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content:self_limited_epilepsy_with_centrotemporal_spikes_selects [2024/03/23 06:45] – biju.hameed@gmail.com | content:self_limited_epilepsy_with_centrotemporal_spikes_selects [2024/03/23 19:44] (current) – [Treatment] biju.hameed@gmail.com | ||
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====== Self-Limited Epilepsy with Centrotemporal Spikes (SeLECTS) ====== | ====== Self-Limited Epilepsy with Centrotemporal Spikes (SeLECTS) ====== | ||
- | (formerly called [[content: | + | |
+ | (formerly called [[content: | ||
* Self-Limited Epilepsy with Centrotemporal Spikes (SeLECTS), is the most common focal epilepsy syndrome of childhood, accounting for 15–20% of all childhood epilepsies. | * Self-Limited Epilepsy with Centrotemporal Spikes (SeLECTS), is the most common focal epilepsy syndrome of childhood, accounting for 15–20% of all childhood epilepsies. | ||
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* Seizures are overall relatively infrequent, with 60–70% of patients experiencing two to ten seizures lifetime and 10–20% only one | * Seizures are overall relatively infrequent, with 60–70% of patients experiencing two to ten seizures lifetime and 10–20% only one | ||
* Rarely Children with SeLECTS evolve to Developmental and/or [[Epileptic Encephalopathy with spike-wave activation in sleep]] (D/ | * Rarely Children with SeLECTS evolve to Developmental and/or [[Epileptic Encephalopathy with spike-wave activation in sleep]] (D/ | ||
- | * D/EE-SWAS incorporates the several syndromes previously named [[Landau-Kleffner syndrome]], [[Epileptic | + | * D/EE-SWAS incorporates the several syndromes previously named [[Landau-Kleffner syndrome]], [[content:Epileptic |
==== Seizure Semiology ==== | ==== Seizure Semiology ==== | ||
+ | |||
* Seizures are brief, typically less than 2-3 minutes | * Seizures are brief, typically less than 2-3 minutes | ||
* usually few in number (most children have less than 10 lifetime seizures) and may occur sporadically, | * usually few in number (most children have less than 10 lifetime seizures) and may occur sporadically, | ||
+ | |||
the next seizure | the next seizure | ||
+ | |||
* Seizures occur during sleep in 80-90% of patients and only while awake in fewer than 20% of children | * Seizures occur during sleep in 80-90% of patients and only while awake in fewer than 20% of children | ||
* Seizures are usually stereotyped, | * Seizures are usually stereotyped, | ||
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* sialorrhea, a characteristic ictal symptom (unclear whether it is due to increased salivation, swallowing disturbance, | * sialorrhea, a characteristic ictal symptom (unclear whether it is due to increased salivation, swallowing disturbance, | ||
* seizures may evolve to a focal to bilateral tonic-clonic seizure[(note1> | * seizures may evolve to a focal to bilateral tonic-clonic seizure[(note1> | ||
- | * cognitive (e.g. gustatory hallucinations), | + | * cognitive (e.g. gustatory hallucinations), |
- | features, especially ictal vomiting is present then [[Self-Limited Epilepsy with Autonomic Seizures]] (SeLEAS) (formerly called Panayiotopoulos syndrome or early-onset benign occipital epilepsy) should be considered)] | + | |
==== EEG ==== | ==== EEG ==== | ||
+ | |||
* normal background activity | * normal background activity | ||
* High amplitude centrotemporal sharp-and-slow wave complexes that activate in drowsiness and sleep are mandatory for diagnosis. | * High amplitude centrotemporal sharp-and-slow wave complexes that activate in drowsiness and sleep are mandatory for diagnosis. | ||
- | * Triphasic, high-voltage (100-microvolts to 300microvolts) sharp waves (initial low-amplitude positivity, then high amplitude negativity followed again by low amplitude positivity), | + | * Triphasic, high-voltage (100-microvolts to 300microvolts) sharp waves (initial low-amplitude positivity, then high amplitude negativity followed again by low amplitude positivity), |
* The discharges may be isolated or occur in trains of doublets and triplets, and focal, rhythmic, slow activity is occasionally observed in the same region as the spikes. | * The discharges may be isolated or occur in trains of doublets and triplets, and focal, rhythmic, slow activity is occasionally observed in the same region as the spikes. | ||
