Definition of epilepsy

1. Definition of epilepsy

2. In 2005, a Task Force of the International League Against Epilepsy (ILAE) formulated conceptual definitions of “seizure” and “epilepsy”.

3. The “two unprovoked seizure” definition of epilepsy has served us well, but it is inadequate in some clinical circumstances. • In order to bring the practical (operational) clinical definition of epilepsy into concordance with how epileptologists think about epilepsy, the ILAE Task Force recommends broadening the definition of epilepsy

4. High recurrence risk • Suchreserchmayincludepatientswith a singleseizureoccurringatleast a monthafter a stroke (Hesdorfferetal. 2009) or a childwith a singleseizureconjoinedwith a structuralorremotesymptomaticetiologyandanepileptiformelectroencephalography (EEG) study. Anotherexampleis a patientinwhomdiagnosisof a specificepilepsysyndromeassociatedwithpersistentthresholdalterationcanbemadeaftertheoccurrenceof a singleseizure. A firstseizuremightpresentasstatusepilepticus, butthisdoesnotinitselfimplyepilepsy. Recurrencerisksarenotknownforthemajorityofindividualcases. • Butif a treatingphysicianisawarethatthelesionhasgeneratedanenduringpredispositionforunprovokedseizures, thenthatpersonshouldbeconsideredtohaveepilepsy.

5. Itisimportanttonotethat a singleseizureplus a lesionor a singleseizureplusepileptiform EEG spikesdoesnotautomaticallysatisfycriteriafordefinitionofepilepsy, becausedatamayvaryamongdifferentstudiesandspecificclinicalcircumstances. • Accordingtodifferentstudiesrecurrenceriskafterfirstseizurevaryfrom 71-56 % during 2-3 years. • Noformulacanbeappliedforadditiverisks, suchcaseswillhavetobedecidedbyindividualizedconsiderations. Recurrenceriskis a functionoftime, suchthatthelongerthetimesincethelastseizure, thelowertherisk.

6. Implications for treatment • Diagnosing epilepsy after a single unprovoked seizure when there is high risk for recurrence may or may not lead to a decision to initiate treatment. The proposed practical definition may provide support to a physician who wishes to treat a patient with high recurrence risk after a single unprovoked seizure. However, a treatment decision is distinct from a diagnosis, and should be individualized depending upon the desires of the patient, the individual risk-benefit ratio and the available options. The physician should weigh the possible avoidance of a second seizure with associated risks against the risk for drug-related side effects and costs for the patients.

7. Epilepsy previously has been defined as at least two unprovoked seizures >24 h apart. The revised practical definition implies that epilepsy also can be considered to be present after one unprovoked seizure in individuals who have other factors that are associated with a high likelihood of a persistently lowered seizure threshold and therefore a high recurrence risk. Such risk should be equivalent to the recurrence risk of a third seizure in those with two unprovoked seizures, approximately at least 60%. The latter risk level occurs with remote structural lesions, such as stroke, CNS infection, certain types of traumatic brain injury, diagnosis of a specific epilepsy syndrome, or in some circumstances with the presence of other risk factors. Those with recurrent reflex seizures, for example, photosensitive seizures, are also considered to have epilepsy. This definition of epilepsy brings the term in concordance with common use by most epileptologists. • Epilepsy is not necessarily life-long, and is considered to be resolved if a person has been seizure-free for the last 10 years, with at least the last 5 year off antiseizure medicines, or when that person has passed the age of an age-dependent epilepsy syndrome. The new definition is more complicated than is the old definition.

8. Making the correct diagnosis • Medical history • The medical history should include: • details of the paroxysmal events (not only the most dramatic ones) as they have been experienced by the patient and witnesses • the circumstances under which the paroxysmal events occurred • timing and circadian distribution • position (standing, sitting or lying) • leisure or occupation (at rest or during exercise) • possible triggering, precipitating or facilitating factors • personal and family medical history.

9. Laboratorydiagnostic procedures The EEG, the most significant investigative procedure in the diagnosis of epilepsies, is often misunderstood, undermined and misused. Brain imaging, another top diagnostic procedure, provides in vivo visualisation of structural causes of epilepsy such as hippocampal sclerosis, malformations of brain development and tumours, as well as other brain diseases. Blood, urine have an important role in the evaluation of the child with epilepsy. Genetic testing has become available for a growing number of hereditary disorders associated withepileptic seizures.

10. Three important steps to take in order to make a correct specific diagnosis, which will determine prognosis and management: 1. First step: are the paroxysmal events epileptic seizures? 2. Second step: what type of epileptic seizures? 3. Third step: what is their cause and what is the epileptic syndrome or disease?

11. First step: are the paroxysmal events epileptic seizures? NEPEs that have been misdiagnosed as epileptic seizures affect as many as 20–30% of patients diagnosed with epilepsy; these patients have often received treatment for epilepsy for many years or have been admitted to tertiary care epilepsy units. The problem is complicated by the fact that approximately 30% of patients with genuine epileptic seizures also suffer from non-epileptic, mainly psycho genic seizures.

13. Second step: what type of epileptic seizures? Minor seizures are more important than major ones for diagnostic procedures, correct diagnosis and appropriate management strategies. Approximately three-quarters (74%) of patients with ‘newly identified unprovoked seizures’ (mainly GTCSs) had experienced multiple seizure episodes before their first medical contact.Yet, studies on the prognosis and treatment of the ‘first seizure’ mainly refer to a GTCS, although this may not be the first seizure in the patient’s life.

14. Third step: what is their cause and what is the epileptic syndrome or disease? • Important features of a syndrome include: • the type of seizures, their localisation and frequency • the chronological sequence of the events • circadian distribution • precipitating factors • age at onset • mode of inheritance • physical and mental symptoms and signs • response to treatment • prognosis. A syndromic diagnosis of epilepsies is now a basic recommendation of good clinical practice.