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Hello and welcome to Noon Conference, hosted by Modality

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Today we are honored to welcome Dr. David Ssim

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for a lecture entitled DNS Vasculopath.

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Dr. Ssim is a neuroradiologist

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and professor of radiology at the Johns Hopkins

0:32

University School of Medicine.

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He's the author of over 350 scientific papers

0:39

and several popular books in radiology,

0:41

and has also served as the president of the as SNR

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and was awarded the Outstanding Educator

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Award from the RSNA.

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We are especially grateful to Dr.

0:49

Usein for his support of modality

0:51

and for serving as our neuroradiology advisor.

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At the end of the lecture, please join him in a q

0:56

and a session where he will address questions you may

0:58

have on today's topic.

1:00

Please remember to use that q

1:01

and a feature to submit your questions so we can get to

1:04

as many as we can before our time is up.

1:06

With that, we are ready to begin today's lecture. Dr.

1:09

Yuson, please take it from here.

1:12

Thank you very much.

1:14

Well happy, uh, Thanksgiving week for those of you

1:18

who celebrate for, for all of us.

1:20

We should always be grateful for all

1:22

of the blessings in our lives,

1:24

and I'm certainly, um, thankful for all my opportunities to,

1:28

uh, teach with you and also the opportunity

1:31

for 50% off on Black Friday Sale for Modality membership.

1:35

Very good. We were just talking about great sales.

1:39

So today I'm gonna talk to you about imaging

1:41

of CNS Vasculopath.

1:43

I changed the topic from CNS vasculitis

1:46

because I did wanna address some of those vascular

1:51

pathologies that are not necessarily in necessarily

1:54

inflammatory, but are very common that we, um, see, uh,

1:59

with regard to my disclosures, of course, I am a consultant

2:02

to modality and a lecturer here,

2:06

and also receive royalties from Elsevier

2:08

and I do medical-legal cons consulting work.

2:12

So fortunately, the nomenclature for vasculitis

2:18

was formalized, um,

2:21

at the 2012 International Chapel Hill Consensus Conference

2:26

on vasculitis.

2:28

And effectively, instead of going with a lot

2:30

of different inflamm, infectious inflammatory

2:34

and autoimmune disorders, the way they decide

2:37

to organize the vasculitis is by the size

2:42

of the vessel that is involved.

2:44

So we have large vessel vasculitis,

2:46

we have medium vessel vasculitis,

2:49

we have small vessel vasculitis, which are related

2:53

to the immune complex

2:54

and other associated small vessel vasculitis.

2:57

However, it's also well known that there are a lot

3:01

of other categories of vasculitis

3:04

that don't fit well into each

3:06

of these different classifications.

3:10

However, when we think about large vessel vasculopathy not

3:14

necessarily inflammatory, we must also

3:18

consider atherosclerosis.

3:19

And a lot of people's opinion atherosclerosis is a type

3:23

of inflammation of the large vessels.

3:26

In addition to TA's arteritis

3:29

and giant cell artery race, which are mentioned in

3:32

that Chapel Hill consensus, there are a lot of other

3:36

vasculopaths that affect the large vessels,

3:38

which I will now cover.

3:41

So let's start with atherosclerosis.

3:45

I have been blessed and I am grateful for the presence

3:48

of Bruce Wasserman in the division

3:50

of neuroradiology at Johns Hopkins for probably

3:53

around 20 years before he moved on

3:55

to the University of Maryland.

3:57

During that time, Bruce was one of the three most

4:02

foremost experts in, uh, evaluation of the,

4:07

um, blood vessels for atherosclerosis with high resolution

4:11

black blood imaging.

4:12

And some of the work that he has, uh,

4:15

that he did was just monumental in looking at not just the

4:19

degree of narrowing of the blood vessels

4:22

that we usually think of with the NASA criteria,

4:25

but also all of these papers that he wrote

4:29

regarding the composition of the atherosclerotic plaque,

4:32

because we all know that there are a lot of patients

4:35

who don't have high degree of narrowing that

4:38

nonetheless flip emboli into the brain

4:42

and cause strokes.

4:43

And this was sort of Bruce's passion for which he worked on,

4:47

uh, particularly with one of his junior faculty.

4:49

Yay Cal. So here's some of the black blood images

4:54

that Bruce produced looking at free

4:58

and post contrast plaques in order to characterize

5:02

the lipid core, the fibrous cap

5:06

and calcification,

5:07

and finally, hemorrhage in the wall of a plaque.

5:11

And what Bruce initially started looking at was the

5:14

enhancement of the wall of the plaque

5:17

and the intima for what is called the fibrous cap.

5:19

And what he initially showed was thinning

5:22

of the fibrous cap cap was associated with an incus risk of

5:27

thrombogenesis and emboli.

5:29

He moved on to looking at things like the intra plaque

5:33

hemorrhage, and it turned out that a lot

5:36

of the fibrous cap lipid core

5:38

and intra plaque hemorrhages worked together

5:41

to expose the lumen to the tima of the and

5:46

and media of the blood vessel that led to thrombogenesis.

5:50

So here are some of the additional dark blood imaging

5:55

that Bruce did with regard to atherosclerotic plaque.

6:00

Here's an example of the intra plaque hemorrhage

6:02

that is confirmed by the histopathology.

6:05

And although he came to the

6:09

intra plaque hemorrhage after fibrous cap thickening and

6:13

and lipid core turned out

6:14

that hemorrhage in the plaque was a very high risk factor

6:20

for thrombogenesis and subsequent emboli.

6:25

Eventually, Bruce moved his work

6:27

to intracranial atherosclerotic plaques

6:30

and showed that in intracranial atherosclerotic disease,

6:34

the plaque di he, uh, enhancement tended

6:38

to be eccentric and heterogeneous

6:41

and often at the bifurcation points

6:46

of the intracranial blood vessels.

6:49

And this enhancement could persist for a long period of time

6:53

with the atherosclerotic disease.

6:57

N was a risk factor for emboli

7:00

and strokes in that vessel's distribution.

7:07

Here's another example of, of three different plaques

7:10

with different amounts

7:11

of contrast enhancement associated with it.

7:14

And he was able to grade the degree

7:16

of the contrast enhancement

7:18

and showed that the degree of contrast enhancement

7:21

of the intracranial atherosclerotic plaque increased the

7:24

risk of the thrombogenesis and ultimate stroke.

7:31

When you look at all of these different components,

7:34

generally in the neck,

7:36

but also in the intracranial compartment, um, you see

7:40

that they may all occur together.

7:43

You can have a thick widened, uh, wall,

7:48

you can have the lipid rich core, you can have ulceration of

7:51

that vessel,

7:54

and that can lead to, uh, intracranial, uh,

7:59

inter plaque hemorrhage.

