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CADASIL or cerebral autosomal dominant arteriopathy

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with subcortical infarcts and leukocencephalopathy

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is another disease entity that can

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cause infarcts with relatively normal vessels.

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It's a non arteriosclerotic,

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nonamyloid arteriopathy of small and medium vessels.

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There's a defect in the notch three gene.

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Clinically, the patients have migraines

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in their thirties and forties,

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followed by TIAs and strokes in the next two

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decades, followed by dementia, and then death.

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What they classically have are seen in this case,

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extensive white matter lesions,

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periventricular and subcortical.

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They have a predilection for the anterior

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temporal lobe and the external capsule,

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more than you'd see in other white matter diseases.

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They can have acute strokes,

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as you can see in this case,

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which tend to be in the deep white matter.

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And in addition to all the white matter lesions,

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30% have microhemorrhages and you may

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have leptomeningeal enhancement.

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These are images of a 63-year-old female who had

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coronary artery disease, hyperlipidemia,

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and a long history of migraines.

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And she had a TIA characterized by visual

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changes and some hand and foot numbness.

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So, first we got a non-contrast head CT.

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And what I noticed on this is

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she's just got a lot of white matter

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disease everywhere.

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External capsules,

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periventricular white matter.

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Looks like she's got a little thalamic lacune.

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Just extensive white matter disease.

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Didn't clearly see an acute cortical infarction.

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We also got a CTA of the head and neck,

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and these are the axial MIP images showing that the

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MCA bifurcations and rest of the MCAs are normal.

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The PCAs look normal.

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We can look at the coronal,

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you can see the vertebral basilar junction,

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looks grossly normal here.

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Not seeing much here.

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Pretty robust vasculature.

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Again, here's the coronal MIP image

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and beautiful posterior circulation the vertebral arteries,

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dazzler artery, posterior cerebral arteries,

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anterior circulation,

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top of ICAs, MCAs, ACAs,

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bilaterally, all look pretty normal

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as we scroll through these MiP images.

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We can look quickly at the raw data.

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Again, you're just going to see beautiful vessels,

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nothing that looks very striking.

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So, we also want to look at

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the neck vessels to make sure we don't

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see much going on in the neck.

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And these are MIPs of the neck vessels

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sagittal reconstructions, and you can see

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common carotid, internal and external normal.

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And they also look normal on the other side.

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And it's a little hard to look

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at the vertebral arteries on these.

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We can look at the coronal images, as well.

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And you can see, you know, the right vertebral artery

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looks pretty good.

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And the left vertebral artery looks pretty good.

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And again,

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the carotid arteries bifurcations look good.

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Not seeing much there.

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We can go back to the raw data,

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and just look at the arch.

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And the arch looks pretty normal.

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Left subclavian, left common,

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brachiocephalic,

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right subclavian, right common.

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We can follow the common carotid arteries up bilaterally.

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We get to the bifurcations,

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and bifurcations look pretty normal.

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And again, I just showed you the

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head up to the skull base.

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They look normal.

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And the vert,

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here's the left vert coming off the subclavian,

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and we can follow that up.

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It's a little hard because there's venous contamination,

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but basically,

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we can follow the vessel all the way up,

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and it looks pretty normal.

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And then, the right side.

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Here's the right vertebral artery origin,

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and that also looks normal.

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So, no major vascular abnormality

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and a lot of white matter disease.

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Now, I'm going to show you the Mr images,

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and we can see on the diffusion weighted images,

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there is one tiny focus of acute ischemia in the

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left thalamic capsular junction.

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It's bright on DWI,

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it's dark on ADC,

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and I don't see any other abnormal

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areas of restricted diffusion.

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You will notice multiple hypointense foci,

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and there is some susceptibility on DWI,

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and that's going to be from multiple microhemorrhages,

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which I will show you on the SWI images.

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So, tiny acute infarction.

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And now, we're going to show you

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the DWI next to the FLAIR images.

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So, these are the FLAIR images on the right

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and you can see that there's extensive white matter

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abnormality in the periventricular region

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extending to the subcortical region.

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You'll notice there's prominent white matter

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abnormality in the anterior temporal subcortical white

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matter, which is very characteristic of CADASIL.

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It's unusual for other white matter diseases.

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There's also marked involvement of the external capsule.

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That's another hallmark of CADASIL.

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There's a lacuna in the left thalamus.

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Again,

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extensive periventricular and subcortical white matter.

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It's very confluent.

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And then, let me just show you the SWI images.

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So if we start at the bottom,

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you can see there are two

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numerous to count post, have susceptibility

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throughout the left greater than right cerebellum,

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and throughout the supratentorial brain,

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both superficially and deep.

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And so, these microhemorrhages and the extensive

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white matter abnormality involving

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the anterior temporal lobes and external capsules,

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and the history of migraines for a decade,

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TIAs for a decade,

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and then in other patients,

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subsequent strokes and dementia

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are very characteristic of CADASIL.