36 yo female with 2 week history of intermittent weakness on both sides, numbness epsodes lasting 4 hours & word finding difficulty.
 

CT Head; CT Perfusion; CTA Neck; CTA head; Post contrast CT head; T1-w MR pre and post contrast; MR diffusion; MR flair; MR susceptibility (swi)

CT Head
1. Negative study for acute arterial or venous occlusion.
2. There is a small completed lacunar infarct in the left caudate head.
3. The combination of a completed stroke and mild global atrophy and prominent basal ganglia calcificiation suggests the presence of underlying vasculopathy.

CT Perfusion
1. CT perfusion and CTA evidence of moyamoya disease with expected alteration of blood flow, as above.
Small completed infarct in the left caudate head.

CTA neck
1. Arterial vascular changes consistent with moyamoya disease.

CTA head
1. Evidence of moyamoya disease vasculopathy
2. There is a prior small completed lacunar stroke in the left caudate head.
3. There is delayed filling of distal MCA pial arteries on the right

Post contrast CT head
1. The brain CT density within the venocapillary pool is essentially normal, despite the delayed pial filling in the right MCA on the CTA head.
2. There are two small areas of persistent oligemia in the caudate head on the left and basifrontal white matter on the right. Both are likely sites of previous ischemic injury.
3. The classic features of moyamoya disease are again evident.

T1-w MR pre and post contrast
1. The vasculopathy of moyamoya disease are evident on both the pre and post contrast T1-w sequences.
2. Two sites of chronic lacunar stroke are evident. One seen in the left caudate head was also seen on the prior Post contrast head CT. The second is a very small lacunar stroke in the right caudate head adjacent to the frontal horn.
3. There is no contrast leak to suggest post ischemic dysautoregulation.

MR diffusion
1. Negative MR diffusion with no acute stroke. Small caudate head lacunes are evident on the Bo sequence.

MR Flair
1. Features of the moyamoya vasculopathy are again evident. They appear more extensive on the right than the left.
2. Both sites of caudate head completed strokes are evident; the cavities are clearly evident consistent with late effects of prior artery of Huebner terminal branch strokes.

MR susceptibility (SWI)
1. Physiologic arterial and venous hyperemia (in response to the moyamoya vasculopathy) are evident bilaterally, but more prominently on the right.
2. There is no evidence of laminar necrosis nor sequestered parenchymal stroke.

1. There is idiopathic adult type of moya-moya vasculopathy

2. There are lacunar brain infarcts in the distribution of the arteries of Huebner terminal perforating, end-arteries to the caudate heads.

1. Moyamoya disease term is relegated to cases where there is no other comorbid disorder that secondarily can have all or part of the moyamoya vasculopathy. 

2. Moyamoya disease is a peculiar, likely inherited, disorder which includes the following: 1. bilateral high-grade stenoses of the ophthalmic segement of the ICA (occuring just after the P-com and anterior choroidal artery take-offs), 2. peculiar enlargement of the A1/2 and M1/2 perforators to the basal ganglia (always) and the thalami (less often), 3. peculiar collateral connections between meningeal and intraaxial pial arteries.

3. When moyamoya vasculopathy is associated with other disorders like sickle cell disease and neurofibromatosis it is called moyamoya syndrome. A third form of moyamoya-like disorder occurs in adults, but it lacks the pial-meningeal connections and often the ICA stenoses.

4. Moyamoya disease and moyamoya syndrome are associated with any accellerated  atherosclerotic process. 

5. Both moyamoya disease and moyamoya syndrome can cause stroke in children and young adults.