The clinical and electroencephalographic spectrum of ESES (Encephalopathy with status epilepticus in sleep) in Indian children (P2.300)

Objective: Retrospective analysis of electroclinical spectrum of “Encephalopathy with status epilepticus in sleep” (ESES) in children Background: Hospital records of children (treated from July,2015 to December,2016) were analyzed. Clinically, patients were categorized as: ESES with seizures and ESES with isolated neuropsychological regression. Treatment details, SQ/IQ and behavioral problems (as per VSMS/MISIC and CBCL) were documented. Electroencephalogram were reevaluated for: spike-wave index (SWI- from 1st 15 minutes of sleep); background (organization, maturity and sleep markers), localization of discharges and response to treatment. Last follow-up record and EEG was used for outcome assessment. Design/Methods: Hospital records of children (treated from July,2015 to December,2016) were analyzed. Clinically, patients were categorized as: ESES with seizures and ESES with isolated neuropsychological regression. Treatment details, SQ/IQ and behavioral problems (as per VSMS/MISIC and CBCL) were documented. Electroencephalogram were reevaluated for: spike-wave index (SWI- from 1st 15 minutes of sleep); background (organization, maturity and sleep markers), localization of discharges and response to treatment. Last follow-up record and EEG was used for outcome assessment. Results: Eighteen children with ESES with minimum follow up of 3months {12 boys; median age at diagnosis: 7.2years (range-2.5years to 13 years); pre-existent developmental delay (50%)} were included. Key clinical features were sociocognitive regression {77.7%; median age: 5.5years (range: 1.5–11years)}, seizures {77.7%; median age at onset: 3.7 years (range: 3months–7years); prominent semiology: focal(6), generalized(5), myoclonic(3), multiple semiologies (4)} and behavioral problems {88.8%; inattention(15/16), hyperactivity(12/16), PDD(14/16), aggression(11/16)}. Etiology were structural(22.2%) and unknown(77%). All children received methylprednisolone pulse followed by oral steroids. ESES with seizures (77.7%): All received antiepileptics. EEG characteristics were - Background: disorganized-7/14, partly organized-7/14; Mean SWI-69.8±21.8; localization: anterior-9/14 and posterior-5/14, secondary synchronization-10/14. Post-immunotherapy EEG responses were complete resolution-4; partial response (u003e25% reduction)-5; no response-5. Mean IQ/SQ improved from 52.65 to 56.2 (p Conclusions: Electroclinical spectrum of ESES is ever expanding. A subset of children may present with neuroregression and behavioral problems without seizures. EEG in these children shows large amplitude dysmature background with SWI=30–50. Suspicion and treatment in this subgroup may be rewarding wih better outcomes. Disclosure: Dr. Madaan has nothing to disclose. Dr. Jauhari has nothing to disclose. Dr. Chakrabarty has nothing to disclose. Dr. Sondhi has nothing to disclose. Dr. Sharma has nothing to disclose. Dr. Gulati has nothing to disclose.