A Revised Definition of Epilepsy

Epilepsy News From: Tuesday, April 15, 2014

Seizures and epilepsy are not the same. An epileptic seizure is a transient occurrence of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain. Epilepsy is a disease characterized by an enduring predisposition to generate epileptic seizures and by the neurobiological, cognitive, psychological, and social consequences of this condition. Translation: a seizure is an event and epilepsy is the disease involving recurrent unprovoked seizures.

The above definitions were created in a document generated by a task force of the International League Against Epilepsy (ILAE) in 2005. The definitions were conceptual, (theoretical) and not sufficiently detailed to indicate in individual cases whether a person did or did not have epilepsy. Therefore, the ILAE commissioned a second task force to develop a practical (operational) definition of epilepsy, designed for use by doctors and patients. The results of several years of deliberations on this issue have now been published (Fisher RS et al. A practical clinical definition of epilepsy, Epilepsia 2014; 55:475-482) and adopted as a position of the ILAE.

A commonly used definition of epilepsy heretofore has been two unprovoked seizures more than 24 hours apart. This definition has many positive features, but also a few limitations. This definition does not allow the possibility of "outgrowing" epilepsy. Inclusion of the word "provoked" seems to imply that people who have photosensitive seizures provoked by flashing lights or patterns do not have epilepsy; whereas, most people think that they do. Some individuals who have had only one unprovoked seizure have other risk factors that make it very likely that they will have another seizure. Many clinicians consider and treat such individuals as though they have epilepsy after one seizure. Finally, some people can have what is called an epilepsy syndrome and these individuals should meet the definition for having epilepsy even after just one seizure. You should not have an epilepsy syndrome but not epilepsy. The new definition of epilepsy addresses each of these points.

A person is considered to have epilepsy if they meet any of the following conditions.

  1. At least two unprovoked (or reflex) seizures occurring greater than 24 hours apart.
  2. One unprovoked (or reflex) seizure and a probability of further seizures similar to the general recurrence risk (at least 60%) after two unprovoked seizures, occurring over the next 10 years.
  3. Diagnosis of an epilepsy syndrome
    • Epilepsy is considered to be resolved for individuals who had an age-dependent epilepsy syndrome but are now past the applicable age or those who have remained seizure-free for the last 10 years, with no seizure medicines for the last 5 years.

In the definition, epilepsy is now called a disease, rather than a disorder. This was a decision of the Executive Committees of the ILAE and the International Bureau for Epilepsy. Even though epilepsy is a heterogeneous condition, so is cancer or heart disease, and those are called diseases. The word "disease" better connotes the seriousness of epilepsy to the public.

Item 1 of the revised definition is the same as the old definition of epilepsy. Item 2 allows a condition to be considered epilepsy after one seizure if there is a high risk of having another seizure. Often, the risk will not precisely be known and so the old definition will be employed, i.e., waiting for a second seizure before diagnosing epilepsy. Item 3 refers to epilepsy syndromes such as benign epilepsy with central-temporal spikes, previously known as benign rolandic epilepsy, which is usually outgrown by age 16 and always by age 21. If a person is past the age of the syndrome, then epilepsy is resolved. If a person has been seizure-free for at least 10 years with the most recent 5 years off all anti-seizure medications, then their epilepsy also may be considered resolved. Being resolved does not guarantee that epilepsy will not return, but it means the chances are small and the person has a right to consider that she or he is free from epilepsy. This is a big potential benefit of the new definition.

What will change as the result of this new definition? Although revision of the definition has generated some controversy, it is likely that real-world changes will be fairly minor. Some people will be able to say their epilepsy is resolved. Others may encounter the problems and stigma of being told that they have epilepsy after one seizure in some circumstances, rather than after two seizures. The definition might stimulate research on how likely another seizure is after a first seizure in various clinical circumstances. Governments and regulatory agencies, people who do therapeutic trials for epilepsy, insurance companies and other third-party payers might have to adjust some of their definitions. One reason changes will be small is that individuals with one seizure and a high risk for another are currently practically thought of as having epilepsy by many treating physicians. This process simply formalizes that thinking.

Making a diagnosis of epilepsy is not the same as deciding to treat. Some seizures are minor; some patients choose to avoid the side effects of medications. Treatment decisions will be individualized between a person with epilepsy and a physician. Sometimes, information is incomplete; for example, a possible seizure may not have been observed. In these conditions it can be impossible to confidently diagnose epilepsy using any definition. Clinicians will apply best judgment when faced with such incomplete information and often will wait for future developments.

This practical definition is designed for clinical use. Researchers, statistically-minded epidemiologists and other specialized groups may choose to use the older definition or a definition of their own devising. Doing so is perfectly allowable, so long as it is clear what definition is being used. In the process of developing the revised definition of epilepsy, consensus was reached by forging opinions of 19 co-authors of the publication, while accounting for criticisms by five anonymous journal reviewers and over 300 public commenters on the ILAE website. The revised definition is not perfect. It will become more useful over time as we gain better information on seizure recurrence risks. But for now, the new definition better reflects the way clinicians think about epilepsy.

**If you think that you do not or might not have epilepsy, please do not change any of your treatments and do not stop any medications without first consulting with your doctor.

Authored by

Robert Fisher MD, PhD

Reviewed Date

Tuesday, April 15, 2014

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