1Running head: EPILEPSY AND COGNITION

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2EPILEPSY AND COGNITION

Effects of Epilepsy on the Cognition of Young ChildrenJulia HartThe George Washington University

AbstractEpilepsy is a neurological condition that affects many children. Epilepsy, seizures, and anti-epilepsy medications can all affect a childs learning and development. Specifically, the cognition of children with epilepsy is frequently different from that of children without seizure disorders. Children with epilepsy often have deficits in attention, memory, and executive functioning. These children can also have social and emotional struggles such as anxiety, depression, and altered behavior. These specific cognitive implications and school-based interventions to address the deficits are discussed.

Effects of Epilepsy on the Cognition of Young ChildrenEpilepsy is a common medical condition in young children. The impact of epilepsy can be seen across multiple developmental domains and is thus a relevant topic of study for educators and interventionists. This paper will explore the medical nature of the condition, its affects on learning and school performance in children, as well as provide specific models of interventions for young children with epilepsy. Definitions, Symptomatology, and Medical InterventionEpilepsy is a disorder characterized by recurrent seizures (Centers for Disease Control and Prevention [CDC], 2013). More than one unexplained seizure leads to a diagnosis of epilepsy (Titus & Thio, 2009). According to the CDC (2013), A seizure is a temporary disturbance in brain function in which groups of nerve cells in the brain signal abnormally and excessively. Seizures can last from just seconds to a few minutes. Depending on which part of the brain is affected, seizure symptoms and appearance will vary. A person may experience involuntary movements, behavior changes, or changes in awareness during a seizure (CDC, 2013).DiagnosisThe diagnosis of epilepsy comes from a medical doctor. According to the National Institute of Neurological Disorders and Stoke (NINDS; 2013), the diagnosis of epilepsy comes after two or more unexplained seizures (i.e., seizures not resulting from fever, drug abuse, or head injury). Electroencephalography (EEG), magnetic resonance imaging (MRI), and computerized tomography (CT) can also be used in the diagnostic process (NINDS, 2013). Further, genetic testing may be utilized to identify a potential cause for the epilepsy, such as a genetic mutation (Iliescu & Craiu, 2013). Iliescu and Craiu (2013) also wrote about the use of metabolic testing to determine the etiology of an epilepsy diagnosis. Types of SeizuresSeizures can be categorized as either primary generalized seizures or partial seizures (Schachter, 2013). According to Solomon and McHale (2012), in generalized seizures the whole brain is affected by changes in electrical activity and the child becomes unconscious of their surroundings (p. 29). Generalized seizures affect both sides of the brain at once (Schachter, 2013). Generalized seizures include tonic-clonic seizures, tonic seizures, atonic seizures, myoclonic seizures, and absence seizures (Solomon & McHale, 2012). Tonic-clonic seizures, once called grand mal, involve the entire brain (e.g. McCandless, 2012). Tonic-clonic seizures involve full-body convulsions, muscle rigidity, and strong muscle contractions. Following a tonic-clonic seizure comes a long period of unconsciousness, amnesia, and drowsiness (McCandless, 2012). Tonic seizures are generally short in duration (under one minute), come on quickly, and involve stiffened muscles (McCandless, 2012). If the child is not seated at the onset of the tonic seizure, a fall is likely, as is possible head-injury (Solomon & McHale, 2012). There is no jerking or convulsing during a tonic seizure (Solomon & McHale, 2012). Atonic seizures typically involve a sudden and complete loss of muscle tone, causing the body to fall to the ground. For some, atonic