Sturge-Weber syndrome: cranial MR imaging with Gd-DTPA.

PURPOSE To study the role of Gd-DTPA in cranial MR of patients with Sturge-Weber syndrome.

METHODS AND MATERIALS Seven patients with Sturge-Weber syndrome, ages 3 months to 36 years, were evaluated with unenhanced and contrast-enhanced (Gd-DTPA) MR imaging. Correlation was made to cranial CT in five patients.

RESULTS MR findings included (in decreasing frequency): pial angiomatosis, cerebral atrophy, decrease in cortical veins, enlargement of deep veins, enlargement of choroid plexus, and parenchymal calcification. Contrast MR imaging was essential in diagnosis in four cases in which cranial CT, unenhanced MR, or angiography was normal or demonstrated nonspecific abnormalities. Pial angiomatosis with adjacent cerebral atrophy were the only consistent radiographic abnormalities.

CONCLUSION In all patients, contrast MR demonstrated the radiographic spectrum of central nervous system abnormalities in Sturge-Weber syndrome to a greater degree than unenhanced MR or CT and may represent the method of diagnosis in these patients. Demonstration of the pial angioma on contrast MR should be considered the most important criterion for the radiographic diagnosis of Sturge-Weber syndrome.