A seizure results from a disturbance in the brain’s electrical system in the form of an abnormal, hypersynchronous firing of cortical neurons. Its manifestations may be behavioral or sensory and these may be obvious or subtle. An individual seizure may have a variety of causes, such as fever in a young child, hypoxia, or infection. Recurrent seizures in the absence of provoking stimuli, such as active infection or fever, are known as epilepsy (5). Nonepileptic seizures, under which psychogenic seizures are classified, may appear similar to epileptic seizures but are not caused by electrical disruptions in the brain. The time leading up to a seizure, the seizure itself, and the time immediately after a seizure are respectively referred to as the preictal, ictal and postictal periods. The time in between seizures is called the interictal period. While seizures are the defining and most dramatic manifestation of epilepsy, they are episodic, whereas the brain dysfunction underlying them continues during the interictal periods, as can the associated psychosocial impact. Thus seizures themselves are rarely what is most impairing for children with epilepsy (Table 7.1.3.4.1).

Each year, approximately 150,000 children and adolescents in the United States will have a seizure of some type (6). The vast majority are children under the age of 5 who experience a febrile seizure. Each year, recurrent unprovoked seizures, or epilepsy, will develop in up to 7 in 10,000 children under the age of 15 (7), or in approximately 42,000 children. Across developed countries the annual incidence is similar (8,9). In U.S. surveys, the incidence of epilepsy is greatest among the very young and the elderly and is generally higher in males, after the age of 5 (10). Whether the incidence of epilepsy is higher in African Americans or populations with lower socioeconomic status, as reported by some (11,12), is contradicted by others (13,14). In any given year 1 in 200 children under 15 will have epilepsy (7), or about 300,000 children. Across age groups, in two-thirds of patients the etiology is unknown (10). Populations at special risk for epilepsy are those with a single unprovoked seizure, autism (15), mental retardation, cerebral palsy (16), and children of parents with epilepsy (17).

Approximately 70% of people with epilepsy will go into remission, defined as five or more seizure-free years, whether on or off medication (18). Prognosis will vary according to age and seizure type, with a better prognosis for childhood onset epilepsy without neurological or developmental disabilities. Only 35% of those with mental retardation, cerebral palsy, or other neurological condition will enter remission (19). A decade’s longitudinal followup study found that more than one-third of patients with childhood onset epilepsy with more than 5 years of remission who then stopped their medications relapsed within the next 5 years (20). Approximately 10% of child patients newly diagnosed with epilepsy will go on to have intractable epilepsy (21). The course and prognosis for the most common psychiatric comorbidities of epilepsy have not been well studied. However, it is clear that for many patients these continue long after seizures have stopped.

The first recorded description of epilepsy dates to Babylonian clay tablets from 2000 BC (22). Hippocrates also wrote about epilepsy in 400 BC. He described it as having a natural cause, like other medical conditions, rather than being a divine curse as previously thought. He noted the association with depression and wrote that “melancholics ordinarily become epileptics, and epileptics melancholics: what determines the preference is the direction the malady takes; if it bears upon the body, epilepsy, if upon the intelligence, melancholy.” (23). In the middle ages, some held the view that seizures were characteristic of witches or that witches caused epilepsy (24). Mid-nineteenth century England considered epilepsy a form of insanity (25). In the late nineteenth century, with the advent of neurology as a specialty and the successful use of bromide to treat epileptic patients, epilepsy became increasingly viewed as a brain disorder, and its social stigma attenuated. However, into the twenty-first century, some segments of the public continue to have highly negative perceptions of epilepsy (26).

Although epilepsy in modern medicine is not viewed as a simple causal explanation for severe psychiatric illness, the overlap between epilepsy and psychiatry is extensive and has been increasingly recognized. Seizures can produce alterations in consciousness that can mimic psychiatric symptoms in the ictal and periictal periods, such as commonly occurs, for example, when the seizure starts in the temporal lobes (27). There is also a high prevalence of psychiatric comorbidity in the interictal period. Clearly, the presence of epilepsy has neuropsychiatric and psychological associations, if not sequelae. These problems can be conceptualized along two domains: 1) The brain dysfunction causing seizures, the seizures themselves, and adverse effects of antiepileptic drugs, and their potential contribution to psychiatric pathology, through common physiologic mechanisms, and 2) the psychosocial and psychological consequences of epilepsy or its treatment, irrespective of any biological overlap.

