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| content:childhood_absence_epilepsy [2024/03/09 18:52] – [Genetics] 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 CCNA1H}} | {{tag>childhood_absence_epilepsy CCNA1H}} |
| Childhood absence epilepsy (CAE) is an age-dependent, idiopathic form of generalised epilepsy characterised by multiple absence seizures per day, as well as bilateral, symmetrical, and synchronous discharges of 3-Hz generalised spike and waves (GSW) in the electroencephalogram. 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, lasting seconds, with immediate return to the baseline level of awareness and activity. Children with CAE develop normally, although attentional deficiencies or other subtle behavioural or cognitive abnormalities may be present at onset. | * Childhood absence epilepsy (CAE) is an age-dependent, idiopathic form of generalised epilepsy (IGE) characterised by multiple absence seizures per day, as well as bilateral, symmetrical, and synchronous discharges of 3-Hz generalised spike and waves (GSW) in the electroencephalogram. |
| | * 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)[(:cite:pmid28276062>{{pmid>long:28276062}})]. |
| | * 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, lasting seconds, with immediate return to the baseline level of awareness and activity. Children with CAE develop normally, although attentional deficiencies or other subtle behavioural or cognitive abnormalities may be present at onset. |
| ===== Investigations ===== | ===== Investigations ===== |
| <imgcaption absenceeeg|A typical absence seizure on electroencephalogram, characterized by 3 Hz generalized spike wave discharges, with abrupt onset and offset, lasting several second>{{ :content:absences.png?400x106}}</imgcaption> | <imgcaption absenceeeg|A typical absence seizure on electroencephalogram, characterized by 3 Hz generalized spike wave discharges, with abrupt onset and offset, lasting several second>{{ :content:absences.png?400x106}}</imgcaption> |
| | 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. ||| | | 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. ||| |
| </tabcaption> | </tabcaption> |
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| | While reporting an EEG the following terms are suggested, whenever appropriate: spike-and-slow-wave complex, 3 c/s spike-and-slow-wave complex, sharp-and-slow wave complex. Use of the term "absences" is discouraged when describing EEG patterns. |
| ===== 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. |