seizures may only involve a dropped head or slumped body, rather than complete loss of muscle control (McCandless, 2012). Myoclonic seizures involve muscle jerks and brief loss of consciousness. Myoclonic seizures typically happen immediately after waking or shortly before going to sleep at night (Solomon & McHale, 2012). Absence seizures typically present as staring spells or a general unresponsiveness and last for a maximum of 30-s (McCandless, 2012). In some cases, an absence seizure may include lip smacking or minor tongue movements (McCandless, 2012). According to McCandless (2012), absence seizures account for 5% of all epilepsy and affect girls more than boys. Because children recover quickly from absence seizures, and there is not a prolonged loss of consciousness, many absence seizures go unnoticed or are treated as inattention (Solomon & McHale, 2012). Partial seizures are caused by an electrical discharge in one specific part of the brain (Schachter, 2013). Forms of partial seizures include simple partial seizures and complex partial seizures (Schachter, 2013). McCandless (2012) wrote, A complex partial seizure involves impaired consciousness, whereas patients exhibiting simple partial seizures do not have impaired consciousness. They are able to answer questions, respond, etc. during the seizure (p. 143). Simple partial seizures often occur with an aura, a special sensory sign and may evolve into a complex partial seizure (McCandless, 2012). These sensory signals generally relate to the affected area of the brain; if the auditory cortex is the seizures location, one might hear strange sounds during the seizure. McCandless also wrote that the etiology of simple partial seizures can usually be identified. Causes may include hypoxia, infections, syphilis, and viral infections (McCandless, 2012). Complex partial seizures occur in approximately one-third of epilepsy patients (McCandless, 2012). Complex partial seizures generally are preceded by an aura and involuntary tics and involve a loss of consciousness (McCandless, 2012). Auras may include sensory illusions, auditory or visual hallucinations, a sense of fear, or a particularly strong emotion (happiness, anger) and older patients learn to recognize these auras as a sign of impending seizure (McCandless, 2012). Automatisms, unconscious behaviors or movements, may occur during a complex partial seizure. According to McCandless (2012), typical automatisms include chewing movements, lip smacking, fumbling/grasping movements, blinking, leg movements resembling running, etc. These automatisms are inappropriate for the situation and tend to be similar in succeeding seizures (p. 168). Complex partial seizures can last from just seconds to 2-min, with shorter seizures appearing similar to absence seizures (McCandless, 2012). Complex partial seizures typically originate in the frontal lobe or the temporal lobe and are often caused by birth defect, birth trauma, head injury, or brain infection (McCandless, 2012). Partial seizures with secondary generalization begin in one area of the brain then spread to involve the whole brain (Massachusetts General Hospital [MGH], 2006). These seizures typically end in a tonic-clonic seizure (MGH, 2006). Partial seizures with secondary generalization often begin with an aura. Because these are partial seizures, treatment with surgery is often an option (MGH, 2006). Status epilepticus is a seizure, or series of seizures, that continues for 15-min or more (MGH, 2006). Status epilepticus can occur in patients with any type of seizure and is considered a medical emergency. Of children experiencing their first seizure before age one, 70% will experience status epilepticus in their life (McCandless, 2012). Because status epilepticus requires swift medical treatment, there is a worldwide mortality rate of 10-30% in cases of status (McCandless, 2012). Generally rectal diazepam is the first treatment for status, with intravenous medication a possible next step (McCandless, 2012).