8:02

Another example of the, uh, thickness

8:05

of the fibrous kappa seen by the contrast enhancement,

8:08

as well as the high signal intensity that may be associated

8:11

with the hemorrhage in the wall of the blood vessel.

8:15

So this was a very nice, uh, article

8:17

that I thought thought was important to demonstrate to you

8:21

because it was a,

8:23

a expert panel looking at the consensus opinions

8:28

about relative risk of stroke associated

8:31

with atherosclerotic plaque components.

8:34

And I wanna highlight this

8:36

or column here, relative risk

8:39

of stroke if the characteristic is present.

8:41

And what you see is that the, uh, highest

8:46

risk factor is intra plaque hemorrhage followed by

8:51

the necrotic core

8:52

or the, uh, plaque thickness calcification, interestingly,

8:57

is associated with a lower rate of stroke within

9:02

that vascular distribution.

9:03

So for all of us who are all concerned about our calcium

9:06

score on our cardiac ct, uh, examination calcification

9:11

because of statins has been actually a favorable

9:16

occurrence in a lot of cases of atherosclerotic plaque,

9:20

at least with regard to the carotid arteries.

9:23

So it sort of stabilizes the plaque such

9:26

that it is not at risk for, uh, stroke.

9:29

So, uh, again, to to emphasize intra plaque hemorrhage,

9:34

a very important risk factor for the

9:38

subsequent stroke risk with atherosclerotic plaque,

9:42

lipid rich corn thickness of the plaque a also important,

9:48

obviously the other thing

9:49

that we look at when we're looking at the blood vessels in

9:52

the neck is not just atherosclerotic disease

9:54

and intra plaque hemorrhage,

9:56

but wall hemorrhage,

9:57

which may occur under such circumstances as trauma

10:00

or fibromuscular dysplasia or other vasculopath.

10:04

And here you see a carotid artery, which is narrowed

10:07

and has this bright signal intensity, um,

10:10

from a wall hemorrhage.

10:12

This is pretty high up in the neck.

10:14

This is not at the expected location

10:16

for the carotid atherosclerosis at the um, bifurcation.

10:20

And here is an angiogram showing a similar case

10:24

with dissection of the carotid artery leading

10:27

to intracranial.

10:28

He, um, stroke.

10:31

In addition to dissections, we can also have the outpouching

10:36

of those blood vessels either associated

10:39

with a traumatic event

10:40

or one of these vasculopaths that predisposes

10:43

to initially dissection and then pseudo aneurysm formation.

10:47

And we're seeing it here on the MRA and the CTA

10:50

and the conventional arteriogram with a pseudo aneurysm.

10:56

I'm gonna get back to intracranial black blood imaging

10:59

for vasculitis,

11:01

but I do want to emphasize what Bruce taught us all,

11:04

and that is that remember

11:05

that the atherosclerotic disease intracranial tends

11:09

to be eccentric around the blood vessel

11:12

and more commonly at bifurcations.

11:14

And you see the contrast enhancement here, whereas

11:17

what we're gonna see with vasculitis is that it is a more

11:21

symmetric and diffuse concentric enhancement.

11:26

And that's gonna be our distinguishing feature

11:28

between atherosclerotic intracranial vascular

11:32

disease versus the intracranial ities such as,

11:37

um, P-A-C-N-S.

11:39

Here again, you see the nice and smooth

11:41

and concentric involvement with ities as opposed

11:46

to the eccentric involvement with atherosclerosis.

11:51

So let's deviate from atherosclerosis

11:55

and get back to the classification of the, uh,

11:59

chapel Hill consensus.

12:00

And as you see, under large vessel vasculitis, the two

12:03

that they describe are tso's, arteritis

12:07

and giant cell arteritis.

12:10

So tso's arteritis generally in neuroradiology we're more

12:15

concerned with the subclavian arteries

12:17

and the potential hit on the vertebral arteries.

12:20

And I remember from residency I had a great case

12:23

of subclavian steel

12:24

because of tso's arteritis affecting the subclavian artery

12:29

proximal to the takeoff of the vertebral artery.

12:32

But frankly, tatsu arteritis is something that we see once,

12:36

twice, three times a year, um, as opposed

12:39

to the chest radiologist who may be more commonly seeing it.

12:42

This is a particular example of a patient

12:44

who has a pulmonary artery, uh, involvement, uh, with

12:49

tatsu arteries.

12:50

But nonetheless, you can see

12:52

that involvement in the narrowing in the subclavian artery

12:56

on the right side over here and on the left side as well.

13:04

Rarely we will see tatsu arteries affect the

13:07

carotid arteries.

13:08

This is an example of that where you see a focal area

13:12

of narrowing actually two areas

13:14

of narrowing in this carotid artery in a patient

13:17

who had bad disease affecting the right-sided

13:20

subclavian artery as well.

13:26

The second of the large vessel vasculitis

13:29

that were was described as the giant cell arteritis, again,

13:33

we usually don't see that intracranial

13:36

that's more commonly seen around the blood vessels

13:39

as the takeoff from the aorta, for example,

13:43

and elsewhere in the body in this case,

13:45

the abnormality is the degree of wall thickening

13:49

that you see around the enhancing lumen on this CTA.

13:53

And this is more than what one would expect for, uh, in

13:58

as far as diffuse involvement, as far as atherosclerosis,

14:02

of course, we would look at the age of the patient

14:04

and this would, um, be uncommon in a younger patient

14:08

to see this amount of wall thickness of all

14:11

of the vessels taking off from the aorta.

14:16

With regard to the intracranial manifestations

14:20

of something like a giant cell art arteritis, remember

14:24

that we wanna look for enhancement

14:27

and thickening that may be narrowing the lumen,

14:32

but that is concentric around the blood vessel

14:35

to distinguish it as Ted previously from the

14:38

atherosclerotic etiology.

14:42

Now, the consensus did not talk about other large vessel

14:46

vasculopath such as Moya Moya disease

14:49

and some of the other entities I'm

14:51

about to describe for you.

14:52

And I think that's because they did not consider them

14:55

primary arteritis

14:57

and inflammatory conditions as opposed

15:00

to a vasculopathy without necessarily those

15:03

inflammatory components.

15:05

However, we see Moya Moya a lot more

15:08

commonly than we see something like giant cell arteritis

15:11

or Taki's arteritis, particularly at Johns Hopkins,

15:16

in part because of the predisposing factors

15:19

for Moyamoya syndrome.

15:21

And that includes things like sickle cell disease

15:23

that we see a lot of in East Baltimore.

15:26

So here we have a patient that we are looking at the, uh,

15:31

circle of voice vessels,

15:32

and as we see, we sort of see this petering out

15:35

of the distal internal carotid arteries bilaterally.

