Occlusion of unilateral carotid artery in Down syndrome

Brain Dev. Jan-Feb 1996;18(1):81-3. doi: 10.1016/0387-7604(95)00114-x.


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.

Publication types

  • Case Reports

MeSH terms

  • Angiography
  • Arterial Occlusive Diseases / complications*
  • Arterial Occlusive Diseases / diagnosis
  • Brain / blood supply
  • Carotid Arteries / pathology*
  • Carotid Artery Diseases / complications*
  • Cerebrovascular Disorders / complications
  • Child
  • Diagnosis, Differential
  • Down Syndrome / complications*
  • Down Syndrome / pathology
  • Humans
  • Moyamoya Disease / diagnosis