Treatment OptionsConsultation with a neurologist or epileptologist (a neurologist specializing in epilepsy) is necessary for the management of epilepsy. Most practitioners will not begin treatment for epilepsy until a patient has experienced two unprovoked seizures (Titus & Thio, 2009). In some cases, epilepsy may have minimal effect on the childs quality of life, and a doctor may decide not to utilize drug therapy (Titus & Thio, 2009). According to McCandless (2012), approximately 70% of people with epilepsy have achieved treatment success (p. 3). For many patients with epilepsy, antiepileptic drugs (AEDs) are utilized to treat seizures. Approximately 90% of epilepsy patients in the United States take AEDs to manage their condition (McCandless, 2012). In many cases, two or more AEDs are required to obtain seizure control; this approach is known as polytherapy (Titus &Thio, 2009). McCandless (2012) noted that 56% of US epilepsy patients receive monotherapy, 26% take two AEDs, 6% take three AEDs, and 2% take four AEDs. Approximately two-thirds of people with epilepsy achieve full seizure control with medication (CDC, 2013). While the remaining one-third of people may experience a reduction in seizures through medication, some will require further intervention (CDC, 2013). Surgical intervention is another treatment option for patients with seizures not controlled by medications. For some patients with partial epilepsy the focal point of seizures can be identified in the brain, making surgical removal of the area a possible treatment (CDC, 2013). A ketogenic diet, high in fat and low in carbohydrates, is sometimes used as treatment for epilepsy. MGH (2006) explained the process of ketosis:As the body metabolizes high-fat foods, it produces chemical compounds calledketone bodies, resulting in a physiologic state known asketosis. It also increases the acidity of the blood. Experts hypothesize that the presence of ketone bodies may be involved in the reduction of the abnormal brain activity responsible for seizures, but still very little is known as to the actual mechanisms of this process. (para. 21) Vagus nerve stimulation (VNS) also is used to successfully treat some cases of epilepsy. VNS involves a small pacemaker-like device is implanted under a persons skin and wired to direct short electrical pulses to the vagus nerve (MGH, 2006)Developmental and Educational OutcomesThere are approximately two million people in the United States with epilepsy (McCandless, 2012). Seventy percent of epilepsy cases develop during childhood, the majority during the first year of life (McCandless, 2012). Researchers have determined that approximately 50% of children with epilepsy have school-related difficulties (e.g. Beghi, Cornaggia, Frigeni, & Beghi, 2006). Berg, Caplan, Baca, and Vikery (2013), in their longitudinal study of young children diagnosed with epilepsy, found that over half of the children in their study group experienced difficulty at school, which they classified as referral for special education services, grade retention, or both. In addition to seizure-related difficulties, anti-epileptic drugs may also affect childrens learning abilities.Selassie, Viggedal, Olsson, and Jennische (2008) conducted a study of children with epilepsy in Sweden. The authors focused on preschool children who had not been identified as having a neuroimpairment other than epilepsy (e.g., cerebral palsy, autism, or intellectual disability). The authors also identified children for their reference group from the same preschools attended by the children with epilepsy. Study participants were evaluated by a speech and language pathologist in multiple language domains, as well as evaluated by a neuropsychologist with multiple intelligence tests. Individual results of these tests will be addressed in the coming sections; however, Selassie et al. (2008) summarized their results:Preschool children with epilepsy and without [intellectual disability], CP, and/or autism may have specific speech-language, auditory attentional, and visuoperceptual difficulties. However, the usual measures of verbal intelligence and comprehension revealed results within normal range. (p. 437)ComorbidityEpilepsy occurs at a significant rate in a number of disability and disease populations. According to McCandless (2012), 30% of children with cerebral palsy and 10% of children with autism also have epilepsy. Oguni (2013) reported that between 7.1% and 32% of children with epilepsy have autism spectrum disorders (ASD). Intellectual DisabilityThe relationship between intellectual disability (ID) and epilepsy has not been fully explored. Oguni (2013) reported that 5-10% of individuals with epilepsy also have ID and developmental disabilities (DD). Vasconcellos et al. (2001) conducted a study of children with intractable epilepsy (seizures which cannot be controlled by medication) who were diagnosed before the age of two. The authors found that children in this study group were more likely to have an IQ less than 70 than were children diagnosed after age two. This link between uncontrolled seizures and intellectual disability underscores the need to stop the seizures through medical intervention as quickly as possible.Learning DisordersBeghi, et