PROGRESSIVE CEREBRAL OCCLUSIVE DISEASE AFTER RADIATION-THERAPY

Background A case of progressive irradiation-induced cerebral vasculop athy with abnormal netlike vessels and transdural anastomoses (moyamoy a syndrome) is presented. Radiological findings in an additional 40 ca ses reported in the literature are analyzed, and their clinical releva nce is discussed. Case Description A 19-year-old woman presented with recurrent ischemic brain lesions after radiation therapy for treatment of a craniopharyngioma during childhood. Cerebral angiography 6 and 1 2 years after completion of radiation therapy revealed progressive cer ebral arterial occlusive disease involving the internal carotid artery on either side of the circle of Willis, with abnormal netlike vessels and transdural anastomoses (moyamoya syndrome). Conclusions Extensive similarities between irradiation-induced cerebral vasculopathy and pr imary moyamoya syndrome (Nishimoto's disease) support the notion that both disorders share common pathophysiological mechanisms. The occurre nce of moyamoya-like vascular changes may not depend on specific trigg er mechanisms but may rather represent a nonspecific response of the d eveloping vascular system to a number of various noxious events.