Cortical calcification in Sturge-Weber Syndrome on MRI-SWI: relation to brain perfusion status and seizure severity

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).

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.

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.

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.