al. (2006) found that 25% of children with epilepsy have a learning disorder, while 50% of children with learning disorders have epilepsy. The authors also noted that earlier onset of seizures correlates with a higher likelihood of learning disorder. Additionally, they noted that children with temporal lobe epilepsy (complex partial seizures) may face specific learning disabilities based on the location of their seizures. For example, Beghi, et al. (2006) wrote that children with right-hemisphere involvement may be impaired in visual tests, while those with left-hemisphere involvement may struggle with verbal tests. Attention and MemoryOguni (2013) wrote that 30-40% of children with epilepsy have co-occurring attention deficit hyperactivity disorder (ADHD). Selassie et al. (2008) reported similar data. The authors wrote, attention problems have been found in 42% of children with epilepsy (p. 432). In the researchers study they found that children with epilepsy have a poorer performance on attention measures of the Dichotic Listening Test than their non-epileptic peers. Timmel (2006) wrote of children with epilepsy, disturbances in their brain that result from seizures make it difficult for students to focus their attention and thus acquire new information (p. 1). Timmel further explained, Seizures can also compromise their working memory, making it difficult for them to store and retrieve any information that they were able to learn (p. 1). Titus and Thio (2009) found in their review of literature that older AEDs (e.g., Dilantin, Tegretol, and Depakote) are associated with memory problems in epilepsy patients. The authors also found that some newer AEDs may cause executive function-related memory problems. Svoboda (as cited in Selassie et al., 2008, p. 437) found that 61% of children with epilepsy at the Kansas City Epilepsy Center have difficulties with short-term memory. Executive DysfunctionExecutive function can be described as metacognitive capacities that allow an individual to perceive stimuli from his or her environment, respond adaptively, flexibly change direction, anticipate future goals, consider consequences, and respond in an integrated manner (Rzezak, Guimaraes, Fuentes, Gerreiro, & Valente, 2012, p. 577). Rzezak et al. (2012) found that children with temporal lobe epilepsy, complex partial seizures localized in the temporal lobe, performed poorly on multiple measures of executive function. The authors also found that executive dysfunction was highly correlated with impaired memory. Titus and Thio (2009), in their review of AEDs and children, found that executive function can be impacted by epilepsy medications. They wrote, The effects of AEDs on executive functioning have been a more consistently reported finding in the literature and have the potential to directly affect school performance in the classroom in more subtle ways (p. 889). As with memory functioning, executive functioning is more strongly impacted in children undergoing polytherapy (Titus & Thio, 2009). Anxiety and DepressionEmotional issues can be a major factor in the quality of life of children with epilepsy. Multiple hospitalizations and medical procedures may trigger anxiety in young children with epilepsy. Solomon and McHale (2012) wrote that children with epilepsy might develop a fear of their seizures and the sense of being out of control. Seyfhashemi and Bahadoran (2013), in their review of literature, found that 23-33% of children with epilepsy were depressed, compared to 1-5% of the general population. Titus and Thio (2009) wrote that anxiety and emotional problems can affect a childs performance in school. The authors noted that issues with school performance are particularly common in newly diagnosed children or those who have recently experienced their first seizure. There is conflicting information about the relation between multiple AEDs and depression in children, but there may be a correlation between polytherapy and increased depressive symptoms (Seyfhashemi & Bahadoran, 2013). Titus and Thio (2009) found the antiepileptic drug phenobarbital was strongly tied to depression in children, with 40% of patients on the drug showing depressive symptoms. Another drug, Levetiracetam (Keppra) has been linked to a greater occurrence of behavior problems such as irritability and personality change in children (Titus & Thio, 2009). Educational InterventionsWhile there is ample research regarding medical interventions for childhood epilepsy, intervention studies on best educational practices for this specific population could not be found. Children with epilepsy are a significant population, yet their struggles in school tend to follow the patterns of other childhood disabilities, which may account for the lack of disease-specific research. Intervention studies on childhood executive function, attention and attention deficit disorder were located in lieu of a variety of epilepsy-specific interventions.Best PracticesAs stated above, children with epilepsy who have been identified as having average or above average intelligence may still struggle with the tasks required of them in school (Selassie et al., 2008). Timmel (2006) provided many strategies for teachers of children with epilepsy, including adapting