Biomaterial Database

Moyamoya disease in an adult with Down syndrome: comparison of magnetic resonance angiography and conventional angiography. 1996

M Park, and F A Raila, and W F Russell

Department of Radiology, University of Mississippi Medical Center, Jackson 39216, USA.

Moyamoya disease, or idiopathic progressive arteriopathy, is a vascular occlusive disease that results in stenosis of the internal carotid arteries, stimulating the development of collaterals and telangiectatic vasculature from basilar and leptomeningeal arteries. Within the past several years, several reports have suggested that children with Down syndrome can develop moyamoya vasculopathy. We present the first reported magnetic resonance angiographic diagnosis of moyamoya disease in an adult with Down syndrome. This was also confirmed by conventional cerebral angiography.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D009072	Moyamoya Disease	A noninflammatory, progressive occlusion of the intracranial CAROTID ARTERIES and the formation of netlike collateral arteries arising from the CIRCLE OF WILLIS. Cerebral angiogram shows the puff-of-smoke (moyamoya) collaterals at the base of the brain. It is characterized by endothelial HYPERPLASIA and FIBROSIS with thickening of arterial walls. This disease primarily affects children but can also occur in adults.	Cerebrovascular Moyamoya Disease,Progressive Intracranial Occlusive Arteropathy (Moyamoya),Moya-Moya Disease,Moyamoya Disease, Classic,Moyamoya Disease, Primary,Moyamoya Disease, Secondary,Moyamoya Syndrome,Classic Moyamoya Disease,Disease, Classic Moyamoya,Disease, Moya-Moya,Disease, Primary Moyamoya,Moya Moya Disease,Moyamoya Diseases, Primary,Primary Moyamoya Disease,Primary Moyamoya Diseases,Secondary Moyamoya Disease
D002533	Cerebral Angiography	Radiography of the vascular system of the brain after injection of a contrast medium.	Angiography, Cerebral,Angiographies, Cerebral,Cerebral Angiographies
D004314	Down Syndrome	A chromosome disorder associated either with an extra CHROMOSOME 21 or an effective TRISOMY for chromosome 21. Clinical manifestations include HYPOTONIA, short stature, BRACHYCEPHALY, upslanting palpebral fissures, epicanthus, Brushfield spots on the iris, protruding tongue, small ears, short, broad hands, fifth finger clinodactyly, single transverse palmar crease, and moderate to severe INTELLECTUAL DISABILITY. Cardiac and gastrointestinal malformations, a marked increase in the incidence of LEUKEMIA, and the early onset of ALZHEIMER DISEASE are also associated with this condition. Pathologic features include the development of NEUROFIBRILLARY TANGLES in neurons and the deposition of AMYLOID BETA-PROTEIN, similar to the pathology of ALZHEIMER DISEASE. (Menkes, Textbook of Child Neurology, 5th ed, p213)	Mongolism,Trisomy 21,47,XX,+21,47,XY,+21,Down Syndrome, Partial Trisomy 21,Down's Syndrome,Partial Trisomy 21 Down Syndrome,Trisomy 21, Meiotic Nondisjunction,Trisomy 21, Mitotic Nondisjunction,Trisomy G,Downs Syndrome,Syndrome, Down,Syndrome, Down's
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults
D018810	Magnetic Resonance Angiography	Non-invasive method of vascular imaging and determination of internal anatomy without injection of contrast media or radiation exposure. The technique is used especially in CEREBRAL ANGIOGRAPHY as well as for studies of other vascular structures.	Angiography, Magnetic Resonance,MRI Angiography,Perfusion Magnetic Resonance Imaging,Perfusion Weighted MRI,Angiographies, MRI,Angiographies, Magnetic Resonance,Angiography, MRI,MRI Angiographies,MRI, Perfusion Weighted,Magnetic Resonance Angiographies