SCOPE OF WORK

In 2010, the Institute of Medicine (IOM) was asked to examine the public health dimensions of the epilepsies with a focus on four areas:

• public health surveillance and data collection and integration;
• population and public health research;
• health policy, health care, and human services; and
• education for providers, people with epilepsy and their families, and the public.

The committee's statement of task (Box 1-1) details the request for realistic priorities and recommendations in these four areas. The committee was asked not to examine biomedical research priorities because the Epilepsy Research Benchmarks, developed in 2000, continue to be updated by the National Institute of Neurological Disorders and Stroke (NINDS) and collaborating agencies and organizations (NINDS, 2007a,b, 2010).

Box 1-1

INSTITUTE OF MEDICINE STUDY ON THE PUBLIC HEALTH DIMENSIONS OF THE EPILEPSIES: TASK AND SPONSORS. An ad hoc committee will conduct a study and prepare a report to recommend priorities in public health, health care and human services, and health literacy (more...)

To accomplish its task the IOM convened the Committee on the Public Health Dimensions of the Epilepsies, comprised of 17 members with expertise in epilepsy care, health services research, epidemiology, public health surveillance, mental health services, health care services and delivery, health literacy, public health, education, and communications. The IOM study had 24 sponsors: 12 federal agencies and 12 nonprofit organizations (Box 1-1). Vision 20-20, a coalition that includes many of the nonprofit organizations and federal agencies that sponsored the study, focuses on epilepsy research, care, services, education, and advocacy efforts.

The committee held five meetings and two public workshops during the course of its work (Appendix A). Throughout the study, many people with epilepsy and their family members and colleagues, as well as study sponsors and other organizations and individuals, provided compelling testimony to the committee about their concerns, burdens, joys, and challenges. The quotes throughout the report highlight some of the issues raised in testimony presented at the workshops and in e-mails to the committee.⁴ In addition to the meetings and workshops, a comprehensive review of the scientific literature and other available evidence formed a critically important part of the committee's efforts. The committee's work also benefited from information provided by sponsoring organizations, health systems, professional organizations, and others on specific topics (e.g., health education programs, health services use). Underpinning all its work was the committee's desire to set forth practical, action-oriented goals to improve the health and well-being of people with epilepsy and their families.

This report provides the committee's findings, research priorities, and recommendations and documents the evidence base. The report was written for a broad audience, including people with epilepsy; family members; health care and human services providers; local, state, and national policy makers; researchers; and foundations and nonprofit organizations.

EPILEPSY IS A FREQUENTLY OCCURRING AND COSTLY NEUROLOGICAL DISORDER

Incidence and Prevalence in the United States

Epilepsy is the fourth most common neurological disorder in the United States after migraine, stroke, and Alzheimer's disease (Hirtz et al., 2007). For many neurological disorders (such as Parkinson's disease and Alzheimer's disease), the number of new cases (incidence) is highest in older adults, while others (such as autism spectrum disorders and cerebral palsy) may be congenital or appear in early childhood (Table 1-1). For the epilepsies, the incidence is bimodal—highest in both young children and older adults (Figure 1-1a), although epilepsy may occur at any point in the life span, with the total number of people in the population who have epilepsy (prevalence) increasing with age (Figure 1-1b).

TABLE 1-1

Prevalence and Incidence of Common Neurological Diseases and Disorders.

FIGURE 1-1a

Incidence of epilepsy by age—composite of 12 studies in developed countries, 1988–2005. SOURCE: Thurman, 2011.

FIGURE 1-1b

Prevalence of epilepsy by age—composite of selected U.S. studies, 1978-2005. SOURCE: Thurman, 2011.

An estimated 1 in 100 people in the United States has had a single unprovoked seizure or has been diagnosed with epilepsy (NINDS, 2011c). It remains challenging to determine the total number of people with epilepsy in the United States and, in particular, the extent of the disorder in various subpopulations (e.g., by age, gender, race/ethnicity, socioeconomic status, geography). The 2.2 million prevalence estimate is most accurately viewed as approximating a midpoint in a wide potential range of 1.3 million to 2.8 million people with epilepsy (Hirtz et al., 2007; see also ^{footnote 1}). This rather high degree of uncertainty exists because the population-based assessments of epilepsy prevalence are outdated and do not reflect the current size and diversity of the U.S. population (Chapters 2 and 3).

