Abstracts

Rationale: ESES is an age dependent epileptic encephalopathy characterized by significant activation of epileptiform activity in the sleep EEG. Different treatment modalities are associated with variable success.High dose diazepam challenge test is used to identify suitable patients for nocturnal high dose diazepam maintenance therapy. Our objective is to quantify the effect of high dose diazepam on the sleep EEG of children with ESES and to identify the variables that correlate with the EEG response. Methods: Inclusion criteria: 1.Children aged 3-12 years diagnosed with ESES during May 2008- June 2010 2.ESES defined as spike-wave index (SWI) of >85% during sleep or >50% if the awake EEG showed a SWI of <20% Exclusion criteria: 1.Known 'myoclonic' epilepsy syndrome (Eg: Lennox-Gestaut syndrome) 2.On Carbamazepine/oxcarbazepine in the preceding 2 months. 1mg/kg of Diazepam (maximum 40 mg) was given in 2 equal half doses 2 hours apart in the late evening PO or PR. Sleep SWI was calculated after the second dose of diazepam. Good response was defined as decrease in sleep SWI by >50%. Results: Of the 15 children with ESES (Table 1), 11 had diazepam challenge test. Six (55%) had a good response. Sex or age of onset of ESES was not associated with EEG response. Delay in administering diazepam after onset of ESES was significantly greater in the non-responder group (Responders: Mean 0.42 yrs with SD 0.24; Non-responders: Mean 4.5 years with SD 3.3; unpaired t-test p=0.01). Duration of ESES significantly correlated with EEG improvement, greater improvement with shorter duration of ESES (duration of ESES Vs pre-post DZP SWI; Pearson correlation: r² = 0.297, one sided p= 0.04) Conclusions: In children with ESES, good EEG response following high dose diazepam challenge test is associated with shorter duration of ESES. This findings needs to be validated by a large prospective cohort.