Abstracts

Rationale: We have previously reported on preliminary studies in the development of quantitative EEG methods to discriminate infants with facial port-wine stain (PWS) who will go on to develop clinical signs of Sturge-Weber syndrome (SWS) from the majority of infants with PWS who will be neurologically asymptomatic. In previous studies (American Epilepsy Society, San Diego, 2006), we developed a metric that discriminated between infants with Sturge-Weber syndrome and those without. This metric requires validation on a new cohort of infants.Methods: Five infants had a standard, 16-channel, 30-minute EEG that was quantified by Fast Fourier Transform. Laterality Scores (LS) were computed to assess degree of asymmetry of EEG power in delta (2.0-3.5 Hz), theta (4.0-7.5 Hz), alpha (8.0-12.5 Hz), beta (13.0-32.0 Hz) and total (2.0-32.0 Hz) bands, relative to the side with greatest PWS involvement, as described in Hatfield et al, Epilepsia, 2007. As in the previous cohort (American Epilepsy Society, San Diego, 2006), a qEEG was scored as positive when two or more frequency bands showed an LS of ≤ -0.2 either in all-channel analysis or in occipital-only analysis. All routine EEGs were also read by an experienced electroencephalographer who was blinded to the qEEG results.Results: Three infants with port-wine stain were diagnosed with SWS by imaging and clinical findings, and 2 were neurologically unaffected at more than 12 months of age. The mean age of the group with SWS at time of EEG was 6.7 months; the mean age of the group without SWS was 5.5 months (p = 0.67). Quantitative EEG correctly identified all subjects as affected or unaffected. Routine visual review of the EEG identified 80% of subjects correctly.Conclusions: The quantitative EEG metric previously developed was able to distinguish all infants with SWS from all those without in this small validation cohort. Further validation is proceeding as more infants are enrolled.