of impairment for children who experienced more than 1 factor.
Different neuropsychological profiles have emerged depending on the type of epilepsy, the specific focus of the seizures, or the type of treatment. The rate of co-occurring attention-deficit/hyperactivity disorder (ADHD) is increased more than that expected by chance, likely as a result of the bidirectional relationship between those diagnoses. The rate of executive functioning struggles seems increased as evidenced by deficits in processing speed, working memory, planning, organization, and/or mental flexibility. Visual spatial functions and concept formation/abstract reasoning are also affected, as are language abilities and motor skills.
Efforts to define cognitive functioning in children with idiopathic absence seizures, complex partial seizures, and generalized tonic-clonic seizures found an overall low average IQ as well as lower verbal skills, visual memory, and visual attention skills in these patients. Hemisphere-specific disruptions of cognitive skills have been found in temporal lobe epilepsy, with left-sided lesions showing lower performance on verbal memory tasks and right-sided lesions having decreased visual memory functioning. Children with cryptogenic localized epilepsy were found to display reduced psychomotor speed, poor alertness, slower central information-processing speed, and poorer visual long-term memory. Additional details on the neuropsychological profiles of children with epileptic syndromes may be obtained from the works of MacAllister and Schaffer and Hamiwka and Wirrell.
Effect of AEDs
Worsening scores in arithmetic abilities for children on topiramate and greater improvement for the maze task for children treated with carbamazepine have been found. In another study, no differences were found in the cognitive scores for children who were newly diagnosed with partial seizures and treated with either oxcarbazepine or carbamazepine/valproate during a 6-month treatment period. In a comparison study, the discontinuation of phenobarbital improved the total IQ score in the case group compared with the control group, mostly in nonverbal performance skills, with verbal skills remaining almost unchanged. More information on studies that are focused on the cognitive side effects of AEDs in children can be obtained from Loring and Meador and Aldenkamp and colleagues.
Academic achievement
Children with epilepsy are more likely to experience school problems, with lower academic achievement. The type of epilepsy (eg, localized and symptomatic generalized epilepsies) has been found to be the dominant factor that explains educational underachievement. Specific neuropsychological vulnerabilities in verbal skills, memory, and executive functioning are strongly related to reading, math, and writing problems. However, it is worth noting that studies show that, in addition to specific cognitive deficits, academic struggles are also moderated by demographic and psychosocial variables. For instance, family environment characterized by support and organization significantly moderated the effect of cognitive deficits on writing and reading in children with epilepsy.
Psychosocial adjustment
Emotional Functioning
Children with epilepsy seem to be at risk for emotional and behavioral problems. However, there has been significant variation across studies exploring the rate of psychopathology in children. Similar to the factors affecting epilepsy prognosis and cognitive functioning, seizure frequency/control, type of epilepsy, age of onset, illness duration, and AED medications also likely contribute to the differences found between studies. In addition, differences in the methodology used across studies to assess emotional functioning may also contribute to the varying prevalence rates of emotional and behavioral difficulties in the pediatric epilepsy population.
The Isle of Wight study found that 28% of children with epilepsy had psychiatric disorders. Ott and colleagues found that children with complex partial seizures or partial generalized with absence seizures had comparable rates of anxiety and affective struggles (16%), disruptive behaviors (23%), and psychiatric comorbidity (20%). The investigators concluded that, whether by parent report or by diagnostic interview, children with epilepsy are 3 to 6 times more likely to develop psychopathology than the general population.
Nonepileptic (or nonelectrical) seizures (NESs) are estimated to occur in 17% to 30% of patients referred to comprehensive epilepsy centers (a slightly lower frequency is reported in children) and are paroxysmal events with clinical similarity to epileptic seizures, in the absence of concurrent electrographic ictal pattern. Because a high proportion of patients with NESs have concurrent epilepsy that can be difficult to distinguish from epileptic events, NESs should be suspected when events (1) are refractory to medication changes, (2) have atypical clinical features, (3) are exacerbated by stress, and (4) are not associated with incontinence or a postictal period. Psychosocial stressors are associated with NESs, and common underlying psychiatric diagnoses include mood and/or anxiety disorders (especially separation anxiety, social anxiety, and school phobia). NESs are common in children, and the gold standard of diagnosis is ictal video electroencephalogram (EEG) recording. Early diagnosis is essential for the institution of appropriate psychotherapeutic interventions, and it allows a reduction in the morbidity of unnecessary AED therapy. Video-EEG evidence also helps parents comprehend the emotional, nonepileptic nature of the events.
In a comparison with healthy children, youngsters with epilepsy had significantly more anxiety/affective disorders (63%) and disruptive disorders (26.1%) as well as suicidal ideation (20%). In the same study, only a few patients (5.2%) had depression as a single-standing diagnosis because most cases of depression were comorbid with anxiety disorders (3.5%) or disruptive disorders (28%). When using categorical and dimensional measures of psychopathology in children diagnosed with epilepsy in the preceding 6 months, Dunn and colleagues found that the risk for a psychiatric diagnosis was 3 to 4 times higher for attention-deficit hyperactivity, oppositional defiant, conduct, and dysthymic disorders than that for healthy comparison children.
