Case History: 7-year-old female with complaints of convulsions for one year. Last attack was six months prior, after which the patient was put on regular anticonvulsive therapy and has shown improvement.
Last attack was six months prior, after which the patient was put on regular anticonvulsive therapy and has shown improvement.
Figure 1. The white matter in bilateral cerebral hemispheres show multiple punctate areas of T2 hyper-intensity and corresponding T1 hypo-intensity.
Figure 2. Altered signal intensity (hyperintense on FLAIR) flow voids are noted involving high cerebral sulci, possibly due to slow flow collateral vessels (Ivy sign).
Figure 3. On 3D phase contrast angiography, there is narrowing of bilateral supra-clenoid internal carotid artery (left > right) with multiple basal collaterals noted within the white matter of bilateral cerebral hemispheres.
Figure 4. On post contrast T1 weighted fat saturated and MPR images, multiple collateral vessels are noted involving lenticulo-striate, thalamo-striate, and thalamo-perforators.
Diagnosis: Moyamoya Disease
Moyamoya disease is a chronic progressive cerebrovascular disease characterized by stenosis or occlusion of the bilateral arteries around the circle of Willis with prominent arterial collateral circulation.
MRI reveals areas of infarctions and allows direct visualization of these collateral vessels as multiple small flow voids at the base of brain and basal ganglia.
The diagnosis of Moyamoya disease is based upon the characteristic angiographic appearance of bilateral stenosis affecting the distal internal carotid arteries and proximal circle of Willis vessels, along with the presence of prominent basal collateral vessels.
