CHILDHOOD ABSENCE EPILEPSY (CAE)

	Mandatory	Alerts	Exclusionary
Seizures	Typical absence seizures	Generalised tonic-clonic seizures before or during the period of frequent absence seizures Seizure duration >30s or with postictal confusion or fatigue Absence seizures less than daily (untreated)	Myoclonic-absence, atonic, tonic, atypical absence or focal seizures Prominent myoclonic or eyelid myoclonia seizures
EEG	2.5–4Hz generalized spike-wave at the start of an absence seizure	Consistently unifocal discharges Lack of hyperventilation (performed effectively for 3 minutes) activation of generalised spike-wave (untreated) Suspected absence captured without EEG abnormality Persistent background slowing (unsedated)	Diffuse background slowing
Age at onset		2–3 or 11–13 years	<2 or >13 years
Development at onset		Mild intellectual impairment	Moderate or greater intellectual impairment
Neurological exam		Abnormal, potentially clinically significant
Imaging		Abnormal, potentially clinically significant
Comorbidities			Cognitive plateau or regression
Other tests			Low CSF glucose and/or SLC2A1 pathogenic variant, testing is not mandatory but is suggested if ≤3 years of age at seizure onset, microcephaly or intellectual impairment
Are MRI or ictal EEG required for diagnosis? An MRI is not required for diagnosis An ictal EEG is not required for diagnosis, provided the interictal study shows 2.5-4Hz generalised spike-wave during wakefulness, however most untreated patients will have absence seizures captured on EEG
Syndrome without laboratory confirmation: in resource-limited regions, this syndrome can be diagnosed in patients without alerts who meet all other mandatory and exclusionary criteria, if there is a witnessed typical absence seizure facilitated by hyperventilation

NOTE Alert criteria are absent in the vast majority of patients with the syndrome, but rarely can be seen. Their presence should result in caution in diagnosing the syndrome and consideration of other conditions