abstract
- PURPOSE: To determine the relationship between calcified cortex and perfusion status of white matter and seizure severity in patients with Sturge-Weber Syndrome (SWS), a sporadic neurocutaneous disorder characterized by a leptomeningeal angioma, progressive brain ischemia, and a high incidence of seizures using susceptibility weighted imaging (SWI) and dynamic susceptibility contrast-enhanced perfusion weighted imaging (DSC-PWI). MATERIALS AND METHODS: Fifteen children (ages: 0.9-10 years) with unilateral SWS prospectively underwent magnetic resonance imaging (MRI). The degree of cortical calcification was assessed using SWI while perfusion status was quantified using DSC-PWI images (asymmetries of various perfusion parameters). Comparisons between calcification, perfusion status, and seizure variables were performed. RESULTS: Patients with severely calcified cortex demonstrated significantly lower perfusion in the ipsilateral white matter (mean asymmetry: -0.52 ± 0.22) as compared to patients with only mildly calcified cortex or no calcification (mean asymmetry: 0.08 ± 0.25). Patients with severely calcified cortex also suffered from a higher seizure burden (a composite measure of seizure frequency and epilepsy duration; P = 0.01) and a trend for earlier seizure onset and longer epilepsy duration. CONCLUSION: Severe calcification in the affected hemisphere is related to severely decreased perfusion in underlying white matter and is associated with more severe epilepsy in SWS patients.