moyamoya syndrome

Preschooler

with cafe au lait spots. AP view of a 3D maximum intensity projection of an MRA of the neck with contrast (above) and AP (below left) and inferior (below right) views of a 3D maximum intensity projection of an MRA of the brain without contrast shows diffuse hypoplasia of the left internal carotid artery. There is stenosis and occlusion of the cavernous and supraclinoid segments of the left internal carotid artery and then there is a thin left M1 segment without evidence of collaterals in the basal ganglia. Additionally, aneurysms are noted in the left A1 and A2 segments.The diagnosis was Moya Moya syndrome in a patient with neurofibromatosis type I.

Neuroimaging

assessment in Down syndrome: a pictorial review. Axial FLAIR image in a 12-year-old girl with Down syndrome and moyamoya pattern depicting the classical ivy sign, corresponding to areas of absence of normal FLAIR suppression of CSF within cortical sulci. This sign probably represents slow flow in leptomeningeal collaterals. The exam was performed without general anesthesia

Neuroimaging

assessment in Down syndrome: a pictorial review. Cerebral angiography in a 46-year-old Down syndrome patient with moyamoya pattern. a Frontal view, right internal carotid artery injection, showing occlusion of the right anterior cerebral artery, with a network of collateral vessels (moyamoya vessels) in the supra-sellar cistern with reconstitution of the anterior cerebral artery arterial flow distally. b Lateral view, left internal carotid artery injection, showing severe narrowing of this artery, which ends on the cavernous segment, where the arterial flow diverges to a meningohypophyseal trunk with anastomosis with the ophthalmic artery and dural branches of the middle meningeal artery, forming a network of collateral capillaries, typical of moyamoya syndrome