Regardless of etiology, the psychiatric considerations include the child’s concerns about self-image and self-esteem, which can be related to the seizure disorder itself or side effects of drug treatment; intrapsychic issues related to temperament and coping skills; psychosocial issues, such as withdrawal and social isolation in contrast to affiliation; the cognitive impact of seizures or their treatment, including decrements in IQ or other neuropsychologic changes; the impact of the disorder or its treatment on daily living (28); and the developmental impact of any or all of these on the individual child in maintaining his or her developmental trajectory.

Learning to live with this chronic, at times severe, disorder while remaining psychiatrically healthy can be a challenge for some children. Child psychiatrists can play an active role in recognizing and treating these psychiatric repercussions or in helping to educate others to do so. With the aim of better helping children who have epilepsy and comorbid psychiatric disorders, it is useful to have a familiarity with the fundamental diagnostic, classification, and treatment considerations in epilepsy. This is reviewed in the following sections, before turning back to psychiatric aspects in more detail.

Most evaluations begin, naturally, with the clinical suspicion that a child is having some form of seizure activity. These can range from the dramatic, such as outright tonic-clonic activity, to the subtle, such as brief staring spells. Other common presentations include unusual sensations, repetitive movements, automatic behaviors, or altered consciousness. Most seizures are brief, lasting from a few seconds to several minutes. Afterward, depending on the type of seizure, a person may have a headache, confusion, muscle soreness, fatigue, odd sensations, or feel nothing at all. Like all diagnostic evaluations, one begins with a detailed clinical history, which is the single most important exam in making a diagnosis, as frequently, diagnostic testing, including EEG— which may not capture definitive interictal clues of seizure activity— will not yield any findings. Since the patient himself may have a poor recollection of the event, collateral information is essential. Generally, one wants to know at minimum the time and speed of onset; the presence of abnormal movements; whether bladder control was maintained; whether the patient bit his lips or tongue; how long the episode lasted; and how long it took for the patient to recover after the episode. The patient can be asked directly how he felt after the event and whether or not he had any premonitions of the event; these can include aural, visual, tactile, or olfactory sensations, among others. The clinical history and exam will help determine the additional workup needed. This usually includes an encephalogram (EEG). Unless the exam is suggestive of obvious triggering pathology, such as a high fever in an infant or a headache, vomiting, and focal neurological findings in a teenager, which would suggest the need for other diagnostic testing first, the single most useful tool in evaluating unprovoked seizures is the EEG.

In the late nineteenth and early twentieth century, the EEG was an experimental tool applied to the scalp of animals to examine electrical currents in the brain. In 1929 German psychiatrist Hans Berger published a report on his experiments using it to record the fluctuating electrical potentials of the human cortex (29). Soon, the EEG became the prime tool in diagnosing seizures and it remains decades later, now along with neuroimaging, a key instrument in diagnosing epilepsy.

To understand epilepsy and its various syndromes, one must also be familiar with the basic terminology used to describe EEG tracings. EEGs are traditionally acquired using the 10–20 international electrode placement system, in which electrodes are placed in a bipolar fashion from the nasion, where the frontal and nasal bones join, to the inion, the prominent point of the occipital bone.

The EEG starts with the technician asking the patient to close her eyes, or in the case of children, placing a hand over the child’s eyes. This is done to bring out the alpha rhythm, which is the predominant rhythm of the brain during wakefulness. Opening the eyes will usually attenuate the rhythm, and other waveforms, such as theta and beta, will appear. While drowsy, more theta and delta waveforms appear in the brain, slowly progressing to more attenuation and the onset of stage 1 sleep. During the acquisition of the EEG, certain maneuvers are attempted to provoke seizures, including hyperventilation and photic stimulation. The response of the brain to these maneuvers helps the observer to determine if the patient is within the normal range, or may be encephalopathic. For example, an encephalopathic child will have prolonged delta after hyperventilation, and may have a slower than normal background at baseline. A patient with absence epilepsy can have an otherwise normal background, but with the onset of hyperventilation, a 3 Hz spike and wave morphology appears along with behavioral arrest. The EEG is also useful because certain epilepsy syndromes have typical and at times diagnostic EEG patterns.

Several elemental EEG waveforms are presented in Table 7.1.3.4.2. In most children, an alpha rhythm with an 8 Hz frequency is reached by age 3 years.

