Neurofibromatosis with extensive intracranial arterial occlusive disease.

Neurofibromatosis produces a broad spectrum of c linical manifestations as a result of widespread dysplasia of mesodermal and neuroectodermal tissues [1]. One of the more serious aspects of tile disease relates to the arterial involvement that may occur. Renal arterial disease with resultant hypertension has been particularly well documented . However, a number of reports have also documented the extensive intracerebral arterial abnormalities that may occur. Since the description in 1971 by Hilal et al. [2] of three ch ildren with neurofibromatosis and cerebral arterial disease, a number of other case reports have substantiated this aspect of the afflic tion. Most of the reports have concerned patients in childhood or adolescence. In 1976, Tomsick et al. [3] reviewed 1 2 previously reported cases and presented two more case reports with cerebral involvement. To our knowledge, there has been no report in which the arterial changes over a long period of time have been documented by serial angiography. We report an adult patient with neurofibromatosis and intracerebral arterial involvement who had two angiographic evaluations spaced 1 2 years apart.