Multiple studies show that suicide is more common in people with epilepsy. A history of psychiatric disease increases the risk of suicide in a person with epilepsy, but the risk of suicide in people with epilepsy is increased also in the absence of a psychiatric history. The relationship between seizures and depression or suicidal behavior may be bidirectional, and major depression and attempted suicide increase the risk of developing seizures.
In children with epilepsy with comorbid nonverbal cognitive problems, psychosocial problems (including internalizing and externalizing problems) were much more frequent than among controls. Although far less frequent, thought disorders in pediatric epilepsy seem to occur at a higher rate when compared with community-based population estimates.
The question of cause-effect relationship between epilepsy and psychiatric difficulty remains an area of inquiry. Behavioral problems have been identified as early as 6 months before the child’s first recognized seizure, with nearly 32% of the newly diagnosed children having a preexisting baseline of psychiatric symptomatology within the clinical or the at-risk ranges. A developmental perspective in explaining the psychological and behavioral difficulties of children with epilepsy suggests a model whereby the cognitive and language difficulties found in pediatric epilepsy adversely affect a child’s ability to follow normal emotional and social skill developmental tracks.
In a meta-analysis examining the types and severity of psychopathology in children with epilepsy, the magnitude of differences revealed small to medium effect sizes when compared with children with other chronic physical illnesses (eg, asthma, diabetes, cardiac disease, and migraine), suggesting that the psychopathology in pediatric epilepsy may be attributed partly to the chronicity of the physical disease as opposed to an illness-specific effect of epilepsy. Furthermore, within the larger population of children with other physical illnesses affecting the central nervous system, the rates for anxiety and depression were similar to those in the pediatric epilepsy population.
Epilepsy during adolescence poses a period of vulnerability that can hinder the development of psychosocial independence, and treatment relies on AEDs, which must be taken daily. Nonadherence is common and problematic because of lack of education about epilepsy (ie, why take medications in the absence of seizures), desire to avoid adverse medication side effects, difficulties in controlling seizures, forgetfulness, disorganization, and/or oppositionality. Negative outcomes of nonadherence include seizure recurrence, missing school, absence from social events, emergency department visits, and/or hospitalization. Studies have shown that treatment nonadherence is as high as 50% in chronic pediatric illnesses. Caregiver involvement in their children’s epilepsy management, which is greater than what has been found in other chronic physical illnesses, has implications with regard to monitoring treatment adherence. A meta-analysis of psychological interventions developed to increase adherence has revealed that applied behavioral interventions (eg, problem solving, parent training) and multicomponent interventions incorporating educational treatment models, social support, social skills training, or family therapy seemed to be relatively potent in promoting adherence among chronically physically ill youth.
Despite the common desire of adolescents with epilepsy to drive, seizures pose the risk of a crash, which may result in property damage, injuries, and even deaths. Today, every state in the United States permits people with controlled seizures to drive with required seizure-free periods ranging from about 3 to 12 months. Many patients with epilepsy frequently do not inform their physicians about seizure occurrence, fearing loss of driving privileges and other social consequences ; therefore, it is recommended that physicians engage adolescents directly in discussions about their health condition, including the safety issues related to driving.
Family Functioning
Physical illness in a child is a significant source of stress for families. Given its chronic nature, childhood epilepsy presents ongoing demands on the family’s ability to adapt and function. Parents of children with epilepsy have reported feeling more emotionally overwhelmed and fearful than parents of children with special health care needs. Parents have been found to experience posttraumatic stress symptoms and major depressive disorder, to express anxiety and decreased quality of life, or to express increased stress levels, especially if comorbid depression and learning difficulties were present in the child.
The increased parenting stress caused by having a child with epilepsy has been found to adversely affect parent-child interaction, leading to behavioral and emotional problems. A meta-analysis of the research on the psychosocial adjustment of children with epilepsy reveals that behavioral and emotional disturbances are related to parental criticism and parental psychological control, whereas parental acceptance was associated with lower levels of externalizing behavior problems. These findings of parental negative perceptions and stigma in relationship with adjustment to childhood epilepsy have offered mixed results; overall, having epilepsy and mental health problems may possibly lead to an increase in the stigma load for these children.
Long-term consequences of epilepsy may be a result of any or all of the childhood psychological struggles, effects on cognitive functioning, and/or family adjustment and stressors. Considering the elevated prevalence of psychopathology in adults with epilepsy (about 65%), the less-than-desirable outcome of children with generalized epilepsy 20 years postdiagnosis, and the poor quality of life for seizure-free or off-medication adults 30 years from the diagnosis, successfully identifying and treating emergent psychopathology in the pediatric population warrants early identification and early preventive interventions.
