Presentation
Developmental delay with right upper limb weakness lasting for 1 day. History of recurrent transient ischemic attacks.
Patient Data
A tortuous network of signal voids is seen along the expected paths of both middle cerebral arteries. The bulk of both middle cerebral arteries is not identified, with absence of the expected normal luminal signal void.
Tortuous lenticulostriate collaterals are seen bilaterally, more pronounced around the left lentiform nucleus.
On the coronal images, there is evidence of grossly reduced luminal diameters of the supraclinoid and terminal segments of both internal carotid arteries.
On axial FLAIR, a diffusely hyperintense gyriform signal is seen particularly in both superior frontal lobes, secondary to prominent leptomeningeal collateral vessels (ivy sign).
No acute ischemia or intracranial hemorrhage.
No significant white matter disease.
Complete segmental occlusion of both middle cerebral arteries and left anterior cerebral artery.
Markedly narrowed terminal segments of both internal carotid arteries.
Extensive collateral vessel formation along the paths of both middle cerebral arteries, with tortuous lenticulostriate collaterals (puff of smoke), bilaterally. A collateral arterial network is also formed medially within both parietal lobes and cingulate gyri, more pronounced on the left side.
No intracranial aneurysm.
Case Discussion
In this particular patient, the diagnosis of Moyamoya disease had been long established following thorough testing for vasculopathy, which yielded negative results.
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