JUVENILE ABSENCE EPILEPSY (JAE)

	Mandatory	Alerts	Exclusionary
Seizures	Typical absence seizures	Staring >30 s or with postictal confusion or fatigue Absence seizure frequency >10 per day	All other seizure types except generalised tonic-clonic seizures Prominent myoclonic and eyelid myoclonia seizures
EEG	3–5.5Hz generalized spike-wave at the start of the absence seizure	Lack of hyperventilation activation of 3–5.5Hz generalised spike-wave in an untreated patient (hyperventilation performed effectively for 3 minutes) Persistent background slowing (unsedated)	Consistently unifocal epileptiform discharges
Age at onset			<8 or >20 years
Development at onset		Mild intellectual impairment	Moderate or greater intellectual impairment
Neurological exam		Abnormal exam
Imaging		Abnormal, potentially clinically significant
Early Comorbidities			Cognitive stagnation or decline
Other			Low CSF glucose and/or pathogenic variant in SLC2A1 (testing not needed in most cases, but recommended if microcephaly or intellectual impairment
Course of illness		Lack of generalised tonic-clonic seizure, in the absence of treatment with anti-seizure medication effective for that seizure type
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 3-5.5Hz 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 no exclusionary criteria, if there is a witnessed typical absence seizure seen 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