IEC 2021 | Update on the progression of status epilepticus

Status epilepticus is one of the most severe epileptic conditions. It’s not a disease, not an entity. It has myriads of different causes. Status epilepticus is known since thousands of years. The oldest descriptions are before Christ in the Babylonian inscriptions. But it has been defined recently, I would say. So the latest definition goes back to the ILE, International League against Epilepsy in 2015, which defines status epilepticus as a condition where seizures don’t stop according to the usual length. So they are longer than normal, which is abnormally prolonged after a time point T1. And which cause brain damage after certain periods. So the longer the seizures last, the more likely that neuronal damage occurs. So what we have to do is we have to identify seizures that are abnormally long and treat them in order to prevent neuronal damage.

So this new definition is widely accepted now, and it has been implemented in the treatment algorithms because it recognizes that we have to protect the brain from ongoing seizure activity. With the definition, there’s also a classification where mainly two clinical groups are identified. One is the one with major motor phenomena, and the other one is the one without major phenomena, and that has been called non-convulsive status epilepticus.

Within each of those groups, there are separate types of status epilepticus. Some of them are very rare, some of them are very common, and all of them have different causes. So the causes are very simple because it’s a medical emergency. Distinguishing symptomatic causes, where you really can identify the cause quite rapidly, or distinctly. And then you have status epilepticus of unknown cause, which is classically called cryptogenic or unknown. And the third group is those with electro clinical syndrome. Epilepsy syndrome, for example, absence epilepsy, and the children or the adults fall into a prolonged absence. So these are the three major groups, and their symptomatic is very important.

Acute symptomatic means that there is an overt insight on the brain or a major disturbance, which you have to treat immediately. It’s the underlying cause. And status will not improve unless you remove the cause or treat the cause appropriately. The second is remote symptomatic. So for example, you had a brain trauma, or an infarction, [inaudible] infarction some years ago, and then you will have a status later. That’s a remote symptomatic cause. And the third group is a progressive symptomatic, which is also important to identify early. It can be a metabolic disorder, a genetic disorder, or a tumor. So that’s the main framework of the definition and classification of status epilepticus.