Global Burden of Epilepsy

Epilepsy is estimated to affect more than 65 million people worldwide, with more than 80 percent of people with epilepsy living in developing countries (Ngugi et al., 2010; Thurman et al., 2011). This disproportionate burden is reflected in prevalence estimates that are at least twice as high in developing countries compared to developed countries (Ngugi et al., 2010). These markedly higher rates may be explained in part by larger numbers of cases caused by specific infectious diseases endemic in some developing nations (Ngugi et al., 2010). Further, as shown in Table 1-2, in terms of impact on disability and premature mortality, epilepsy ranks fifth among mental health, neurological, and substance-use disorders in low- and middle-income countries (Collins et al., 2011).

TABLE 1-2

Global Burden of Mental Health, Neurological, and Substance-Use (MNS) Disorders.

Although data are scant and developed using varying methodologies (Leonardi and Ustun, 2002), a number of consistently identified barriers to healthy living confront people with epilepsy globally, including inadequate infrastructure (e.g., health care services and workforce, rehabilitation programs, social supports), poor access to medications and other treatments, limited public knowledge and awareness, and stigma (Dua et al., 2006).

Generally, the availability of diagnostic services and community services for people with epilepsy varies, with lower-income countries having fewer services (Dua et al., 2006). The “treatment gap,” or the difference between the number of people who need treatment for epilepsy and the number who receive it, is significant. While the treatment gap is less than 10 percent in many high-income countries, it rises to more than 50 percent in middle-income countries and more than 75 percent in low-income countries (Meyer et al., 2010). Furthermore, variations are seen within countries, with rural areas having a wider treatment gap than urban ones, which likely reflects some combination of inadequate access to services; stigma, negative beliefs, and discriminatory attitudes about epilepsy; and low health literacy (Ngugi et al., 2010). However, as described further below, stigma is universal: “[E]verywhere in the world it is a hidden disease” (de Boer, 2010, p. 631).

DEFINING THE EPILEPSIES

While most people only see the seizures themselves, there is far more to epilepsy. Being proactive in treatment means not only taking daily medication, but also participating in activities, talking to doctors or therapists as necessary, actively participating in school, and thriving at work.

–Elizabeth Musick

The occurrence of two or more unprovoked seizures separated by at least 24 hours is the broad operational definition of epilepsy (ILAE, 1993), which the committee uses for the purposes of this report. Seizures⁶ are, in essence, symptoms of epilepsy, and epilepsy is the disorder. However, the details are much more complex.⁷ Seizures differ from person to person with respect to their cause and severity, the areas of the brain involved, the location(s) and functions of the body affected, the effectiveness of medications and other treatments, and many other factors. These large and significant differences are why epilepsy, as noted, is understood as a spectrum of disorders—the epilepsies. More than 25 epilepsy syndromes and other epilepsy disorders have been delineated (Berg et al., 2010). While epilepsy is a chronic disorder, some people with epilepsy, particularly children, go into remission (Berg et al., 2001; Callaghan et al., 2007; Choi et al., 2011; Sillanpää and Schmidt, 2006).

Individuals with epilepsy are at risk not only for seizures, but also for a myriad of comorbid health conditions (i.e., conditions that occur in persons with epilepsy more than would be expected by chance; Chapter 3). Often the comorbidities that accompany epilepsy outweigh the burden of the seizures themselves. Common comorbidities that occur in epilepsy include cognitive dysfunction, such as memory, attention, or concentration problems; mental health conditions, such as depression or anxiety; and somatic comorbidities, such as sleep disorders, migraines, or cardiovascular disease. Other health problems can occur as a result of ongoing seizures, the cause of the epilepsy, or problems associated with the treatment, such as fractures and other injuries, osteoporosis, reproductive problems, and even death. Several disorders are causally related to developing epilepsy. These include traumatic brain injury, brain tumor, stroke, central nervous system infection, autism spectrum disorders, Alzheimer's disease, and genetic disorders (such as Rett syndrome and tuberous sclerosis complex, among others). Further, epilepsy is associated with substantially higher rates of mortality than experienced in the population as a whole (Chapter 3). Goals for epilepsy efforts focus on preventing seizures in people at risk, controlling seizures in those with epilepsy, eliminating side effects of treatments, and helping people with epilepsy and their families achieve a high quality of life.

