Epileptic syndrome is defined as a cluster of seizure types, EEG patterns, imaging features, age of onset, triggers, and prognosis that tend to occur together. The presentation discusses epileptic syndrome in pediatrics. It notes that epilepsy requires at least two unprovoked seizures more than 24 hours apart or convincing EEG/clinical evidence of seizure predisposition. For some patients, no evidence-based treatments exist and seizures are managed with antiepileptic drugs. Approximately one-fourth of patients may have a family history or genetic factors like mutations in KCNQ2 linked to chromosome 15q14. Mild cognitive and behavioral impairments can also occur.

1. Epileptic syndrome
Presenter:
Dr Subodh Kumar Shah
2ND year Resident
Pediatric
Moderator:
 Associate Prof. Dr Sunil Kr Yadav
DM Neonatology
 Associate Prof. Vijay 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.

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

17. 1/4th of patients has family history , chromosome15q14 ,
pathogenic variant inKCNQ2
Mild cognitive dysfunction and behavioral changes

43. 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