15:38

And we have that kind of lenticular stripe

15:43

c collateral appearance that people will talk about

15:47

as a sort of puff of smoke, better seen, for example,

15:51

on conventional arteriography.

15:53

But we do see this as well with our, uh, CTAs,

15:58

and you can see that the distal involvement

16:01

of the internal car arteries kind of classic,

16:03

however it may extend into the proximal A one

16:07

and M1 segments of the anterior cerebral

16:09

and middle cerebral arteries.

16:11

Now, we have a expert in our, uh,

16:16

in our institution, Raphael to Margo on Moya Moya,

16:20

and he makes the distinction really pushes the, uh,

16:25

the emphasis here that Moya Moya disease is that entity

16:29

that affects bilateral involvement

16:32

of the distal carotid arteries in these vessels

16:35

and generally is of an idiopathic etiology when we see

16:40

a single carotid artery with a puff of smoke

16:43

that's secondary to sickle cell disease

16:45

or to radiation vasculopathy or to severe atherosclerosis.

16:50

That term should be Moya Moya syndrome

16:53

or Moya Moya appearance.

16:54

But the disease, as it is described,

16:58

should be bilateral involvement

17:01

and generally of an idiopathic

17:03

and more common in the, uh, Asian population.

17:07

Um, the other entity that is a large vessel vasculopathy

17:10

that I should mention is fibromuscular dysplasia.

17:14

Here we have several examples

17:15

of different patients from our teaching file

17:18

with fibromuscular dysplasia where we have

17:21

that beaded appearance of widening and narrowing

17:24

and widening and narrowing on multiple blood vessels.

17:28

Here you can see that area of narrowing and widening

17:31

and, um, in this case, even a dissection

17:35

with a pseudo aneurysm of the right

17:37

distal internal carotid artery.

17:40

The widening here,

17:42

the dissection flap on the right side

17:45

of the distal internal carotid artery,

17:47

which is also seen here.

17:49

So fibromuscular dysplasia, you know, we used

17:51

to have all these different varieties, the medial

17:54

and intimal and adv vental varieties.

17:56

Now the nomenclature basically is,

17:59

is it focal or multifocal?

18:01

In other words, is it a single happening in a single vessel

18:07

or is it in multiple vessels with multiple changes

18:11

over the same vessel?

18:12

So the widening and narrowing

18:14

and widening narrowing as opposed to a single widening

18:17

and narrowing, if you will, for the focal versus

18:20

the multifocal involvement bilateral

18:22

and in multiple portions of the same vessel.

18:27

Fibromuscular dysplasia, again, not listed as a vasculitis,

18:31

but considered a vasculopathy

18:33

because it's not, uh,

18:35

really inflammatory cells affecting the wall.

18:38

Um, can may manifest intracranial with strokes

18:42

or with TIAs with dissections subarachnoid hemorrhages

18:47

of of the dissected vessels

18:49

or pseudo aneurysms of the vessel.

18:51

Or there is a higher rate of aneurysms itself in these

18:56

patients who have fibromuscular dysplasia such

18:58

that it may be one of the entities that we do screening

19:01

for aneurysms, intracranial, uh,

19:05

the carotid arteries are affected more commonly than

19:09

the vertebral arteries.

19:10

And bilateral involvement

19:12

of the carotid artery is seen in 61%

19:14

of those that are affected.

19:16

It's usually an extracranial manifestation,

19:20

so not really central nervous system, if you will,

19:23

more in the neck, but you may see it in 14%,

19:26

both intracranial and extracranial,

19:29

and 11% just intracranial.

19:32

So obviously 25% have intracranial involvement,

19:36

and that involvement may be narrowings, widenings,

19:39

aneurysms, dissections, intracranial.

19:43

The risk of stroke in a patient

19:44

who has fibromuscular dysplasia is around, uh, one

19:47

to 5% per year according to this article.

19:53

Of course, fibromuscular dysplasia is not just

19:56

manifested in the central nervous system

19:57

or in the cerebral cranial or cranial cerebral vessels, um,

20:03

or cervical cranial vessels in the neck and intracranial.

20:07

Um, the renal arteries are often affected

20:11

even more so than the internal carat arteries

20:14

and the manifestations in the renal with the renal arteries,

20:16

as it may be a source of patients hypertension,

20:20

you can have involvement of the mesenteric arteries leading

20:23

to mesenteric ischemia mean age

20:26

57, 40 3% of patients

20:29

with cervical cranial disease have renal disease.

20:33

Anyway, we see the same sort of manifestation, the string

20:37

of bead sign, maybe the, you know,

20:39

the pseudo aneurysm dilatation of the blood vessel.

20:42

You could see that on both the conventional arteriogram here

20:46

as well as a nicely done CTA showing those vessels.

20:53

Finally, of those large vessel vasculopaths

20:56

that may affect the intracranial

20:58

or the extracranial vessels, I should report on, um,

21:02

those patients who are affected by radiation.

21:05

Radiation, while it can lead to a vasculopathy in

21:09

and of itself, also leads to acceleration

21:13

of atherosclerotic disease.

21:14

And this was actually taught to me

21:16

by my good friend SAB Chung from, um, South Korea,

21:21

who was visiting us in at the University of Pennsylvania.

21:24

And, um, asked to do a project on looking at the degree

21:29

of vascular narrowing on pre radiation

21:33

and post-radiation carotid arteries

21:36

to see whether there was a manifestation

21:39

from the radiation therapy.

21:40

Normally, we wouldn't expect that much

21:42

of a difference in the degree of narrowing over the course

21:46

of, say, six months in, um, in adult patients.

21:50

However, when we looked at the pre radiation in the six

21:54

month host radiation scan,

21:57

what we found was here we have a patient

21:59

who pre radiation the grade

22:00

of narrowing was zero host radiation.

22:03

You can see that the, it got into the mild and mi moderate

22:07

and and severe forms.

22:09

And so there was this upgrade in the degree

22:13

of atherosclerotic narrowing or,

22:14

or vascular narrowing in the neck in 56%

22:19

of the vessels that were within the radiation portal.

22:23

And the average grade upgrade

22:26

was 1.4 from like one to effectively 2.4

22:30

or from three going up to the, uh, five or four range.

22:35

So you see that here. Here's the vessel pre radiation,

22:40

here's the vessel post-radiation,

22:42

and there were areas of narrowing.

22:44

So radiation vasculopathy both intracranial

22:48

where you may see narrowing.

22:49

And as I mentioned, it may be the source of, uh, moyamoya

22:54

syndrome, not disease, but syndrome

22:56

affecting the VD blood vessels.

22:58

And that may be unilateral or bilateral.

23:00

Frankly, a lot of times we see radiated, uh, cell

23:04

for pituitary adenomas that could not be completely resected

23:08

or meningiomas that are being treated.

