Epilet sydr.pptx

EPILEPTIC SYNDROME IN CHILDREN

1. Epileptic syndrome Presenter: Dr Subodh Kumar Shah 2 ND year Resident Pediatric Moderator: Associate Prof. Dr Sunil K r Yadav DM Neonatology Associate Prof. V ijay kumar shah

2. Introduction : Epilepsy is a disorder of the brain characterized by an enduring predisposition to generate seizures and by the neurologic, cognitive , psychological , and social consequences of this condition . The clinical diagnosis of epilepsy usually requires the occurrence of at least one unprovoked epileptic seizure with either a second such seizure or enough EEG and clinical information to convincingly demonstrate an enduring predisposition to develop recurrence . For epidemiologic and commonly , for clinical purposes, epilepsy is considered present when two or more unprovoked seizures occur in a time frame of longer than 24 hrs.

3. Definition of an epilepsy syndrome an epilepsy syndrome is a cluster of features incorporating typical seizure types, EEG, and imaging features that tend to occur together, often with age-dependent features such as age at onset and remission (where applicable), seizure triggers, diurnal variation, sometimes prognosis, and distinctive comorbidities such as intellectual and psychiatric dysfunction. Scheffer IE, Berkovic S, Capovilla G, Connolly MB, French J, Guilhoto L, et al. ILAE classification of the epilepsies: position paper of the ILAE Commission for Classification and Terminology. Epilepsia . 2017;58:512–21.

4. No evidence based treatment, seizures are treated with AED.

5. 1/4 th of patients has family history , chromosome15q14 , pathogenic variant inKCNQ2 Mild cognitive dysfunction and behavioral changes

6. Reference : ILAE classification and definition of epilepsy syndromes with onset in neonates and infants, DOI: 10.1111/epi.17239, Epileptic Syndromes in Childhood. A Practical Approach: Paulo Breno Noronha Liberalesso1, DOI: 10.25060/residpediatr-2018.v8s1-10 Nelson