Psychiatric Management
Establish a collaborative health care approach
A pattern of delayed recognition and diagnosis of epilepsy has been observed with a variety of interrelated reasons including parental delay in seeking treatment, health care providers not recognizing a presentation as epilepsy, and/or health care providers initiating inappropriate treatment. Unrecognized seizures can have adverse physical and psychosocial consequences. According to a survey of pediatricians and child neurologists, most of them were unaware of the greater need for mental health referrals in children with epilepsy, identified limited resources in their own expertise about the mental health system, and recognized their lack of collaboration with mental health providers. This becomes evident because more than 60% of children with epilepsy do not receive needed mental health services despite diagnosable psychiatric disorders. In this context, it is critical for the child mental health clinicians to establish an integrated health care approach characterized by communication and collaboration with a child’s family, primary care clinician, and specialty care clinician (eg, child neurologist). This will allow the mental health clinician not only to convey important psychiatric considerations to the physical health clinicians but also to be alert to important changes in the course and physical treatment of the child’s epilepsy.
The care of patients with pediatric epilepsy does involve workforce shortages, which create significant challenges for the patient, family, and primary care clinicians in obtaining specialty care. There are approximately 8000 child psychiatrists, 1200 child neurologists, and 500 developmental behavioral pediatricians in the United States. This means that primary care clinicians often need to undertake the tasks of both medical and psychiatric care of childhood epilepsy without the ability to collaborate with child-trained psychiatrists or neurologists who specialize in the developmental, psychosocial, or biologic aspects of these patients.
Because not every youngster with epilepsy needs mental health and educational assessment or intervention, primary care clinicians are increasingly adopting standardized screening tools and/or queries to identify only the youngsters who are most in need of mental health intervention. In this context, child mental health clinicians with strong interests in chronic pediatric physical illnesses have tremendous opportunities to collaboratively work with primary care clinicians to develop innovative and integrative health care approaches for children and their families.
Evaluate academic functioning
The use of neuropsychological evaluations for the pediatric epilepsy population has been a topic of debate. Arguments for and against this intervention have been discussed: the need for early identification of learning or mental health problems, ability to learn in school, or successful negotiation of social peer interaction versus an indiscriminate use of time-consuming and expensive evaluations, the potential inappropriate labeling of children as “learning disabled,” or the risk of attached stigma. Overall, the conclusion is that the option of a neuropsychological evaluation should be at least considered by mental health clinicians in the course of their evaluation and management of children with epilepsy.
Children identified as “students in need of services” can benefit from services ranging from simple classroom accommodations to specialized instruction. Communication among home, school, and medical providers is an essential step in ensuring that an integrated health care approach that increases a child’s chance for success is implemented. The mental health clinician needs to be alert to parental stress levels when negotiating with the educational system because research has noted increased parent struggles when their children present with learning disabilities co-occurring with epilepsy. To communicate effectively, school personnel and parents must be educated about neurocognitive effects that a particular child may experience. In addition, knowledge of state and federal laws regarding the education of children with special needs will aid in the parents’ ability to navigate through the education system and to best advocate for their child.
As part of this process, the child mental health clinician must provide psychoeducation and guidance to parents regarding the neuropsychological correlates of their child’s epilepsy, from a thorough evaluation of potential cognitive weaknesses to explaining the role of neuropsychologists and educational advocates in the community or the school.
Consider psychotherapy
Psychotherapy is an important treatment that should be considered for emotional and behavioral problems in children with epilepsy. The treatment, which should include the child and family, targets the enhancement of coping mechanisms that promote continued psychological development and adaptation to illness. Effective psychotherapy can help patients understand the meaning of and responses to their illness, improve treatment adherence, and enhance psychosocial functioning. As with other physical illnesses, there continues to be a paucity of empirical evidence that supports the efficacy of any psychotherapeutic modality in children with epilepsy.
In a review of the existing successful intervention programs designed to provide support to children and families facing epilepsy, identified treatment goals were delineated, such as: reducing children’s concerns and fears; problem behaviors; minimizing associated stress; increasing epilepsy knowledge and decision-making skills; improving attitudes toward epilepsy; and improving epilepsy management, independence, coping skills, and efficient communication. These interventions were delivered in through a variety of modalities including individual meetings, parent groups, phone interviews/conferences, video conferencing, and summer camps. Both individual and group cognitive behavioral intervention strategies, including relaxation and biofeedback, predominated as the treatment approach.
Overall, these evidence-based psychological interventions have shown improved psychological adjustment and some evidence that seizure severity and frequency may be reduced. Although these therapeutic efforts provide support for the use of psychological interventions in childhood epilepsy, methodologically sound randomized clinical trials are still needed before taking for granted the implications of their effectiveness. Nonetheless, despite numerous methodological weaknesses in existing studies, there is a sufficiently strong evidence base to support the integration of psychotherapy treatment as part of comprehensive pediatric care in physically ill children and adolescents.
Manage psychopharmacologic treatment
It is beyond the scope of this article to review the pharmacologic treatment of the different types of epilepsy in childhood. The prescribing mental health clinician should be familiar with the medications being used in the treatment of seizures in each individual child and their common associated neuropsychiatric effects ( Table 1 ).
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