23:11

And you may see bilateral cavernous carotid artery

23:14

or resinoid internal carat artery narrowing secondary

23:18

to the radiation that may occur.

23:20

Also with nasal pharyngeal carcinoma, which again may be

23:23

one side if the phos romule on the left side is involved.

23:28

For example, another entity

23:31

that will look like a large vessel vasculopathy is RCVS,

23:36

reversible Cerebral Vasoconstriction syndrome.

23:40

This is a syndrome that is characterized

23:42

by the thunderclap abrupt onset headache.

23:45

Again, worse headache of life.

23:48

Um, the vasoconstriction is temporary.

23:51

It usually does resolve over the course of weeks to months.

23:55

And the risk factors for RCVS overlap press.

23:58

And in fact, sometimes you will have white matter changes

24:03

in the brain on MRI that simulate press with patients

24:07

who have RCVS.

24:09

However, they also will show vasoconstriction large vessel

24:13

narrowing vasoconstriction in the patients.

24:17

Unfortunately, that vasoconstriction may not be present

24:22

on the first day of presentation

24:25

with the thunderclap headache.

24:26

It actually may develop over the course of three

24:29

or four days that you see vasso constriction.

24:31

So it's almost, uh, if you will,

24:33

it's almost like subarachnoid hemorrhage where we don't see

24:36

that vasoconstriction initially on the original, uh,

24:40

subarachnoid hemorrhage from say, an aneurysm.

24:42

But over the course of days, it gets worse.

24:45

And you can see just how narrowed that may be in the mca,

24:50

for example, or the A one segment on this patient.

24:53

And, um, also in, on some of these examples, uh, note that

24:57

with RCVS, there may be some element

25:01

of subarachnoid hemorrhage that is associated

25:04

with the RCVS seen here as the lack of suppression

25:08

of the CSF on the flare image.

25:13

Another large vessel entity

25:15

that I should mention is amyloid angiopathy.

25:17

Again, not necessarily a vasculitis.

25:20

This is usually seen in older patients.

25:23

I won't say elderly, I'm not sure what

25:25

that is anymore since I'm now Medicare eligible.

25:29

Uh, multiple cortical hemorrhages, different ages,

25:33

sometimes leading to subarachnoid hemorrhage,

25:35

sometimes leading to subdural hematomas

25:38

and sometimes leading to hem cirrhosis

25:41

or cirrhosis of the, uh, p arachnoid

25:45

differential diagnosis is often malignant hypertension,

25:49

vascular malformations, multiple mbi.

25:51

Obviously we're going look into the EMR.

25:53

If the patient is not hypertensive, never been treated

25:55

for hypertension, et cetera, then we're going

25:58

to more likely float the idea

26:00

that this may be amyloid angiopathy.

26:02

Here's a patient with a low bar hemorrhage on MRI as well

26:07

as CT scan negative with the arteriogram,

26:11

but a vasculopathy amyloid angiopathy, it's important

26:15

to do your grade and echo.

26:16

Or in most cases now,

26:17

people now have susceptibility weighted imaging

26:20

because that will show the multiple hemorrhages

26:23

that can occur in a patient

26:25

with amyloid angiopathy in addition to the parenchymal

26:29

or subcortical white matter hemorrhages.

26:32

What we see here is that hemorrhage that has been,

26:37

uh, implanted or

26:38

or deposited in the PIA arachnoid such that we have acidosis

26:43

or hemo acidosis for those of us who like multiple, um,

26:48

syl syllabic words.

26:51

Um, so both hemorrhage in the periphery

26:54

and the cortex as well as hemorrhage leaking into

26:57

and depositing in the p retinoid amyloid angiopathy

27:01

that occurs in about 30% of cases, the hemo cirrhosis.

27:06

Um, there is the Boston Criteria 2.0 for

27:11

cerebral amyloid angiopathy.

27:13

I'm gonna let you read this on your own or look it up.

27:17

Suffice it to say that they do include for probable

27:22

C-A-A-M-R-I scanning findings of intra cerebral hemorrhage,

27:27

micro bleeded, superficial cirrhosis,

27:29

and subarachnoid hemorrhage.

27:32

So we'll move ahead. Uh, another example,

27:35

this is the same patient I showed previously with that

27:38

low bar hemorrhage, but if you look elsewhere, you see

27:41

multiple other little micro hemorrhages in the brain

27:44

scattered about, and that's pretty typical

27:46

for amyloid angiopathy.

27:49

So it's about time to move from the large vessels,

27:52

which we took about 28 minutes

27:54

and move to the medium vessel vasculitis.

27:58

And the two that are mentioned in the Chapel Hill consensus

28:01

are poly arteritis, Noosa, and Kawasaki disease.

28:08

Poly arteritis. Noosa is a necrotizing vasculitis medium,

28:13

as well as some overlap to small vessels

28:16

that may be associated with kidney problems as well

28:20

in the central nervous system, which is affected one third

28:23

of the time, you can have aneurysms, hemorrhage thrombosis.

28:28

However, the VAs novarum

28:31

of the peripheral nervous system is a more common

28:35

manifestation than in the central nervous system.

28:37

So it may lead to a peripheral neuropathy.

28:41

So in addition to all those other entities, usually,

28:43

you know, most commonly diabetes

28:45

or, uh, for peripheral neuropathy, um,

28:48

PI arteritis OSIS is one of those etiologies

28:51

for peripheral nervous system involvement.

28:54

And interestingly, ous OSA may affect spinal vessels.

28:59

In fact, I have a good case thereof.

29:02

So, aneurysms in the brain

29:05

and stenosis, this was a patient who had, uh, aneurysm

29:08

of the vertebral artery with a small pseudo aneurysm,

29:12

and you could see that thrombosis of

29:15

that aneurysm on the fat suppressed t One way its scan.

29:19

So by applying the fat suppression, we eliminate any,

29:22

you know, of the atherosclerotic plaque

29:25

components being bright.

29:27

And what we see on the,

29:28

as the bright signal is usually the hemorrhage in the wall

29:32

or hemorrhage in the thrombo aneurysm.

29:36

Occasionally you will have that with slow flow.

29:38

And, um, the artifact of slow flow.

29:44

Here is the example of a patient who had, uh,

29:48

aneurysms a as well as vascular narrowings.

29:51

You could see the left T nine, the right T seven vessel

29:56

in the spinal vessels with involvement of

29:59

high arteritis Noosa here, the vertebral artery with areas

30:04

of multiple narrowings as well as,

30:08

uh, dilatation.

30:09

Now this, this might look like fibromuscular dysplasia.

