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”[1]. According to the ILAE commission (2017)[2] “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:

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.

1. a Berg AT, Berkovic SF, Brodie MJ, Buchhalter J, Cross JH, van Emde Boas W, Engel J, French J, Glauser TA, Mathern GW, Moshé SL, Nordli D, Plouin P, Scheffer IE. Revised terminology and concepts for organization of seizures and epilepsies: report of the ILAE Commission on Classification and Terminology, 2005-2009. Epilepsia. 2010 Apr;51(4):676-85. doi: 10.1111/j.1528-1167.2010.02522.x. Epub 2010 Feb 26.
[PMID: 20196795] [DOI: 10.1111/j.1528-1167.2010.02522.x]
2. a Scheffer IE, Berkovic S, Capovilla G, Connolly MB, French J, Guilhoto L, Hirsch E, Jain S, Mathern GW, Moshé SL, Nordli DR, Perucca E, Tomson T, Wiebe S, Zhang Y, Zuberi SM. ILAE classification of the epilepsies: Position paper of the ILAE Commission for Classification and Terminology. Epilepsia. 2017 Apr;58(4):512-521. doi: 10.1111/epi.13709. Epub 2017 Mar 8.
[PMID: 28276062] [PMCID: 5386840] [DOI: 10.1111/epi.13709]
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