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content:childhood_absence_epilepsy [2024/03/09 12:20] – [Childhood Absence Epilepsy] biju.hameed@gmail.com | content:childhood_absence_epilepsy [2024/03/19 17:06] (current) – [Differentiating between CAE and JAE] biju.hameed@gmail.com | ||
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====== Childhood Absence Epilepsy ====== | ====== Childhood Absence Epilepsy ====== | ||
- | + | {{tag> | |
- | Childhood absence epilepsy (CAE) is an age-dependent, | + | |
+ | * The 2017 International League Against Epilepsy (ILAE)classification suggested that the term IGE could be reserved for the four syndromes including childhood absence epilepsy (CAE), juvenile absence epilepsy (JAE), [[juvenile myoclonic epilepsy]] (JME), and epilepsy with generalized tonic–clonic seizures alone (GTCA)[(: | ||
+ | * CAE makes for 2% to 10% of all paediatric epilepsies and 8-15% of school-aged childhood epilepsies. Seizures occur many times daily and consist of brief staring spells, sometimes with rhythmic eye blinking or motor automatisms, | ||
===== Investigations ===== | ===== Investigations ===== | ||
< | < | ||
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* Because VPA slows down the metabolism of LTG by blocking liver enzymes, titration of LTG starts at an even lower dose, moves more slowly, and hits a lower target in a person who is also taking VPA than in a person who is not taking any other medication. | * Because VPA slows down the metabolism of LTG by blocking liver enzymes, titration of LTG starts at an even lower dose, moves more slowly, and hits a lower target in a person who is also taking VPA than in a person who is not taking any other medication. | ||
* There are important cognitive, behavioural, | * There are important cognitive, behavioural, | ||
- | ===== Treatment resistant CAE ===== | ||
- | * If first- and second-line drugs don't work, clobazam might be worth a try. However, there isn't much literature on its use in treatment-resistant CAE | ||
- | * The ketogenic diet has also been used successfully in children with treatment-resistant CAE[(: | ||
- | * There is mixed evidence about how well levetiracetam works for children absence epilepsy. Some reports say it works pretty well[(: | ||
===== Differentiating between CAE and JAE ===== | ===== Differentiating between CAE and JAE ===== | ||
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* Valproate can be considered as the drug of choice in men and lamotrigine as the first drug of choice in women with JAE. See <tabref absenceseizuremedication> | * Valproate can be considered as the drug of choice in men and lamotrigine as the first drug of choice in women with JAE. See <tabref absenceseizuremedication> | ||
* CAE has a good prognosis (most patients will become seizure-free off ASMs[(: | * CAE has a good prognosis (most patients will become seizure-free off ASMs[(: | ||
+ | < | ||
+ | ^ Feature | ||
+ | | Age at onset | ||
+ | | Usual | 4–10 years | ||
+ | | Range | 2–13; caution if diagnosing at <4yrs of age | 8–20 years; exceptional cases may present in adulthood | ||
+ | | Development | ||
+ | | Absences | ||
+ | | Frequency | ||
+ | | Duration | ||
+ | | Impaired awareness | ||
+ | | Other seizure types | | ||
+ | | Febrile | ||
+ | | Generalized tonic-clonic seizures | ||
+ | | Myoclonic | ||
+ | | EEG background | ||
+ | | Interictal epileptiform\\ discharge | ||
+ | | Awake | 2.5–4-Hz generalized spike-wave | ||
+ | | Asleep | ||
+ | | Irregular generalized spike-wave | ||
+ | | Photoparoxysmal response | ||
+ | | Hyperventilation induction | ||
+ | | 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 | ||
+ | | Abbreviations: | ||
+ | </ | ||
+ | While reporting an EEG the following terms are suggested, whenever appropriate: | ||
===== Pathophysiology ===== | ===== Pathophysiology ===== | ||
* Absence seizures result from disruptions in thalamocortical rhythms via T-type calcium channel dysfunction. | * Absence seizures result from disruptions in thalamocortical rhythms via T-type calcium channel dysfunction. | ||
+ | see also [[absence seizures]] | ||
+ | ===== Genetics ===== | ||
+ | |||
+ | * There are few genes that confer monogenic risk for CAE, found through family studies or large cohort studies (e.g., GABRG2, GABRA1, SLC2A1[(: | ||
* The [[https:// | * The [[https:// | ||
+ | * Several recurring Copy Number Variations (CNVs), such as 15q11.2, 15q13.3, and 16p13.11 microdeletion[(: | ||
+ | |||
+ | ===== Treatment resistant CAE ===== | ||
+ | * If first- and second-line drugs don't work, clobazam might be worth a try. However, there isn't much literature on its use in treatment-resistant CAE | ||
+ | * The ketogenic diet has also been used successfully in children with treatment-resistant CAE[(: | ||
+ | * There is mixed evidence about how well levetiracetam works for children absence epilepsy. Some reports say it works pretty well[(: | ||
+ | |||
===== References ===== | ===== References ===== | ||