30:12

We'd give contrast FMD by

30:15

and large for, uh, for

30:20

black blood imaging, FMD not usually enhancing

30:24

piase OSA would show that concentric ring enhancement

30:27

around the, the blood vessel.

30:33

Okay? One of the other main, uh,

30:37

VA vasculopaths that we see in neuroradiology

30:41

is the primary angiitis of the central nervous system,

30:45

or P-A-C-N-S, they call it here,

30:47

primary central nervous system vasculitis.

30:50

So that's listed in the variable vessel vasculitis category

30:55

for, uh, the consensus.

30:58

So not necessarily one that,

31:01

that they described when they're looking at the full body

31:04

picture of vasculitis.

31:07

But, um, primary angiitis of the CNS,

31:10

and you'll see it as PA CNS

31:13

has specific criteria which include, you know,

31:15

confirmation obviously on a tissue biopsy,

31:18

but high probability is with, uh, on angiograms

31:21

or with abnormal findings on the MRI

31:24

or A CSF profile consistent with it.

31:27

Um, this is distinguishable from the RCVS

31:32

by the symptomatology, not usually presenting

31:34

with a thunder cup headache, for example.

31:38

And also the vascular findings

31:40

that we'll see momentarily on our

31:44

in black blood imaging.

31:48

So primary angiitis of the central nervous system as defined

31:52

by the archives of neurology.

31:54

This is showing the difference between P-A-C-N-S

31:58

and RCVS, again, chronically progressive versus

32:02

acute severe thunderclap headache.

32:06

Um, the, the neurologic symptoms occur with the headache,

32:10

whereas with P-A-C-N-S, you may have, you know,

32:13

strokes basically that are occurring

32:15

and causing the neurologic findings.

32:18

The CSF showing the leukocytosis, the

32:22

vasculopathy vasculitis in the CSF

32:26

for P-A-C-N-S not seen in the RCVS patient.

32:30

So, uh, what do we see with, um,

32:33

primary angiitis of the central nervous system?

32:36

We usually see strokes often in the subcortical white matter

32:38

or the deep gray matter, not as much cortical involvement.

32:42

They're in multiple vascular distributions,

32:45

but not out to the periphery.

32:47

So, you know, sometimes we see things in multiple vascular

32:50

distributions and we think, ah,

32:52

maybe this is cardio embolic disease.

32:54

Um, those are usually out in the periphery

32:57

and more cortical, whereas the

33:00

P-A-C-N-S is more in the subcortical white

33:02

matter, for example.

33:04

They usually, um, are not enhancing

33:07

with thromboembolic disease, but may enhance in one third

33:11

and meningeal enhancement with P-A-C-N-S.

33:14

And that's very useful

33:16

because that location of the meningeal enhancement may lead

33:19

to the targeted biopsy of the, um,

33:24

meninges to make the diagnosis when the

33:29

angiogram and the MRI are not suggestive,

33:32

because sometimes they may have, uh, normal angiograms,

33:35

abnormal MRI scans.

33:39

So here you can see a very large differential diagnosis

33:42

of P-A-C-N-S.

33:43

I won't go through it, but anything that basically can cause

33:47

repetitive multiple neurologic episodes.

33:54

So here again, is, uh, where our vascular imaging

33:57

of the intracranial vessels is very useful, both for the mrs

34:02

as well as the black blood imaging.

34:04

Here, for example, we see the patient

34:06

that's just look at this right posterior cerebral artery

34:09

where we see areas of vascular narrowing, uh,

34:12

amidst the normal caliber of the blood vessel,

34:16

both in the longitudinal section of the blood vessel,

34:20

as well as in this case a focal area at the bifurcation.

34:23

When we look at our post gadolinium enhanced MRA,

34:28

we see enhancement on both sides

34:30

of the posterior cerebral artery, which corresponds to this

34:34

peres cephalic portion of the blood vessel,

34:38

and its more distal portion over here.

34:42

This is probably the most flagrant example of an artery

34:46

that I had seen and was loaned to me by, um, Bruce

34:49

and Y Cow.

34:51

And what you see here is just the remarkable enhancement

34:56

of the middle cerebral artery

34:58

beyond the bifurcation on both sides of the blood vessel

35:03

bilaterally and just incredibly thick.

35:05

And I, I will admit that on this vessel,

35:07

it looks like it's a little bit eccentric,

35:09

but you have lots of vessels where you see it on both sides

35:12

of the vessels and associated with the narrowing.

35:18

Another example, sometimes the manifestation looks like it's

35:22

parenchymal with the blood vessel central within

35:26

that parenchymal abnormality.

35:28

And I, I give this example, here's a small flow void

35:33

with enhancement around it,

35:35

but you can see that there's actually other little

35:37

black dots here.

35:38

And if we were doing seven Tesla imaging,

35:40

I imagine this would really be quite, uh, extraordinary.

35:44

But this patient who has primary angiitis

35:46

of the central nervous system had some parenchymal

35:49

enhancement around these vascular narrowings

35:52

and enhancement, as well as the meningeal enhancement,

35:56

which I mentioned can be used

35:59

to target for biopsy.

36:05

Okay, we're moving from the, uh, medium vessel vasculitis

36:09

to our small vessel vasculitis

36:11

and some of these small vessel vasculitis we don't see very

36:15

commonly in neuroradiology or in the central nervous system,

36:18

and, uh, frankly at at Johns Hopkins.

36:22

So, um, some

36:23

of these I'm gonna be showing images from the literature.

36:26

So this is iga, a vasculitis, that's the new name

36:29

that has been applied, uh,

36:32

previously henna shown line disease.

36:35

And the CNS manifestations of IGA

36:38

vasculitis are more commonly revolving

36:41

around hypertensive encephalopathy, such

36:44

that it is in the differential diagnosis with press, as well

36:49

as some of those cases of RCVS that are, uh, also associated

36:53

with hypertension that may be, uh,

36:56

manifesting white matter disease.

36:58

So you can see here, uh, this is, uh, before and

37:02

after treatment,

37:04

and you see the high signal intensity bilaterally in the

37:06

cerebellar hemispheres as well as in the

37:09

bridal occipital lobes.

37:10

And that with treatment with steroids, for example,

37:14

can improve dramatically.

37:16

You may also find hemorrhages in the brain associated

37:20

with IGA vasculitis with regard to

37:24

what was previously called wegener's granulomatosis,

37:27

but now we refer to it as GPA

37:31

granulomatosis with polyangiitis.

37:34

So GPA, uh, this is again, generally considered small

37:39

to medium vessel vascularity that is, um,

37:43

associated with anti neutrophil

37:47

cytoplasmic antibodies.

37:48

So you may have seen on that initial discussion

37:52

of the nomenclature of vasculitis, we were talking about

37:56

ANCA and non ANCA vasculitis.

