NEUROLOGIC EVALUATION An MRI of the brain with and without gadolinium enhancement is recommended for visualization of the leptomeningeal angioma. Early in infancy the abnormal blood vessels on the surface of the brain may not be seen even by MRI, however. If the initial MRI is normal, it can be repeated at 2-3 years of life to exclude the presence of a leptomeningeal angioma. Brain calcification is most easily seen with a head CT but this is not usually an early finding. Other typical findings on brain imaging include brain atrophy (decreased brain mass) and enlarged deep draining veins on the effected side. Angiography is NOT routinely performed in the evaluation of children with SWS, but may be required in atypical cases to evaluate for other associated vascular malformations. PET and SPECT imaging is not routinely indicated except when surgery for seizures is being considered. If seizures occur, then an electroencephalogram (EEG) is obtained to evaluate brain function and seizure foci. Infants with SWS are at increased risk for developing weakness on one side of the body (hemiparesis), headaches, visual field cuts, epilepsy, mental retardation and developmental delays. The period of highest risk for the neurologic deterioration in SWS is in the first few years of life. Therefore, close neuro-developmental follow-up, in infancy and as issues arise, is essential to the care of children with SWS. At the JHHKKI Sturge-Weber syndrome center, patients with SWS are evaluated by specialists in Neurology and Epilepsy. As needed, patients with behavioral or psychiatric issues will be evaluated by a psychiatrist. Through the Kennedy Krieger Institute full rehabilitative medicine assessments are available, including occupational therapy, physical therapy, speech and language assessment and neuropsychological assessment.