Not all seizures or seizure-like events are epilepsy (Figure 1-2). One of the challenges for neurologists and other health care providers is to determine whether their patient is having seizures because of electrical activity in the brain and, if so, the seizures' type and cause. For some seizures, such as febrile seizures, the cause and treatment may be relatively straightforward (AAP Subcommittee on Febrile Seizures, 2011), although even a single seizure can have health and quality of life implications. Many medical problems including migraines, cardiac problems, or sleep disorders can give rise to events that appear similar to seizures. These seizure-like events, including those with a psychological basis, are not caused by electrical disturbances in the brain, and identifying the cause and determining appropriate treatments may be challenging (Binder and Salinsky, 2007; Devinsky et al., 2011).

FIGURE 1-2

Seizures and seizure-like events. NOTES: Neonatal seizures are seizures that occur in infants ≤ 4 weeks old (ILAE, 1993). While epilepsy can begin in the neonatal period, neonatal seizures are frequently reactive to an acute injury and often (more...)

TERMINOLOGY, STIGMA, MISPERCEPTIONS, AND CULTURAL BELIEFS

A major challenge for people with epilepsy, as well as for the epilepsy field, has been the multitude of ways that epilepsy is perceived and, in many cases, misperceived. The unpredictable nature of seizures, the feelings of helplessness of those who witness them, and the centuries of misperceptions and misinformation about epilepsy have resulted in people with epilepsy being stigmatized and isolated. Baker and colleagues (2008), for example, found that 36 percent of students with epilepsy said that they had kept their epilepsy a secret, because they did not want to be treated differently (23 percent of their parents also did not disclose the diagnosis because they did not want their son or daughter to face the potential stigma). This type of internalized stigma (i.e., “felt” stigma) can reduce quality of life even when seizures are well controlled by medications or other treatments. In a history of epilepsy, Eadie and Bladin (2001) wrote, “It can be safely said that epilepsy has been one of the least understood and most maligned of medical conditions” (p. 230).

Epilepsy was recognized as early as circa 1050 B.C.E. in Babylon, and Hippocratic writings talk about epilepsy as a disorder of the brain as early as circa 400 B.C.E. (Eadie and Bladin, 2001; Epilepsy.com, 2011c; Reynolds and Kinnier Wilson, 2008; Temkin, 1971). Throughout the centuries, associations of seizures with mental health conditions, witchcraft, and demonic or divine possession have resulted in terminology with negative and sensationalized connotations and led to cultural and societal beliefs, perceptions, and stereotypes about epilepsy that can be difficult to modify. For example, although depictions in the movies of characters with epilepsy are becoming more realistic, examples of characters with seizures being portrayed as violent or dangerous still persist, as do inaccurate and misleading depictions in print media (Baxendale, 2003; Krauss et al., 2000). Some cultural beliefs include misperceptions that the person with epilepsy is being punished for sins, has a lack of spiritual faith, is taking illegal drugs, or is possessed by spirits (Sirven et al., 2005; Szaflarski et al., 2006). As a consequence, people with epilepsy and their families may be faced with a lack of social support from extended family members, feelings of parental guilt, social isolation, embarrassment and fear (particularly connected with having a seizure in public), and discrimination. Although efforts are being made to correct these misconceptions and to better inform people about the epilepsies, doing so remains a challenge (Chapter 8). Awareness and sensitivity to individual and cultural beliefs about epilepsy and about medicine and health care (including traditional healing techniques in some cultures) are key considerations for efficient and effective interactions between health professionals and individuals with epilepsy.

Epilepsy-related terminology is complex. Historically, terms to describe the disorder have included “the sacred disease” and “falling sickness,” and, until recently, seizures have been termed “fits” and “spells.” The committee considered the terminology to be used throughout this report carefully. As noted above, epilepsy is a spectrum of disorders—ranging from severe, life-threatening, and disabling disorders to ones that are much more benign and sometimes transient. Therefore, the term “epilepsies” is more descriptive of the spectrum. The plural use of the term, however, can be cumbersome as in “people with the epilepsies.” In this report, for the sake of clarity, the committee generally uses the singular “epilepsy,” except when it is important to remind readers of the considerable spectrum of disability that the epilepsies represent. The report does not use the term “seizure disorders” because, as discussed above, there are a number of conditions that result in seizures that are not epilepsy.