37:59

The ANCA refers

38:01

to this anti neutrophil cytoplasmic antibodies, which can be

38:05

detected that are associated

38:08

with the myelo peroxidase system.

38:11

In this example, we have necrotizing granulomas.

38:13

And frankly, most of the time when I'm thinking about GPA,

38:17

I'm usually thinking about manifestations in the perinasal

38:20

sinuses and in the orbits with inflammatory conditions

38:24

with inflammatory disease, often with associated

38:28

nasal septal perforations, for example,

38:31

and paranasal sinus disease with bony erosions.

38:35

So nonetheless,

38:36

the central nervous system is involved in about seven to 11%

38:41

of cases with GPA common,

38:43

more commonly seen in the body manifestations,

38:46

including in the kidneys and in the chest.

38:50

So, um, there is another entity which is called eosinophilic

38:55

granulomatosis with polyangiitis.

38:57

It's similar to GPA,

38:59

but the difference is that the white blood cell,

39:01

the blood cell that's associated with it is an eosinophil

39:04

as opposed to the neutrophil that is classic

39:08

with the, um, GPA.

39:11

So here is my vision of GPA

39:16

as a neuroradiologist.

39:17

That is CY nasal disease associated

39:20

with nasal septal perforation, bony erosion,

39:23

and potentially involvement of the orbits

39:28

with this diffuse inflammatory process that encase the

39:32

extraocular muscles and the extra extra conal space,

39:37

and generally low signal intensity on

39:40

T two weighted images of the orbits as well

39:44

as the cy nasal cavity.

39:45

However, um, as I mentioned, the meningeal involvement,

39:50

uh, is one of the more common

39:52

of the central nervous system involved

39:54

and more so than what we see with regard to the vasculitis.

39:57

But nonetheless, this patient was one

39:59

that did show manifestations in the middle cerebral artery,

40:03

uh, distribution of the, um, vasculitis.

40:10

Um, sometimes with both, um, GPA wagoners as well

40:14

as sarcoidosis, we see these little micro hemorrhages,

40:18

particularly around these small vessels at the

40:21

periphery of the brain.

40:23

And that is another of the manifestations of, uh, GPA.

40:27

You see all this little tiny enhancement

40:30

of the small vessels similar to

40:32

what we were seeing in previously in the patients

40:35

who had primary angiitis of the central nervous system.

40:38

So this kind of spotty enhancement, which may be associated

40:42

with the wall enhancement

40:45

of a vasculitis would be another picture

40:47

that you should put in your mind's eye with regard to, um,

40:54

granulomatosis with polyangiitis.

41:00

So I mentioned the other entity that's associated

41:03

because it's has eosinophils as opposed

41:05

to neutrophils associated with it.

41:07

And this is the eosinophilic granulomatosis polyangiitis.

41:11

The American College of Rheumatology says you have

41:14

to have four or more of these manifestations,

41:16

and it really kind

41:17

of looks almost like allergic fungal sinusitis from the

41:21

standpoint of asthma, blood eosinophilia, involvement

41:25

of the perinasal sinuses,

41:26

sometimes pulmonary in infiltrates peripheral neuropathy

41:30

and extravascular eosinophilic infiltration,

41:34

central nervous system involvement, relatively uncommon

41:37

with regard to breast intracranial hemorrhage vasculitis.

41:45

Here is a case from the literature

41:47

of a patient showing the cy nasal involvement

41:50

with the eosinophilic granulomatosis as well

41:54

as white matter disease

41:56

and areas of vascular narrowing that you can see.

41:59

Again, the white matter disease may look like press.

42:01

Here's something a little bit more anterior.

42:04

This is more of the posterior reversible encephalopathy

42:07

syndrome example with, uh,

42:11

eosinophil granulomatosis polyangiitis.

42:15

So let's look at a few of these other entities.

42:17

We, we've dealt with the kind of the non ANCA ones

42:21

and the ANCA associated small vessel vasculitis,

42:24

including GPA

42:25

and the eosinophilic granulomatous with polyangiitis,

42:29

formerly known as Chi Strauss Disease.

42:32

Let's look at some of these other entities

42:33

that we may see in neuroradiology.

42:37

So I mentioned neurosarcoidosis.

42:39

By and large, most of the time when we're looking at

42:41

neurosarcoidosis, we're looking at meninge pathology

42:44

or enhancement along the cranial nerves,

42:46

and not so much the vascular manifestations of sarcoidosis,

42:50

however you can see those vascular manifestations.

42:54

It used to be that we thought this was more on the venous

42:56

size side, and you can have venous infarctions

43:00

and peripheral bleeding from venous infarctions.

43:03

However you may manifest as you see here in the middle,

43:06

cerebral arteries and arterial variety

43:10

of neurosarcoidosis, vascular epithelial granulomas

43:14

of perforating arteries

43:16

and veins, often with, uh, manifest

43:20

manifesting as stroke.

43:21

In addition to those meningeal

43:24

and cranial nerve pathologies that we see

43:26

with neurosarcoidosis, this is another entity

43:28

where clearly you wanna look at the perinasal sinuses

43:31

and the orbits, because you may see the sarcoidosis

43:35

embalmment of that as well.

43:38

What about lupus? Well,

43:39

they didn't mention lupus vasculitis.

43:41

So a lot of times lupus vasculitis

43:44

or vasculopathy is more considered a thrombotic entity

43:49

rather than an inflammatory disease, and that's

43:51

because of the antiphospholipid antibodies, the,

43:55

the lupus anticoagulant, um, uh, associated, uh,

43:59

serology that may predispose one to a stroke.

44:05

So most of the time we're not really seeing

44:08

vascular enhancement with lupus.

44:09

What we're seeing is a patient who has multiple strokes,

44:14

unknown source, doesn't really have a coagulopathy that is

44:19

manifested by, you know, the typical compliment cycle or,

44:24

or coagulation cycle,

44:26

but is associated with the lupus antiphospholipid antibody,

44:31

tuberculous, anti angi, angiitis well known,

44:36

manifested secondary to the meningitis that affects the

44:40

basal cisterns, and

44:41

therefore those perforating arteries,

44:43

particularly articular strides

44:45

or thalamic, uh, perforating vessels, et cetera, um,

44:50

may have central sort of stroke involvement.

44:54

Here's a patient who has the meningitis of, with, uh,

45:00

TB showing, um, communicating hydrocephalus with gumming up

45:04

of the subarachnoid space

45:06

and the, um, you know, the,

45:11

the CSF production and resorption,

45:15

but also showing the enhancement all around the circle

45:19

of Willis, as well as in the interocular cistern by virtue

45:22

of that vasculitis.

