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Let's talk about primary angiitis of the CNS.

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Patients present with headaches, encephalopathy,

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and stroke-like symptom syndromes.

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This is typically caused by T cell-mediated

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inflammation in the small and medium parenchymal

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leptomeningeal vessels.

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Angiography is only 20% sensitive,

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so many patients have to go to biopsy.

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The classic findings are infarcts of multiple

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ages in multiple vascular distributions.

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Relatively small percent have hemorrhage.

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On vessel wall imaging,

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you can have smooth concentric enhancement,

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and the treatment is immunosuppressive agents.

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So these are images of a 38-year-old

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female who has acute visual loss.

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And you can see acute infarcts in the bilateral PCA,

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territories involving the right occipital lobe,

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the left occipital and temporal lobes,

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the splenium of the corpus callosum,

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and the left thalamus.

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They all have restricted diffusion,

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but you can also see chronic infarctions in the

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bilateral centrum semiovale characterized by FLAIR

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hyperintensity and facilitated diffusion.

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And this patient had a CTA that showed multifocal

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stenosis in the left MCA, the bilateral PCAs,

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and the right MCA.

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You can also see these findings in angiography

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of these multiple focal areas of beating.

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These are images of a 66-year-old female who

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has PACNS, or primary angiitis of the CNS.

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She presented with right-sided weakness.

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This is a non-contrast head CT.

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And scrolling up through it,

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I don't see any hyperdense vessel signs.

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I do see an infarct in the left lentiform

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nucleus. I see one in the right thalamus.

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I see maybe a little abnormality in the

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splenium of the corpus callosum.

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Some scattered white matter foci.

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Unclear whether those are acute or chronic.

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I don't see any hemorrhage.

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I don't see a lot of mass effect.

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And I'm going to go to the.

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CTA,

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and we can look at the neck briefly.

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You can see the vertebral arteries, and.

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They look pretty normal. You can see the.

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Carotid bifurcation on the left.

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That doesn't look very exciting.

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Carotid bifurcation on the right.

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Maybe a little atherosclerotic disease.

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Doesn't look too exciting.

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Has a big thyroid goiter,

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but that's not our focus of interest.

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Same thing in the sagittal of the neck.

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Again,

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a little atherosclerotic disease of

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the carotid bifurcation.

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On both sides.

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Here's the other side, so not very exciting.

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Vertebral arteries didn't see too much,

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so not much going on in the neck.

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We're going to look at the.

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MIPs in the head.

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And again, this is abnormal.

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You can see multifocal areas of narrowing

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in the PCAs. In the left MCA,

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you can see foci of narrowing.

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In the right MCA,

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there are some mild foci of narrowing.

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Let's look at the ACA on the.

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Sagittal MIPs,

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and you can see a little irregularity.

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Again,

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the PCAs

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look abnormal.

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Here's one side, here's the other side.

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We'll look at the coronal images to

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get another look at the MCAs.

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And again,

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you can see these multifocal areas

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of narrowing in the MCAs and.

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The left ACA here. So multifocal areas of narrowing.

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We saw multiple infarcts.

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It was hard to tell what age they were.

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The MIPs are the best way to look at these.

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We can look at the raw data,

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but it can be very hard to see the stenoses.

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We know there are stenoses in the PCAs.

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Here are the two vertebral arteries

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coming up to the PCAs.

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You can see them if you look.

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Closely,

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but it's pretty hard.

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It's much easier with the MIPs.

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Same thing with the MCAs.

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You can see the stenoses,

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but much easier with the MIPs.

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Here's the left MCA.

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One looks a little narrow.

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The left MCA.

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Some mild areas of narrowing.

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So anyway, the MIPs are the way to look at them.

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That's the raw data.

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We can look at the CTA source images to see

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if we see a bigger infarction anywhere.

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And I don't see a big hypoperfused area.

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So let's take a look at the MRI.

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This is the diffusion-weighted image.

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This is the post-contrast T1-weighted image.

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This is the ADC map,

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and this is the FLAIR images.

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And we're going to just line those up.

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And what you see here as we.

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Go up is you can see an area that's bright on DWI.

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It's sort of isointense on ADC,

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suggesting a late subacute infarct, and there's.

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Some contrast enhancement as we go up further.

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There are similar lesions in the left thalamus,

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thalamocapsular region,

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some DWI hyperintensity,

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but it's sort of isointense on ADC.

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There are some areas that are actually bright on ADC,

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suggesting it's even getting to feel older in bark.

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It's enhancing.

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There's a lesion in the splenium of the corpus

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callosum. Same story. Bright on DWI.

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There's a little restricted diffusion,

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so that's maybe a little earlier.

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There's some early enhancement.

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So those infarcts were all probably occurred.

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Within a few days.

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But we get to some other lesions up here.

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They're bright on DWI, they're isointense on ADC.

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So it's not still restricted,

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but it's not enhancing yet.

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So it's earlier than the other infarctions.

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We can see all these little multifocal

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infarcts that don't have enhancement.

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Here's another one that has restricted diffusion,

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minimal to no enhancement.

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So we have basically some early subacute

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and late subacute infarcts,

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and then maybe even this one that's cavitated

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is a little more chronic.

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So infarcts of different ages in multiple vascular

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distributions. She had inflammatory markers,

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and this was primary angiitis of the CNS.