Introduction: Absence status epilepticus in patients with absence epilepsy is uncommon. Absence status as the first presentation of absence epilepsy in children is even less common and not well documented in the medical literature.

Method: This is a retrospective study by means of chart review in a cohort of patients who presented with absence status epilepticus at the Children's Hospital of Eastern Ontario, Canada, between January 2008 and November 2013 inclusive.

Results: We identified 8 patients with a diagnosis of absence epilepsy and a history of absence status epilepticus. Half of the cases presented with absence status as their first presentation. In these, the patients presented to the ED with a 4 to 36 hour history of ongoing confusion. Absence epilepsy was confirmed by EEGs. All 4 responded to classical first line anticonvulsivants for absence seizures and had no more confusional states during follow up.

Conclusion: Non convulsive status, especially absence status, should be considered in the differential diagnosis in children presenting in the ED with acute prolonged or recurrent confusional state, even in the absence of a prioir history of epileptic seizures.