A seizure’s manifestations depend on where it starts and where it spreads. Recalling that the definition of epilepsy is recurrent seizures that occur spontaneously in the absence of provoking stimuli, an isolated seizure is not considered to be epilepsy. An epilepsy syndrome is defined by a distinguishing pattern of signs, symptoms, and history. These include the seizure type, whether it starts at an identifiable place or focus in the brain (a focal, partial or localization-related seizure), or whether it does not (a generalized seizure or one with bihemispheric involvement from the start). Partial seizures are further classified according to whether they cause alterations in consciousness (complex partial) or consciousness remains intact (simple partial seizure), and whether they progress to generalized tonic-clonic seizures (with secondary generalization) or not. Seizures are called unclassified or undetermined if there is insufficient information on whether they have a focal onset or if they occur in the neonatal period. The definition of an epilepsy syndrome also commonly includes the age of onset, history, including family history, physical exam, EEG findings during and between seizures, etiology, imaging findings, and prognosis. An idiopathic epilepsy syndrome is one in which there is only epilepsy with no underlying structural brain lesions or other neurologic signs or symptoms. These are usually presumed to be genetic and are usually age dependent. An epilepsy syndrome is considered “symptomatic” if there is an identifiable structural cause such as a tumor or infarct. An epilepsy syndrome is considered “cryptogenic” if it is presumed that there is an underlying structural cause even though it is undiscoverable at this time, for example if a child has moderate mental retardation plus epilepsy but normal neuroimaging studies. A benign epilepsy syndrome is
one characterized by epileptic seizures that are easily treated, or require no treatment, and remits without sequelae.

In 1989, the International League Against Epilepsy (ILAE) met to determine a classification scheme for epilepsy syndromes. The currently approved ILAE classification divides epilepsies into focal- or localization-related syndromes versus generalized ones, and according to etiology. There is also a newer classification system that is under development to further classify seizures according to ictal semiology, seizure type, and syndromes. The complete ILAE classification of epilepsy syndromes is presented in Table 7.1.3.4.3.

Common causes of seizures in the first year of life are perinatal complications, febrile seizures, infections, metabolic problems, and ischemic events. In adolescents and young adults, common causes are trauma, congenital abnormalities, tumors, and central nervous system infections. A concern germane to psychiatrists is differentiating paroxysmal nonepileptic events and nonepileptic seizures from true epilepsy. Complicating matters, patients with nonepileptic seizures may also have concomitant epileptic seizures. Video EEG monitoring is the ideal modality to differentiate these events.

In the first year of life, generalized seizures are the most common type. The incidence then declines and remains constant throughout childhood and adulthood. Thereafter, partial seizures are the most common type in childhood (30), and the incidence is constant until after age 65 years, when a precipitous increase occurs attributable to cerebrovascular disease. Absence (or petit mal) seizures, considered in the differential diagnosis of ADHD, rarely occur in the first year, peak from the ages of 5 to 10 years, and then decline, being quite rare by the age of 30. The incidence of various epilepsy syndromes in newly diagnosed children is described in Table 7.1.3.4.4, while clinical features and treatment of the various epilepsy syndromes commonly found in children is described in Table 7.1.3.4.5.

Children often have behaviors that are paroxysmal and may be mistaken for seizures. In most cases, these events are easily defined and there is no treatment needed. There are some instances, however, when the only way to differentiate these events from true seizures is to use video EEG to confirm an EEG correlate. To ascertain a true seizure, determining the duration of the event, observing the presence of a postictal state, and obtaining a history to uncover any provocative stressors are all useful.

Behavioral events that may appear epileptiform were examined by Kotagal and colleagues in a retrospective study looking at 134 children and adolescents admitted to their pediatric epilepsy unit. They found that in children less than 5 years the common diagnoses were parasomnias and sleep jerks. In the older age group, nonepileptic seizures were common (31).

Behaviors that can raise the concern for epilepsy but are not epileptiform in etiology are outlined in Table 7.1.3.4.6.

