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content:epileptic_encephalopathies [2020/02/16 22:08] – icna | content:epileptic_encephalopathies [2022/04/30 11:54] (current) – changed pubmed syntax administrator@icnapedia.org | ||
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+ | Epileptic encephalopathies are a heterogenous group of conditions where the epileptic activity itself contributes to severe cognitive and behavioral impairments above and beyond what might be expected from the underlying pathology alone (e.g., cortical malformation), | ||
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+ | The report of the International League Against Epilepsy (ILAE) Task Force on classification and terminology includes 8 syndromes under epileptic encephalopathies: | ||
+ | * [[Early Myoclonic Encephalopathy]] | ||
+ | * [[Early Infantile Epileptic Encephalopathy]] ([[Ohtahara Syndrome]]) | ||
+ | * [[Epilepsy of infancy with migrating focal seizures]] | ||
+ | * [[West Syndrome]] | ||
+ | * [[Dravet Syndrome]] | ||
+ | * [[Epilepsy with Myoclonic Astatic Seizures]] | ||
+ | * [[Lennox-Gastaut Syndrome]] | ||
+ | * [[Epilepsy with Continuous Spike-Waves during Slow Wave Sleep]] including [[Landau-Kleffner Syndrome]] and [[Atypical Benign Partial Epilepsy]]. | ||
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+ | One approach to epileptic encephalopathies is based on whether the presentation is with seizures as a prominent/ | ||
==== Presenting with seizures as prominent/ | ==== Presenting with seizures as prominent/ | ||
* Vitamin or enzymatic cofactor dependency | * Vitamin or enzymatic cofactor dependency | ||
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==== Presenting with seizures associated with a syndromic phenotype ==== | ==== Presenting with seizures associated with a syndromic phenotype ==== | ||
- | * **Chromosomopathies** | + | * **Chromosomal abnormalities** |
* [[1p36 monosomy]] | * [[1p36 monosomy]] | ||
* [[Wolf-Hirschhorn syndrome]] | * [[Wolf-Hirschhorn syndrome]] |