Abstracts

Ten to 35 percent of infants with facial port-wine stain (PWS) develop radiographic leptomeningeal angioma and clinical signs of Sturge-Weber syndrome (SWS), usually within the first year. Radiographic signs are absent at birth in the vast majority of those who go on to be affected, making the diagnosis in infancy unclear. Screening with serial neuroimaging carries a sedation risk. We hypothesized that quantitative EEG (qEEG) can be used to help determine which infants are most likely to develop SWS and to benefit from imaging., Ten 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 previously described (Hatfield et al, Epilepsia, in press). A qEEG was positive when two or more frequency bands showed an LS of [le] -0.2 either in all-channel analysis or in occipital-only analysis. All routine EEGs were read by an experienced electroencephalographer who was masked from the qEEG results., Four infants with port-wine stain were eventually diagnosed with SWS by imaging and clinical findings, and 6 were not at age 12 months or older. The mean subject age at EEG was 5.7 months (affected) and 4.8 months (unaffected) (p = 0.66). Quantitative EEG correctly identified all subjects as affected or unaffected. In 3 affected infants, the diagnosis was known by imaging and clinical signs prior to the confirmatory qEEG; however, in one infant, qEEG correctly identified the infant as having SWS before clinical signs developed or changes were visible on clinical review of the EEG. Routine visual review of the EEG only identified 50% of subjects correctly., In this small series of infants with facial PWS, quantitative EEG accurately identified infants with SWS, whereas routine EEG did not. Given that the majority of infants with PWS will not go on to have SWS, and early MRI is not always diagnostic, quantitative EEG may be a safe, helpful technique., (Supported by supplementary funds on NIH Grant R01NS40596, Hunter[apos]s Dream for a Cure Foundation and the Sturge-Weber Foundation.)