Table 1.

Definitions.

Operational (practical) clinical definition of epilepsy (8)

Epilepsy is a disease of the brain defined by any of the following conditions: • 1. At least two unprovoked (or reflex) seizures occurring >24 h 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.

Status epilepticus (7)

“A condition resulting either from the failure of the mechanisms responsible for seizure termination or from the initiation of mechanisms, which lead to abnormally, prolonged seizures (after time point t1). It is a condition, which can have long-term consequences (after time point t2), including neuronal death, neuronal injury, and alteration of neuronal networks, depending on the type and duration of seizures.”

Super refractory status epilepticus (7)

SE continues for more than 24 h after the first administration of general anesthesia.

Autoimmune encephalitis (9)

Autoimmune encephalitis encompasses a wide variety of protean pathologic processes associated with the presence of antibodies against neuronal intracellular proteins, synaptic receptors, ion channels and/or neuronal surface proteins.

Rasmussen encephalitis (10)

Unilateral hemispheric encephalitis whose main clinical features include refractory focal epilepsy or epilepsia partialis continua, hemiparesis, and progressive cognitive decline.

New-onset refractory status epilepticus (NORSE) (11)

NORSE is a clinical presentation, not a specific diagnosis, in a patient without active epilepsy or other preexisting relevant neurological disorder, with new onset of refractory status epilepticus without a clear acute or active structural, toxic or metabolic cause.

Febrile infection-related epilepsy syndrome (FIRES) (11)

FIRES is a subcategory of NORSE, applicable for all ages, that requires a prior febrile infection starting between 2 weeks and 24 h prior to onset of refractory status epilepticus, with or without fever at onset of status epilepticus.

Electrical status epilepticus in sleep (ESES) (12)

Electrical status epilepticus in sleep (ESES), a childhood-onset epileptic encephalopathy, is characterized by epilepsy, cognitive regression, and marked activation of epileptiform activity during non-rapid eye movement (NREM) sleep to produce an electroencephalography (EEG) pattern of near-continuous spike-wave discharges.