Sturge-Weber-Dimitri disease? In association with an astrocytoma

A patient with parieto-occipital cortical calcification characteristic of Sturge-Weber-Dimitri disease (SWDD) is of interest in that she lacked the facial portwine lesion and almost all other features of the disease. She subsequently developed an astrocytoma in the underlying white matter. Although the absence of the facial lesion in SWDD has previously been described, there has been no report of a glioma developing in such a patient. The association of SWDD and astrocytoma in this case most likely has been fortuitous.