The epilepsy field has moved away from the use of the term “epileptics” and toward the phrase “people with epilepsy,” just as other general terms such as “the disabled,” “the elderly,” and “the homeless” have been replaced with the phrases “people with disabilities,” “older adults,” and “homeless individuals.” Because the term “epileptic” has a pejorative connotation, the committee believes it should be discontinued. To paraphrase a 16-year-old with epilepsy, “Epilepsy is what I have, not who I am” (Clark, 2011). Throughout the report the committee suggests using more precise—and less negative terms—such as “seizure medications” (to replace “anti-epileptic drugs”), epilepsy seizures (to replace “epileptic seizures”), and “seizure-like events with a psychological basis” (rather than psychogenic, non-epileptic seizures).

OVERVIEW OF EPILEPSY

Types of Seizures and Syndromes

Although much remains to be learned about the neuroscience of the epilepsies and the causes of specific types of epilepsy, generally seizures are caused by excessive and hypersynchronized neuron discharges in the brain (McNamara, 1994; Pitkanen and Lukasiuk, 2011). These discharges can involve widespread areas of the brain simultaneously or be focused in one specific area. The effects of seizures on a person's health and well-being depend on the location and extent of the nerve cells involved; as a result, seizures can range from mild (such as a momentary loss of awareness) to severe (such as body convulsions).

Defining and categorizing the multiple types of epilepsy can be difficult. In 1964, the International League Against Epilepsy (ILAE) developed a classification system for epilepsy seizures and syndromes that continues to be updated (Arnautova and Nesmeianova, 1964; Berg et al., 2010). Because of the complex and disparate nature of where and to what extent the brain is affected by seizures, the epilepsies can be categorized according to multiple dimensions:

• Seizure type—Seizures are classified into two major categories: (1) focal seizures that originate in a network of neurons limited to one hemisphere of the brain and (2) generalized seizures that originate in a network of neurons that is distributed to both brain hemispheres (Berg et al., 2010). Seizures also can be categorized as of unknown type. Box 1-2 provides an overview of seizure types.
• Syndromes—Berg and colleagues (2010) recently defined a syndrome as “a complex of clinical features, signs, and symptoms that together define a distinctive, recognizable clinical disorder” (p. 681). Often, a syndrome is characterized by the typical age of onset, specific characteristics of the electroencephalogram (EEG), and seizure types. Table 1-3 provides an overview of a few of the many epilepsy syndromes.

Box 1-2

SEIZURE TYPES. Focal seizures originate within a network of neurons limited to one hemisphere of the brain, and the signs and resulting symptoms depend on precisely where the disruptions in brain activity occur. Focal seizures may have motor, sensory, (more...)

TABLE 1-3

Examples of Epilepsy Syndromes with Differing Severities.

As described by Engel (2001), in addition to seizure type and syndrome, other dimensions used to characterize the epilepsies can include the specific etiology (cause), the extent of impairment, and general descriptions of the seizure(s).

Most seizures last from fractions of a second to less than a minute and end on their own without intervention. However, sometimes a seizure does not stop spontaneously. Status epilepticus is usually defined as a prolonged seizure or series of seizures without full recovery of consciousness in between (Bazil and Pedley, 2009). In clinical care this generally involves seizures lasting longer than 5 minutes. Status epilepticus can occur in individuals who do not have a prior history of seizures. Status epilepticus is a neurological emergency and can be fatal.

In the past two decades, awareness has been raised about high rates of SUDEP; people with epilepsy have a more than 20 times higher rate of sudden death than does the general population (Ficker et al., 1998). Little is known about the causes of SUDEP, although rates are higher in people with seizures that are refractory⁸ (Tomson et al., 2005; Chapter 3).

CROSS-CUTTING THEMES

Throughout this report several cross-cutting themes are highlighted by the committee:

