content:epileptic_encephalopathies

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content:epileptic_encephalopathies [2020/02/16 22:08] icnacontent:epileptic_encephalopathies [2022/04/30 11:54] (current) – changed pubmed syntax administrator@icnapedia.org
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-====== Approach to epileptic encephalopathies ======+====== Epileptic encephalopathies ====== 
 +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), and that these can worsen over time”[(:cite:20196795>{{pmid>long:20196795}})]. According to the ILAE commission (2017)[(:cite:28276062>{{pmid>long:28276062}})] “in an epileptic encephalopathy, the abundant epileptiform activity interferes with development resulting in cognitive slowing and often regression, and sometimes is associated with psychiatric and behavioural consequences” and that the encephalopathic impact of the epileptiform activity could cause regression in patients with both normal development and pre-existing developmental delay, who then show developmental plateauing or regression. The disorders are usually refractory to standard antiepileptic drugs (AEDs). 
 + 
 +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]]. 
 + 
 +One approach to epileptic encephalopathies is based on whether the presentation is with seizures as a prominent/unique symptom or where they are associated with movement disorders, dysmorphic features or multi-organ involvement. 
 ==== Presenting with seizures as prominent/unique symptom ==== ==== Presenting with seizures as prominent/unique symptom ====
   * 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]]
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