MOYAMOYA SYNDROME WITH UNUSUAL ANGIOGRAPHIC FINDINGS AND PROTEIN-C DEFICIENCY - REVIEW OF THE LITERATURE

We describe the findings in a six-year-old female with typical clinica l symptoms of Moyamoya phenomena, but with an unusual angiographic app earance. The vertebral artery showed segmented occlusion in the upper cervical region, with reconstitution via collateral circulation throug h muscular branches. The carotid circulation showed involvement of the external carotid artery (ECA) branches and marked narrowing of the in ternal carotid arteries (ICA) bilaterally, suggesting that this diseas e is systemic in nature. (C) 1998 Elsevier Science B.V. All rights res erved.