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 occlusion 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.
Division of Child Neuropsychiatry, Istituto G. Gaslini, Genoa, Italy.
R
R
Gaggero
P T
PT
Donati
R
R
Curia
M
M
De Negri
D000792:Angiography; D001157:Arterial Occlusive Diseases / Q000150:complications* / Q000175:diagnosis; D001921:Brain / Q000098:blood supply; D002339:Carotid Arteries / Q000473:pathology*; D002340:Carotid Artery Diseases / Q000150:complications*; D002561:Cerebrovascular Disorders / Q000150:complications; D002648:Child; D003937:Diagnosis, Differential; D004314:Down Syndrome / Q000150:complications* / Q000473:pathology; D006801:Humans; D009072:Moyamoya Disease / Q000175:diagnosis