Isolated frontal lobe calcification in Sturge-Weber syndrome.

The Sturge-Weber syndrome is a phacomatosis characterized by encephalofacial angiomatosis, seizure disorder, and cortical calcification. There are several cases of atypical or incomplete forms of Sturge-Weber syndrome in which the patient presents with a seizure disorder and cortical calcification. The cortical calcifications have been described in the occipital, parietal, and temporal areas [1-4]. Our case is unique in that the calcification was isolated to the frontal lobe. Also, the patient did not have a facial angioma. She did present with a seizure disorder. An angiogram revealed abnormalities of the superficial cortical veins, and an excisional biopsy of the frontal lobe lesion revealed changes characteristic of Sturge-Weber syndrome.