Teaching NeuroImages: Neurovascular features of suspected antenatal-onset Sturge-Weber syndrome without skin involvement

A female infant was born at 35-week gestation by emergency C-section because of abnormal cardiotocography. No birthmarks were evident. Cord pH was 6.87; therefore, an amplitude-integrated electroencephalography monitoring was performed soon after birth, showing left-sided electrical seizures. Cranial ultrasound revealed left hemisphere atrophy, with subcortical hyperechoic areas in the frontoparietal lobe. Frontoparietal NIRS monitoring showed interhemispheric asymmetry of cerebral oxygenation and total hemoglobin concentration (figure 1) that serves as a proxy for cerebral blood volume. Brain CT and MRI (figure 2) revealed characteristic features consistent with Sturge-Weber syndrome. Based on the evidence of polymicrogyria, the insult was dated back to the second trimester of pregnancy. The ophthalmologic examination results were unremarkable. The infant was started on phenobarbital, with seizure remission.