De novo absence status epilepticus in three paediatric patients: a new idiopathic epilepsy syndrome?

Absence status epilepticus (ASE) is a prolonged generalized absence seizure that usually lasts for hours and can even last for days. The main symptom is the altered content of consciousness while the patient may be alert and partly responsive. We describe the electroclinical features, treatment, and evolution of three paediatric patients with de novo ASE with an excellent response to valproic acid (VPA). Three paediatric patients presented with non-convulsive status epilepticus and an acute confusional state with impaired consciousness and EEG abnormalities compatible with typical ASE, associated with generalized spike-and-wave paroxysms at 2.5-4 Hz, as the first epileptic manifestation at eight, three, and nine years of age, respectively. No significant personal and/or family history was reported. None of the patients had absence seizures or any other type of seizure before the occurrence of the ASE. All of them responded well to VPA and had a benign disease course. Neuroradiological imaging was normal in all patients. These three cases presented with ASE as the first manifestation of their epilepsy; none of them had any other type of seizure before the event or during their follow-up, which was long-term in one. All patients had an excellent response to VPA. Our three cases presented with generalized typical ASE as the first manifestation of their epilepsy. De novo ASE might be considered as a well-defined idiopathic epilepsy syndrome or a variant of an idiopathic generalized epilepsy syndrome, such as a particular type or variant of childhood absence epilepsy.