• Epilepsy is a common and a complex neurological disorder. Epilepsy is not a single disorder but rather a spectrum of disorders—the epilepsies. Further, epilepsy is more than seizures and may be accompanied by a range of associated comorbid health conditions that can have significant health and quality-of-life implications. Some people with epilepsy have lives that are essentially unchanged, while others' health and well-being are severely affected, and for some people, epilepsy is fatal. Communicating this range of outcomes and meeting the spectrum of needs are major challenges faced by the epilepsy field.
• Epilepsy often affects quality of life. For many individuals with epilepsy and their family members, living with epilepsy means challenges in school and work, social functioning and relationship dynamics, limits on driving, and daily worries about the possibility of seizures.
• A whole-patient perspective is needed. Because the effects of epilepsy go beyond health concerns and seizures, a whole-patient, patient-centered perspective is needed that provides people with epilepsy, their families, and caregivers with a coordinated, individual-specific approach to health care, mental health care, educational opportunities, and community services and promotes optimal self-management and quality of life.
• Effective treatments are available for many types of epilepsies, but timely referrals and access to those treatments fall short. For many people with epilepsy, seizures can be effectively reduced or eliminated by medications, surgery, devices, and dietary or other therapies. However, in the United States, referrals to epileptologists and epilepsy centers for surgical consultations can take 15 years or more.
• Data are lacking that could improve epilepsy care. Accurate, timely data on the extent and consequences of epilepsy and comorbid conditions and on health care and community services use and outcomes are sorely needed to make improvements in epilepsy prevention; diagnosis; health care access, quality, and value; and community services.
• Many health professionals need to be better informed about epilepsy. Improvements in epilepsy care can be made only if the quality and quantity of education about epilepsy for health care professionals are improved dramatically through undergraduate and graduate levels and lifelong learning programs.
• Education efforts for people with epilepsy and their families need to be thorough and sensitive to health literacy and cultural considerations. Across the continuum from initial diagnosis through ongoing treatments and services, people with epilepsy and their families need to be aware of the disorder's potential risks, including SUDEP, and the range of treatments and services available. Information must be conveyed in ways that are easily understandable and relevant to specific age groups and cultures.
• The stigma associated with epilepsy needs to be eliminated. The long history of epilepsy is full of examples of discrimination and secrecy due to misinformation and lack of understanding by the general public. Since stigma can have a detrimental effect on people with epilepsy, continued and sustained efforts are needed to raise public awareness and convey what epilepsy is and what it is not, as well as the basic messages embodied in these themes.

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Footnotes

1

The committee used the prevalence and incidence ranges from Hirtz and colleagues (2007) and applied them to a U.S. population number of 313,000,000. (The U.S. Census population estimate for January 30, 2012, was 312,933,845; www​.census.gov/main/www/popclock.html.) In the paper by Hirtz and colleagues (2007) the median for incidence, based on the four studies of all age groups, was 48 per 100,000; median prevalence rate for all age groups was 7.1 per 1,000.

2

Data are in 2004 dollars. As discussed later in this chapter and in Chapter 4, estimates of the cost burden of epilepsy vary widely and more data are needed on the use of health care services and on indirect costs.

3

Begley et al., 2000; Gaitatzis et al., 2004; Hauser et al., 1980; Hesdorffer et al., 2011; Hirtz et al., 2007; Thurman, 2011; Thurman et al., 2011; Tomson et al., 2008; Yoon et al., 2009.

4

Public testimony and other materials submitted to the committee are available by request through the National Academies' Public Access Records Office.

5

The lower estimate is in 2004 dollars and is an estimate of direct costs (Yoon et al., 2009). The higher estimate is in 1995 dollars, 85 percent of which is attributable to indirect costs (Begley et al., 2000).

6

An epilepsy seizure has been defined as a “transient occurrence of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain” (Fisher et al., 2005, p. 470).

7

This report does not provide an in-depth clinical description of the epilepsies; resources such as Ropper and Samuels (2009) and Bazil and Pedley (2009) can be consulted for additional information.

8

Refractory epilepsy is defined as the failure to control seizures after two seizure medications (whether as monotherapies or in combination) have been appropriately chosen and used (Chapter 4) (Kwan et al., 2010).

9

Neurologists with concentrated training in epilepsy are designated as epileptologists. A new subspecialty board certification in epileptology is being created by the American Board of Psychiatry and Neurology. A board-certification examination for epileptologists will be offered for the first time in 2013 (Chapter 5).

10

Self-management for epilepsy includes the information and resources that people with epilepsy and their families need to develop skills and behaviors that enable them to actively participate in patient-centered care; it is “the sum total of steps taken and processes used by a person to control seizures and manage the effects of having a seizure disorder” (DiIorio, 1997, p. 214). The committee adopted the concept of “optimal self-management,” recognizing that it represents a wide range of possibilities toward autonomy and independence and that what is optimal for one person may be beyond the capacity of another (Chapter 7).

11

Actual spending in fiscal year 2011 was $152 million (NIH, 2012).