Abstracts

EESA_EEG represent a continuum of disorders that are characterized by cognitive and/or language impairment, with or without clinical seizures or motor impairment. Typical examples include the [apos]continuous spikes and waves during slow wave sleep[apos] (CSWS) syndrome and the Landau[ndash]Kleffner syndrome (LKS). Treatment may be challenging, and high dose steroids may be frequently needed. We have introduced a high[ndash]dose diazepam treatment (HDDT) protocol, which has less associated morbidity as our routine second-line treatment for children with EESA_EEG. The aim of this retrospective analysis is to examine the efficacy and tolerability of this treatment. A computer search identified 53 children with EESA_EEG who received 1 mg/kg of diazepam with gradual tapering thereafter. EEGs immediately and 1 month after treatment were reviewed. Parental reports at the one month follow-up visit were used to appreciate cognitive improvement. The mean patient age was 7.8 years. There were 37 males (62%) and 16 females (38%). The underlying etiology was idiopathic in 21 and symptomatic in 32. The most common causes in the symptomatic group were: developmental delay, perinatal depression, intraventicular hemorrhage/hydrocephalus, cortical malformations; 78% were diagnosed with developmental delay before age one. The EEG responded immediately in 83% of patients; 75% had a response in one month, with a better response in the idiopathic group (p[lt].025). The degree of pre-treatment EEG epileptiform activity didn[apos]t predict the immediate EEG treatment response. However, patients with pre-treatment discharges involving less than 85% of non-REM sleep had a better EEG response in one month after treatment (p[lt] .05). Additionally, the immediate EEG response didn[apos]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[lt] .1). Adverse effects were reported in 47% of patients, were primarily neurologic and gradually subsided. 1. HDDT may improve both the EEG and cognitive outcomes, which are closely associated, of patients with EESA_EEG; this is particularly true in patients with LKS.
2. The symptomatic group had an association with developmental delay and had less dramatic EEG response to HDDT.
3. Patients even with less than 25% sleep activation responded to HDDT.
4. The immediate post-treatment EEG response did not predict the ultimate HDDT response.
5. Most children developed a gradual tolerance to HDDT.