Nonepileptic seizures (NES) can be classified as physiologic or psychogenic. Physiologic NES are events that resemble seizures but are not caused by epileptiform discharges; these include, for example, syncopal episodes or hypoglycemia. Psychogenic NES refer to behaviors that may resemble seizure activity, but which also are not epileptiform, and are rooted in a psychological etiology. In DSM IV TR, if they are volitional, NES might be classified as factitious disorder with predominantly psychological signs and symptoms or as malingering if secondary gain is a motive. If the NES are not volitional, conversion disorder with seizures or convulsions is the appropriate diagnosis. NES are well documented in adult populations, and approximately 5 to 20% of patients with epilepsy also have nonepileptic seizures. Nonepileptic seizures occur in children, but can be more difficult to delineate from true epilepsy as they are not as common. Moreover, children diagnosed with paroxysmal nonepileptic seizures (PNES) also often carry a diagnosis of EEG-confirmed epilepsy. For example, one study of children with intractable seizures found that one-fifth had nonepileptic seizures, and three-quarters of these children also had documented epileptic seizures (32). Kotagal and colleagues found that 15% of all patients monitored on their pediatric epilepsy unit had PNES; of these, close to half also had epilepsy (31).

The presence of nonepileptiform seizures in children is attributable to a variety of precipitants, most of which have some identifiable psychological or psychosocial component. In adults they are frequently attributed to dissociative phenomena (33), posttraumatic stress disorder (34), and mood disorders. In one study designed to study the psychiatric features of children with nonepileptic seizures, severe environmental stress and major mood disorders were the most common findings (35). The most common environmental stressor involved the family, including parental divorce, parental discord, or the death of a close family member. Sexual abuse was the second most common environmental stressor, occurring in 32% of children. Separation anxiety with school refusal was found in 24% of the children. In their study, Bhatia and Sapra found that school phobias and fear of examinations were the most common precipitating factors (36). Some investigators have found that a family history of epilepsy is commonly found in children with nonepileptic seizures (37).

What does PNES look like? Many patients mimic their own seizures (38). Lancman and colleagues found unresponsiveness with generalized violent and uncoordinated movements or generalized trembling to be the most common manifestations (37). Others have found that tremors, intermittent stiffening, out of phase hand movements, and kicking and thrashing of the legs are most common, especially among adolescents; younger children often present less dramatically with staring or closing of the eyes and no responsiveness (39).

Diagnosing nonepileptic seizures can be difficult. If a typical nonepileptic event is captured on video without an EEG correlate, there is a greater likelihood that the event is nonepileptic. However, frontal lobe seizures occasionally present without an EEG correlate. Hence, clinical observation is crucial. Generally, an event that has a duration of more than 10 minutes, that does not have a postictal state, and in which the movements are neuroanatomically incongruent with a seizure, is unlikely to be epileptic. Early psychiatric intervention is warranted to address these behaviors and prevent them from becoming more entrenched (40).

Epilepsy is a common comorbidity in children with mental retardation, with a direct correlation between the severity of intellectual compromise and the severity of chronic epilepsy. The prevalence of mental retardation is about 1% of the general population (41), but epilepsy is found in approximately 20–40% of children with mental retardation, depending on its severity (42). In addition, about 40% of children with epilepsy have mental retardation. The age at which epilepsy presents is related to the etiology of the mental retardation.

In children with mental retardation, it can be difficult to differentiate seizures from behavior, as this population has more frequent repetitive and stereotyped behaviors than the general population. Several types of nonepileptic events are associated with mental retardation and include self-stimulation, hyperventilation, Sandifer syndrome, spasticity, clonus, dystonic posturing, and choreoathetosis (43). A study by Donat and Wright examined the most common types of behavior in a sample of 31 girls with mental retardation. They found 23% had behavioral staring, 40% had abnormal eye movements, and 42% had tonic posturing (44). As a result, a misdiagnosis of seizures can occur.

Several studies have investigated the age at which seizures present in mental retardation. One study of 98 children found that the average age of the first seizure was 1.3 years, with an earlier onset in children with severe mental retardation and a later onset for patients with mild mental retardation (1). Another study of 151 children with mental retardation, found that 69% had epilepsy by age 3 years without difference between severe and mild mental retardation for onset before or after age 3. Partial seizures predominated, with 72% of the children having these and 28% having generalized seizures. In this population, as in most studies, a prenatal cause for the epilepsy was found in more than 40% (45). When severe epilepsies occur in infancy, they profoundly impact neuropsychological development (46). They are also usually quite difficult to control with AEDs, with many patients undergoing multiple AED trials before seizure control is improved.