This study outlines the multisystem manifestations of TSC, observed through a large pediatric referral center. Epilepsy and neuropsychiatric disorders are the major source of morbidity in this age group and provide many challenges to the treating clinician. Because a subset of the study population is still quite young, the prevalence of neuropsychiatric disorders is likely underestimated.

Thirteen term newborns with CHD underwent pre-operative MR imaging and continuous EEG recordings (cEEG). The proportion of cEEG with discontinuous vs. continuous background activity was quantified by visual analysis. During continuous epochs, we differentiated between two states: wakefulness/active sleep vs. quiet/transitional sleep, and applied algorithms to measure spectral power and EEG complexity. Results: Three patients had multifocal WMI which was associated with greater EEG background discontinuity (P<0.05). Moreover, lower white matter diffusivity was associated with higher power of fast activity (P<0.05 for wakefulness/active sleep EEG pattern), while higher white matter FA showed a trend toward being associated with increased EEG complexity (P<0.1 for quiet/transitional sleep pattern). Conclusions: In this series of term neonates with CHD, structural and microstructural white matter abnormalities are associated with impaired maturation of brain function.

Thirteen term newborns with CHD underwent pre-operative MR imaging and continuous EEG recordings (cEEG). The proportion of cEEG with discontinuous vs. continuous background activity was quantified by visual analysis. During continuous epochs, we differentiated between two states: wakefulness/active sleep vs. quiet/transitional sleep, and applied algorithms to measure spectral power and EEG complexity. Results: Three patients had multifocal WMI which was associated with greater EEG background discontinuity (P<0.05). Moreover, lower white matter diffusivity was associated with higher power of fast activity (P<0.05 for wakefulness/active sleep EEG pattern), while higher white matter FA showed a trend toward being associated with increased EEG complexity (P<0.1 for quiet/transitional sleep pattern). Conclusions: In this series of term neonates with CHD, structural and microstructural white matter abnormalities are associated with impaired maturation of brain function.