45:24

And therefore, what we usually see are the, um, you know,

45:27

the M1 segment and the lenticular stride involvement, either

45:31

with the VAs vascular enhancement

45:33

or the strokes that are seen in the basal ganglia,

45:36

brainstem, thalamus, et cetera.

45:38

And here you can see a pretty dramatic one.

45:40

This is actually a flare post contrast scan.

45:43

So patient has hydrocephalus secondary to the, you know,

45:48

arachnoid VII being gummed up with TB

45:52

and with, um, central strokes here around the basal ganglia,

45:56

maybe even the hypothalamus

46:02

with respect to the clinical manifestations,

46:04

they may be motor deficits more so related

46:06

to basal ganglia involvement rather than, for example,

46:09

the motor cortex confusion, headaches, coma, seizures,

46:13

et cetera, strokes in 50% hydrocephalus,

46:16

40% meningitis as you see here.

46:19

Look at, look at the sylvan fissure

46:21

with the meningeal enhancement

46:22

and obviously all the blood vessels within the subarachnoid

46:26

space of the Sylvan fisher exposed to

46:29

that inflammatory disease

46:31

and potentially manifesting ischemic injury.

46:36

Usually the MCA another

46:39

of the infectious inflammatory vasculopath includes

46:43

varicella zoster vasculitis, again,

46:47

more commonly lenticular stride, M1 disease.

46:50

This one, unfortunately,

46:51

having a large middle cerebral artery distribution

46:53

infarction took out portions of the A one segment on this,

46:58

knows that the distal internal carotid artery

47:00

here is narrowed.

47:01

The proximal M1 is narrowed.

47:04

We don't have a good puff of smoke to say

47:07

that this is Moya Moya,

47:08

but th this could be a manifestation that could lead

47:12

to Moya Moya disease.

47:13

And then with the contrast enhanced black blood imaging,

47:17

you see the enhanced more on both sides

47:19

of the vascular wall, a little bit eccentric I give you,

47:22

and also in the, um, meninges

47:27

ankylosing spondylitis.

47:29

Generally we don't see intracranial manifestations of

47:33

that vasculopathy,

47:34

but we may see the involvement of the aorta

47:37

manifesting in the proximal branches that can lead to, uh,

47:42

strokes associated with the vascular involvement.

47:44

And usually that's more

47:46

of a distal aorta disease than a central

47:49

nervous system disease.

47:50

Inflammatory bowel disease can lead to a vasculitis,

47:54

and that also can lead to these areas of narrowing,

47:58

narrowing, narrowing within, in this case,

48:02

a pretty good segment of the peric collosal artery, as well

48:04

as the colossal marginal artery with areas

48:08

of vascular narrowing

48:09

and even cutoff of some of those blood vessels.

48:12

So this is, um, Crohn's disease, for example.

48:16

All those other inflammatory bowel disease,

48:17

ulcerative colitis may manifest

48:20

with a central nervous system.

48:22

Vasculitis, it's unusual,

48:23

but another of the things to consider lymphoma may also lead

48:28

to a vasculitis, and this may be a vasculitis associated

48:31

with either the treatment of the lymphoma,

48:33

the lymphoma itself,

48:34

or sometimes remember we do have intravascular lymphoma,

48:38

which can lead to presentation with, with even strokes,

48:43

even though it's a lymphoma final diagnosis.

48:47

And once again, you may see the enhancement

48:50

of the vascular wall associated with either treatment

48:53

of lymphoma with some of the agents such as, uh,

48:56

l asparaginase that can do that,

48:58

or the lymphoma itself in this case.

49:00

Note also that we have really nice manifestation

49:03

of peel enhancement

49:05

of the periphery here in the parietal occipital lobe

49:09

bilaterally, as well as the vascular contrast enhancement.

49:15

All other autoimmune vasculitis one was not

49:18

well characterized.

49:20

Um, you can see that this may

49:23

progress pretty severity from 2014 to 2016

49:27

and have this ill-defined VA enhancement

49:31

that when you drill down on it more closely,

49:34

you can see is also associated with the vascular wall

49:38

I mentioned, uh, as, uh, l asparaginase.

49:42

But a lot of the other medications that are given

49:44

for things like lymphoma, leukemia may lead to a vasculitis.

49:49

And as you can see here,

49:50

this was pretty dramatic in the right middle cerebral artery

49:53

beyond the bifurcation, as well as in the M1 segment

49:56

and the A one segments

49:58

after the asparaginase was discontinued,

50:03

they repeated the MRA

50:05

and look at, look how well it all healed

50:07

and returned to a more normal appearance.

50:11

So asparaginase may be a thrombotic effect,

50:13

but it may also, as with other medications used in leukemia,

50:18

lymphoma may actually lead to a, a vasculopathy that

50:22

may manifest by areas

50:23

of narrowing intracranial neurofibromatosis.

50:28

Remember that neurofibromatosis may be associated with

50:31

internal carotid artery aplasia or hypoplasia,

50:35

and that actually is one of the other manifestations

50:38

or etiologies for Moya Moya syndrome affecting a unilateral

50:44

internal carotid artery.

50:45

Here you see the, um,

50:47

normal carotid artery on the left side, the absence

50:50

of the carotid artery on the right side.

50:52

And the giveaway here is that the patient also has

50:56

sphenoid wing dysplasia, which is one

50:58

of the other manifestations of NF one,

51:00

and we don't really see a, a blood vessel here.

51:03

This patient may develop moyo as well.

51:06

Finally, the last entity I wanna mention is the amyloid beta

51:11

related angiitis.

51:13

This is a form of

51:16

cerebral amyloid angio angiopathy,

51:19

but with an autoimmune inflammatory component

51:23

that targets the amyloid in the blood vessel wall.

51:26

Remember I initially talked about amyloid angiopathy

51:29

as a vasculopathy without the vasculitis.

51:32

However, there is a form of this

51:36

that may be an inflammatory vasculitis,

51:39

the amyloid beta related angiitis, it's, um,

51:44

one of the manifestations

51:45

that may be fatal if not diagnosed,

51:47

because it's usually late diagnosis.

51:49

People don't understand that there is this component

51:52

of in inflammation that has to be treated with the

51:56

anti-inflammatory, uh, drugs including steroids,

52:00

for example, immunosuppression.

52:02

And in addition to the cerebral amyloid angiopathy findings,

52:07

and with strokes edema, you will see a more, uh, common

52:11

leptomeningeal enhancement, which may be the tip off

52:15

that this is not your run of the mill ca a a,

52:19

this is the amyloid beta related angiitis.

52:25

And here's a, uh, a example from the AJ

52:29

and R with, uh, multiple small strokes as well

52:33

as the meningeal enhancement that was associated with the

52:37

occult amyloid beta related angiitis of,

52:42

um, amyloid angiopathy.

52:45

So I'll, I'll end with this, uh, final slide.

