HIGH DOSE DIAZEPAM (HDD) IN CHILDHOOD-ONSET EPILEPTIC ENCEPHALOPATHIES WITH SLEEP ACTIVATED EEG (EESA_EEG)

Objectives: EESA_EEG represent a continuum of disorders characterized by cognitive and/or language impairment, with/without clinical seizures. Typical examples include the 'continuous spikes and waves during slow wave sleep' (CSWS) syndrome and the Landau–Kleffner syndrome (LKS). Our aim is to examine the efficacy and tolerability of HDD.

Methods: A medical record search identified 53 children who received 1mg/kg of HDD that was gradually tapered thereafter. EEGs done immediately and 1 month after treatment were reviewed. Parental reports at the one month follow-up visit were used to appreciate cognitive improvement.

Results: Mean patient age: 7.8 years; 33 males (62%), 20 females (38%). Underlying etiology: 21 idiopathic, 32 symptomatic. Immediate EEG response: 83% of patients; 75% had a response in one month; the idiopathic group responded better (p<.025). Pre-treatment EEG activation didn't predict the immediate EEG treatment response but less than 85% activation of non-REM sleep had a better EEG response one month after treatment (p<0.05). The immediate EEG response didn't predict the EEG response one month after treatment. 87% of the patients with LKS responded to HDDT. 47% of the overall patients had cognitive improvement compared to 89% of the LKS patients. There was a trend toward a cognitive improvement with EEG improvement (p<0.1).

Conclusion: HDDT may improve both the EEG and cognitive outcomes, which are closely associated, of patients with EESA_EEG and in particular LKS. 2. The symptomatic group had less dramatic EEG response to HDD. The immediate post-treatment EEG response did not predict the ultimate response. HDD was well tolerated.