Differentiating between CAE and JAE [ICNApedia]

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====== Differentiating between CAE and JAE ======
  * Age at onset of CAE is considered to be 2 to 13 years of age and for Juvenile Absence Epilepsy (JAE) to be 8 to 20 years of age; there is five years of overlap[(:cite:pmid35503716>{{pmid>long:35503716}})].
  * Patients with JAE more often have GTCS and more frequently experience seizure-related injuries compared with patients with CAE
  * Valproate can be considered as the drug of choice in men and lamotrigine as the first drug of choice in women with JAE.
  * CAE has a good prognosis (most patients will become seizure-free off ASMs[(:cite:pmid30856420>{{pmid>long:30856420}})], whereas in JAE many patients have poor seizure control and the syndrome may last a long time[(:cite:pmid24684814>{{pmid>long:24684814}})].
<tabcaption caevsjae| CAE and JAE. Updated diagnostic criteria for IGE syndromes determined by the expert consensus opinion of the ILAE's Task Force on Nosology and Definitions (2017-2021) and international external experts outside our Task Force>
^ Feature                                                                                                                                                                                                                                                                                                                                                                                                                                                                                          ^ CAE                                                                                                                                                                                                                                                                                                               | JAE                                                                                                                                                                                                                |
| Age at onset                                                                                                                                                                                                                                                                                                                                                                                                                                                                                     |                                                                                                                                                                                                                                                                                                                   |                                                                                                                                                                                                                    |
| Usual                                                                                                                                                                                                                                                                                                                                                                                                                                                                                            | 4–10 years                                                                                                                                                                                                                                                                                                        | 9-13 yrs                                                                                                                                                                                                           |
| Range                                                                                                                                                                                                                                                                                                                                                                                                                                                                                            | 2–13; caution if diagnosing at <4yrs of age                                                                                                                                                                                                                                                                       | 8–20 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 = 3–20 s                                                                                                                                                                                                                                                                                         | Typical duration = 5–30 s                                                                                                                                                                                          |
| Impaired awareness                                                                                                                                                                                                                                                                                                                                                                                                                                                                               | Severe loss of awareness                                                                                                                                                                                                                                                                                          | Less complete impairment of awareness                                                                                                                                                                              |
| Other seizure types                                                                                                                                                                                                                                                                                                                                                                                                                                                                              |                                                                                                                                                                                                                                                                                                                   |                                                                                                                                                                                                                    |
| Febrile                                                                                                                                                                                                                                                                                                                                                                                                                                                                                          | Occasional                                                                                                                                                                                                                                                                                                        | Occasional                                                                                                                                                                                                         |
| Generalized tonic-clonic seizures                                                                                                                                                                                                                                                                                                                                                                                                                                                                | 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                                                                                                                                                                                                                                                                                                                                                                                                                                                                                            | 2.5–4-Hz generalized spike-wave                                                                                                                                                                                                                                                                                   | 3–5.5-Hz generalized spike-wave                                                                                                                                                                                    |
| Asleep                                                                                                                                                                                                                                                                                                                                                                                                                                                                                           | Polyspike and wave may be seen in drowsiness and sleep only                                                                                                                                                                                                                                                       |                                                                                                                                                                                                                    |
| Irregular generalized spike-wave                                                                                                                                                                                                                                                                                                                                                                                                                                                                 | Uncommon                                                                                                                                                                                                                                                                                                          | More common than CAE                                                                                                                                                                                               |
| Photoparoxysmal response                                                                                                                                                                                                                                                                                                                                                                                                                                                                         | Rare\\ IPS triggers generalized spike-wave in 15%–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 = 2.5–4 Hz) generalized spikewave; 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 dischargesa less frequent  | Regular 3–5.5-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 dischargesa 8 times more frequent than CAE  |
| Abbreviations: ADHD, attention-deficit/hyperactivity disorder; CAE, childhood absence epilepsy; EEG, electroencephalogram; IGE, idiopathic generalized epilepsy; IPS, intermittent photic stimulation; JAE, juvenile absence epilepsy; OIRDA, occipital intermittent rhythmic delta activity. a Disorganized discharges are defined as either brief (less than 1 sec and and transient interruptions in ictal rhythm or waveforms of different frequency or morphology during the ictal rhythm.                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                         |||
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