- | * The discharges may be unilateral or bilateral and independent | + | * The discharges may be unilateral or bilateral and independent |
* There may be discharges seen outside the centrotemporal region (midline, parietal, | * There may be discharges seen outside the centrotemporal region (midline, parietal, | ||
* A marked increase in the frequency of epileptiform activity in drowsiness and sleep always occurs. | * A marked increase in the frequency of epileptiform activity in drowsiness and sleep always occurs. | ||
- | * The EEG pattern may also change such that sharp- or spike-and-slow waves have a broader field and become bilaterally synchronous | + | * The EEG pattern may also change such that sharp- or spike-and-slow waves have a broader field and become bilaterally synchronous |
- | * In 10-20% of children, centrotemporal sharp- or spike-and-slow wave may be activated by sensory stimulation of the fingers or toes 40 | + | * In 10-20% of children, centrotemporal sharp- or spike-and-slow wave may be activated by sensory stimulation of the fingers or toes |
- | * Seizures are typically infrequent - it is rare to obtain an ictal recording and there are few published reports in the literatureFIXME. | + | * Seizures may be accompanied by a brief decrease in amplitude of the background EEG, followed by diffuse sharp wave discharges of increasing amplitude, predominantly in one centrotemporal |
- | * Seizures may be accompanied by a brief decrease in amplitude of the background EEG, followed by diffuse sharp wave discharges of increasing amplitude, predominantly in one centrotemporal | + | * With focal to bilateral tonic-clonic seizures, ictal rhythms may become bilaterally synchronous (as opposed to generalized) sharp- or spike-and slow-wave |
- | * With focal to bilateral tonic-clonic seizures, ictal rhythms may become bilaterally synchronous (as opposed to generalized) sharp- or spike-andslow-wave activity 42–44 | + | |
- | Note | + | |
- | + | ||
- | + | ||
- | + | ||
- | If a continuous | + | |
==== Genetics ==== | ==== Genetics ==== | ||
+ | |||
* Centrotemporal spikes can be a trait that is passed down through siblings in a way that depends on their age and is autosomally dominant, even if they don't have seizures. | * Centrotemporal spikes can be a trait that is passed down through siblings in a way that depends on their age and is autosomally dominant, even if they don't have seizures. | ||
* Mutations in GRIN2A have been implicated in various types of Epilepsy-Aphasia-Spectrum (EAS) disordersS[(: | * Mutations in GRIN2A have been implicated in various types of Epilepsy-Aphasia-Spectrum (EAS) disordersS[(: | ||
+ | |||
+ | ==== Practice Points ==== | ||
+ | |||
+ | * If a continuous spike-and-slow-wave pattern is present in sleep, the child should be evaluated for progressive language or cognitive impairment or regression. This EEG pattern should only lead to a diagnosis of D/EE-SWAS if developmental plateauing or regression is also present[(: | ||
+ | * Patients with SeLECTS may show " | ||
+ | |||
+ | ==== Treatment ==== | ||
+ | |||
+ | * As the seizures associated with SeLECTS often stop around the age of puberty, it is not clear whether it is necessary to prescribe ASMs to all children who present with this condition. | ||
+ | * When considering the use of antiseizure medications for SeLECTS, it is important to have a thorough discussion with the individual, their family, and caregivers (if applicable). This discussion should focus on developing a personalized medication strategy based on the specific epilepsy syndrome, treatment objectives, and the preferences of the individual and their family or caregivers. | ||
+ | * lamotrigine and levetiracetam should be considered as first-line treatment[(: | ||
+ | * As second-line treatment, carbamazepine, | ||
+ | * school performance is a good indicator of cognition since it measures processing and retention.If any deterioration is noted, an EEG should be performed to exclude [[content: | ||
+ | |||
==== References ==== | ==== References ==== | ||