curriculum, thematic teaching, and using tutorials. Particularly relevant for young children is the use of thematic teaching. Thematic teaching helps children with epilepsy by providing repeated exposure to concepts and information throughout the school day (Timmel, 2006). Timmel (2006) suggested collaboration between teachers, interventionists, and families, so that all the adults in a childs life work to incorporate the thematic concepts into their routines. This practice aligns with Division for Early Childhood (DEC) of the Council for Exceptional Childrens Recommended Practice C.18, Practices are used systematically, frequently, and consistently within and across environments (e.g. home, center, community) and across people (i.e. those who care for and interact regularly with the child) (Sandall, Hemmeter, Smith, & McLean, 2005, p. 87). Titus and Thio (2009) wrote that children with epilepsy may struggle with verbal tasks in school; some of these problems may be linked to anti-epilepsy medications. The authors recommended giving these children extended time to respond verbally, limiting performance expectations, and rewording verbal instructions when needed. This practice aligns with DEC Best Practice C.22, Systematic naturalistic teaching practices such as models, expansions, incidental teaching, mand-model procedure, and naturalistic time delay are used to promote acquisition and use of communication and social skills (Sandall et al., 2005, p. 89). Titus and Thio also wrote that children with epilepsy may exhibit symptoms typical of children with ADHD. The authors explained that classroom interventions for children with ADHD are relevant for children with epilepsy-related attention issues. Specific strategies mentioned included frequent repetition, redirection, cueing, one-on-one instruction, and highly structured and organized environments (Titus & Thio, 2009). This approach aligns with DEC Best Practice C.25, Specialized procedures (e.g., naturalistic strategies and prompt/prompt fading strategies) are embedded and distributed within and across activities (Sandall et al., 2005, p. 91). Tools of The MindAccording to Titus and Thio (2009), classroom accommodations designed to address deficits in executive functioning are often sufficient to help with AED-related memory issues (p. 888). The Tools of The Mind curriculum was designed to address executive functioning in young children, as well as symbolic representation and early math and literacy skills. According to Barnett et al. (2008), the Tools of the Mind curriculum focuses on broad foundational skills, including childrens ability to regulate their own social and cognitive behaviors, to attend and remember on purpose, the use of symbolic representation, and early math skills (p. 301). The curriculum was designed to help children create their own set of psychological tools which will help them in social and cognitive situations. Barnett et al. (2008) wrote, The Tools curriculum incorporates 40 Vygotsky-inspired activities designed to promote mature dramatic play, encourage the use of self-regulatory private speech, and teach the use of external aids to facilitate attention and memory (p. 301). One such tool is the use of mediators, something that stands as an intermediary between an environmental stimulus and an individual response to that stimulus (Bordova & Leong, 2007, p. 51). Mediators are used to develop attention and memory in young children. Examples of mediators may include being given a carpet square to sit on during circle time to help the child remember where to sit, a song that cues an activity being nearly over, or a picture symbol to hold while remembering to listen (Bordova & Leong, 2007). Another tool in the curriculum is the guided use of private speech to support thinking. Teachers encourage and model using private speech while working through challenging tasks. Barnett et al. (2008) conducted a randomized trial comparing 88 children in Tools of the Mind classrooms to 122 children in control group classrooms in a single school district. The authors found that children in Tools classrooms showed improvements in social and cognitive development. Diamond, Barnett, Thomas, and Munro (2007) also found that children in Tools of the Mind classrooms preformed better on tests of executive functioning than did their peers in typical preschool classrooms. Diamond et al. (2007) summarized their results, Tools successfully moves children with poor [executive functions] to a more optimal state (p. 1388). Physical Activity for Improved AttentionBecause many children with epilepsy struggle with attention, strategies to improve attention are necessary in the classroom. A study by Palmer, Miller, and Robinson (2013) investigated the relationship between vigorous physical activity and improved attention in preschool-age children. In the study, the researchers had children in the exercise condition group participate in 30-min of planned movement activities, led by a kinesiology graduate student. The control group participated in 30-min of sedentary classroom-based activities, such as crafts, stories, and dramatic play. Immediately following the 30-min period, children completed the Picture Deletion Task for Preschoolers (PDTP; Corkum, Byrnes, & Ellsworth, 1995), which tested their sustained attention and response inhibition. Children in the exercise group produced fewer errors on the PDTP and showed greater sustained attention to the task. Children with epilepsy will likely benefit from increased physical activity. Many parents worry about seizure related injuries and will keep children under close watch. Roberts and Whiting (2011) found that many parents found it necessary to put activity restrictions on their children with epilepsy. Conversely, the authors also wrote that many parents believed that their childs school placed unnecessary restrictions on their child in the name of safety. Case StudyMalik is a 45-month-old boy with epilepsy. Malik was diagnosed at 11 months after a series of partial seizures. He lives with both of his parents and has an older sister (7 years) and a baby brother (9 weeks). His maternal aunt has epilepsy. Because of his aunts epilepsy diagnosis, Maliks doctors believe that his seizures have a genetic cause. Maliks parents report that he has met all developmental milestones as expected and that he is a happy and talkative little boy. His teachers, however, report that Malik is slower to follow directions in class and have some concerns about his attention/engagement in class as well as his memory skills. Maliks Leiter IQ score was 89 (23rd percentile). Malik was put on an antiepileptic drug at 18 months, after experiencing several additional complex partial seizures. He took the medication for about two years, at which time his parents and his neurologist determined that Maliks condition was stable and that he could temporarily stop taking the medication. He has been off of his medication and seizure-free for one month. Maliks doctors are pleased that the medication worked so well for Malik and are cautiously optimistic that his seizures may not return. Currently, he has no specific diagnosis beyond epilepsy, as it remains to be seen whether he will continue to have seizures and what the frequency and severity of the seizures might be. His parents continue to monitor him closely. Intervention PlanMaliks teachers can make a number of changes to help Malik be successful in school. School-based interventions should be utilized because his parents did not report any troubles at home. By utilizing best practices for children with disabilities and specifically for those with epilepsy, it is likely that Malik will have an easier time with the tasks required of him. Specifically, interventions are needed to help Malik develop his attention, memory, and direction following ability. Because Maliks IQ falls within the range of normal, interventions should be used to combat these specific deficits rather than cognition in general. Maliks memory may have been affected by his epilepsy or by the medication he took. As Malik is no longer on an AED, he may experience improved attention and memory with time. AEDs affect children in a variety of ways, and it is possible that his medication had a negative impact on his cognitive skills. To support Maliks memory his teachers should implement thematic teaching practices. Repetition of concepts throughout the school day and across settings will help Malik. Collaboration amongst teachers and interventionists who work with Malik will help him learn new concepts. For example, if Maliks class is learning about insects, the teachers can read stories about insects, observe insects during science class, act like insects during movement periods, sing insect songs during music class, and complete insect-themed art projects. Receiving this information throughout his day and across environments makes it more likely that new concepts will be learned. Adults working with Malik should also strive to keep his routines and behavioral expectations consistent across settings. Maliks day should be highly structured, with visual scheduling prompts to help reinforce the order of activities. When the demands of the day are consistent and known, it is likely that Maliks attention will be stronger during demanding tasks.Because his teachers find that Malik struggles with following directions, tasks should be broken down to simple parts and directions should be short and involve non-verbal prompts. Along with clear and consistent expectations, using visual prompts will help Malik as he moves through his school day. Tools such as an individual picture schedule and other visual or physical prompts can be utilized to support Malik. In addition to these classroom-based interventions, Maliks teachers and family should incorporate adequate physical activity into Maliks day. If playground or gross motor play is not available during the school day, his parents should try to have him ride a bike or scooter to school or visit the playground shortly before the school day begins. This burst of physical activity may help Malik with attention at school.With all of these strategies being implemented, Malik will have improved school performance. Epilepsy is a complicated condition that has a variety of neurological developmental implications. If these interventions are not successful, Tools of the Mind strategies would be a possible next step.

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