Occlusion of unilateral carotid artery in Down syndrome

The association between moyamoya phenomena and Down syndrome (DS) is reported in the literature. This paper reports a case of DS, which at age 9 presented right hemiparesis, secondary to the occulusion of the left internal carotid artery; cerebral angiography (CAG) showed a collateral circulation th...

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Bibliographic Details
Published in:Brain & development (Tokyo. 1979) 1996, Vol.18 (1), p.81-83
Main Authors: Gaggero, Roberto, Donati, Paolo Tortori, Curia, Roberto, De Negri, Maurizio
Format: Article
Language:English
Subjects:
Angiography
Arterial Occlusive Diseases - complications
Arterial Occlusive Diseases - diagnosis
Biological and medical sciences
Brain - blood supply
Carotid Arteries - pathology
Carotid Artery Diseases - complications
Cerebrovascular Disorders - complications
Child
Diagnosis, Differential
Down syndrome
Down Syndrome - complications
Down Syndrome - pathology
Humans
Medical sciences
Moyamoya disease
Moyamoya Disease - diagnosis
Moyamoya syndrome
Neurology
Stroke
Vascular diseases and vascular malformations of the nervous system
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Summary:The association between moyamoya phenomena and Down syndrome (DS) is reported in the literature. This paper reports a case of DS, which at age 9 presented right hemiparesis, secondary to the occulusion of the left internal carotid artery; cerebral angiography (CAG) showed a collateral circulation that mimicks the moyamoya phenomenon. Clinical recovery was almost complete; a second CAG after 15 months showed a persistent occlusion of the left internal carotid artery and an opacification of the left middle cerebral artery from abnormal vessels; but the collateral circulation is not enhanced. This case proves that in DS cerebrovascular occlusions may present moyamoya-like phenomena. These differ however from the true moyamoya disease in a number of aspects: the arterial occlusion is unilateral, the evolution is favorable and revascularization does not occur through the peculiar abnormal vessels of the moyamoya syndrome.
ISSN:0387-7604
1872-7131
DOI:10.1016/0387-7604(95)00114-X