Moyamoya syndrome with unusual angiographic findings and protein C deficiency: Review of the literature

• Published in: Journal of the neurological sciences Vol. 159; no. 1; pp. 11 - 16
• Main Authors: Andeejani, A.M.I, Salih, M.A.M, Kolawole, T, Gader, AMA, Malabarey, T.O, Al Damegh, S, Al Boukai, A
• Format: Journal Article
• Language: English
• Published: Shannon Elsevier B.V 15-07-1998; Elsevier Science
• Subjects: Biological and medical sciences; Brain - pathology; Carotid angiography; Carotid Arteries - diagnostic imaging; Cerebral Angiography; Cerebrovascular Circulation; Child; Female; Humans; Magnetic Resonance Imaging; Medical sciences; Moyamoya disease; Moyamoya Disease - complications; Moyamoya Disease - diagnostic imaging; Moyamoya Disease - pathology; MRI in Moyamoya Disease; Neurology; Protein C Deficiency; Tropical medicine; Vascular diseases and vascular malformations of the nervous system; Vertebral angiography; Carotid angiography; Moyamoya disease; Vertebral angiography; Protein C deficiency; MRI in Moyamoya Disease; Cerebral angiography; External carotid; Nishimoto Kudo disease; Angiography; Stenosis; Cardiovascular disease; Hemopathy; Anticoagulant; Arterial disease; Vascular disease; Female; Localization; Child; Cerebrovascular disease; Human; Nervous system diseases; Radiodiagnosis; Deficiency; Clinical form; Protein C; Cerebral disorder; Genetic disease; Case study; Concomitant disease; Central nervous system disease; Vertebral artery; Coagulopathy; Bibliographic review
• ISSN: 0022-510X; 1878-5883
• DOI: 10.1016/S0022-510X(98)00133-6

We describe the findings in a six-year-old female with typical clinical symptoms of Moyamoya phenomena, but with an unusual angiographic appearance. The vertebral artery showed segmented occlusion in the upper cervical region, with reconstitution via collateral circulation through muscular branches. The carotid circulation showed involvement of the external carotid artery (ECA) branches and marked narrowing of the internal carotid arteries (ICA) bilaterally, suggesting that this disease is systemic in nature.