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Features seen in childhood absence epilepsy and juvenile absence epilepsy

Features seen in childhood absence epilepsy and juvenile absence epilepsy
Feature CAE JAE
Age at onset
Usual 4 to 10 years 9 to 13 years
Range 2 to 13; caution if diagnosing at <4 years of age 8 to 20 years; exceptional cases may present in adulthood
Development Typically normal, but may have learning difficulties or ADHD Typically normal, but may have learning difficulties or ADHD
Absences
Frequency At least daily to multiple per day but may be underrecognized by family Less than daily
Duration Typical duration = 3 to 20 s Typical duration = 5 to 30 s
Impaired awareness Severe loss of awareness Less complete impairment of awareness
Other seizure types
Febrile Occasional Occasional
Generalized tonic-clonic seizure Rarely precede or occur during period of frequent absences but may occur later with evolution to other IGE syndrome May precede and commonly occur during the period of frequent absences
Myoclonic Prominent myoclonus exclusionary Prominent myoclonus exclusionary
EEG background OIRDA in 21% Normal
Interictal epileptiform discharge
Awake 2.5 to 4-Hz generalized spike-wave 3 to 5.5-Hz generalized spike-wave
Asleep Polyspike and wave may be seen in drowsiness and sleep only Polyspike and wave may be seen in drowsiness and sleep only
Irregular generalized spike-wave Uncommon

More common than CAE

Discharges are more frequent than in CAE
Photoparoxysmal response

Rare

IPS triggers generalized spike-wave in 15 to 21% but does not induce seizures

Rare

IPS triggers generalized spike-wave in 25% but does not induce seizures
Hyperventilation induction 87% 87%
Ictal EEG

Regular 3-Hz (range = 2.5 to 4 Hz) generalized spike-wave; 21% may have absences starting at 2.5-Hz spike-wave, and 43% may have absences starting at 4 Hz; if no generalized spike-wave is seen with hyperventilation for 3 min in an untreated patient, CAE can be excluded

Disorganized discharges* less frequent

Regular 3 to 5.5-Hz generalized spike-wave

If no generalized spike-wave is seen with hyperventilation for 3 min in an untreated patient, JAE can be excluded

Disorganized discharges* 8 times more frequent than CAE

CAE: childhood absence epilepsy; JAE: juvenile absence epilepsy; ADHD: attention deficit/hyperactivity disorder; IGE: idiopathic generalized epilepsy; EEG: electroencephalogram; OIRDA: occipital intermittent rhythmic delta activity; IPS: intermittent photic stimulation.

* Disorganized discharges are defined as either brief (<1 s) and transient interruptions in ictal rhythm or waveforms of different frequency or morphology during the ictal rhythm.
From: Hirsch E, French J, Scheffer IE, et al. ILAE definition of the idiopathic generalized epilepsy syndromes: Position statement by the ILAE Task Force on Nosology and Definitions. Epilepsia 2022; 63:1475. Copyright © 2022 The Authors. https://onlinelibrary.wiley.com/doi/10.1111/epi.17236 (Accessed on May 15, 2023). Reproduced under the terms of the Creative Commons Attribution License 4.0.
Graphic 141086 Version 1.0

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