52:48

We've covered a lot of these entities, some

52:50

of which we would characterize as vasculitis.

52:53

Some of these we characterize

52:54

as a vasculopathy without necessarily a

52:59

dominant inflammatory component

53:01

that would define a vasculitis.

53:03

But I think that, um, we've given, gotten a nice overview

53:07

of a relatively obscure topic,

53:09

and I hope you learned something from it.

53:12

So with that, I am going to stop my share,

53:16

show my, uh, happy, um, face

53:20

and go to the question and answer.

53:23

So, uh, one of the persons asked,

53:25

what exactly is black blood imaging?

53:28

So black blood imaging is a technique in which the flow

53:33

of the blood is dark.

53:36

And we look at it on T two Wade, T one wade,

53:40

and post gad t one wade to look at the vessel wall.

53:44

So it's vessel wall imaging.

53:46

You may see VWI or, uh,

53:49

and um, this is the idea

53:54

that stenosis

53:55

and degree of narrowing is not the wherewithal

53:58

of risk factors for stroke.

54:00

That at least in the neck, for example, you, you know, um,

54:05

it's not just the degree of narrowing by NASA or ECAs

54:08

or whatever criteria that you may use.

54:11

It's also the quality

54:13

and characteristic of the plaque,

54:15

which may be predisposing to stroke.

54:18

So, uh, if you look at any of Bruce Wasserman's papers,

54:23

including techniques, you'll see how it is that you have

54:27

to do multiple suppression pulses back

54:29

and forth, inversion pulses in order to eliminate that,

54:34

that flow ghosting artifact

54:36

or the flow artifacts in order to get it

54:39

as a black blood vessel to see the wall that much better.

54:43

At what time after contrast enhancement do you scan the

54:46

patient in order to best depict vessel wall enhancement?

54:50

We are, uh, we are, um, we are doing two sequences

54:54

after the contrast enhancement,

54:57

but we are not delaying the con uh, the imaging

55:02

after the injection.

55:04

Uh, how do you differentiate

55:05

between intra plaque hemorrhage versus thrombosis?

55:10

So with intra plaque hemorrhage, the location of

55:13

that blood product is different.

55:14

Obviously it's in the plaque,

55:17

outside the lumen of the vessel.

55:19

With luminal thrombosis, you're not seeing flow.

55:23

In most cases it can be a umin,

55:27

a non occlusive thrombus.

55:29

Um, but this is again,

55:30

much better visualized on the black blood imaging

55:33

where you see the lumen as the black area

55:35

and the wall of the vessel.

55:38

Often on contrast enhancement

55:40

because of the fibrous cap,

55:41

you can actually see the enhancing wall of the intima.

55:45

So, um, that's, uh, that's how we do that.

55:49

Um, what is your experience with vessel wall imaging

55:52

and the stenosis commonly seen in press?

55:57

So I will admit that we don't, um,

56:01

we don't usually do vessel wall imaging in patients

56:03

with press.

56:04

It's known to be a type of vasculopathy,

56:08

but I don't have experience with stenosis in press.

56:13

Um, we often do not give

56:18

contrast enhancement in the press cases as well,

56:21

so we might not see that

56:23

because we're not doing a, um, an enhanced scan.

56:27

Um, with press you may see contrast enhancement in the

56:30

parenchyma that occurs in about 10

56:32

to 15% of patients who have press.

56:34

We may see, um, cortical or juxta cortical enhancement.

56:38

Um, 10 to 15% of patients

56:41

with press will also have DWI positive ischemic injuries

56:45

that, um, are, you know, basically strokes.

56:48

Okay. Next question. In your experience, what is sensitivity

56:51

and specificity in black blood

56:53

MRI in the diagnosis of vasculitis?

56:55

It is, I think a very much, uh, entity

57:01

dependent, um, at Hopkins for consideration

57:05

of primary angios of the central nervous system.

57:08

It is done routinely,

57:11

and I would say that we see something,

57:13

be it the mening G enhancement

57:15

or the vessel wall enhancement,

57:16

or, you know, even we're doing a lot of patients

57:19

with giant cell arteritis and superficial temporal arteries.

57:22

Um, I would say about three fourths of the time,

57:25

but I will, um, I'm not sure whether Bruce is on the, uh,

57:28

on the lecture or y cow is on the lecture.

57:30

If so, please, uh, give us your, um,

57:33

overall experience about, um,

57:36

black butt imaging and vasculitis.

57:38

Some of the entities, particularly if they're very small,

57:40

the small vessel variety,

57:42

usually we do not see enhancement in the

57:46

larger medium vessels.

57:47

Maybe we see the meningeal enhancement with diffusion weight

57:51

and, uh, diffusion weight.

57:52

And MRI have a role in the diagnosis of vasculitis.

57:56

Yes, obviously, a lot

57:58

of times the patient's manifestation is a neurologic deficit

58:01

for which we are doing diffusion weight, uh, scans.

58:05

And for example, in patients with P-A-C-N-S,

58:09

the most common thing that we're seeing is ischemic injury

58:12

of different ages on the

58:15

conventional MRI more so than, uh, the black blood imaging.

58:20

Um, if a patient comes in for an MRI scan for P-A-C-N-S,

58:24

we initially do the, um, plain MRI with, with gadolinium,

58:29

maybe we'll get lucky in seeing the vessel,

58:31

but they get a separate vessel wall imaging study at a

58:36

later date with often with surface coils to look at, um,

58:40

you know, the vessels that in the periphery.

58:43

Um, so yeah, you,

58:46

you vasculitis obviously the risk of vasculitis is

58:50

thrombotic events, embolic events,

58:52

and primary vascular events leading to stroke.

58:55

So we do DWI, uh,

58:57

have you seen any vasculitis cases missed at three T

59:00

and seen at 17?

59:02

So I'll answer that kind of, um, obliquely,

59:06

and that is with regard to multiple sclerosis.

59:10

So we know that multiple sclerosis is a per

59:15

disease entity

59:17

and we rarely will see the per ular nature

59:23

of multiple sclerosis on three T.

59:26

But when you do 70 imaging

59:29

and you see those veins so well, you can see

59:33

that MS is eccentric around those veins

59:35

and as a per ular demyelinating disorder.

59:38

So I believe

59:39

that 70 T would be very helpful in the evaluation

59:42

of patients with, uh, vasculitis in,

59:46

in enabling us to see the enhancement in the vessel wall

59:49

and those vessel walls

59:51

and maybe even some of those entities that I mentioned

59:54

that may be on the venous side, particularly, uh,

59:57

lupus particularly.

59:59

Um, sarcoidosis it may be very useful in, in that setting.

60:04

Uh, and we do have a seven T scanner,

60:06

but it's a research scanner, um, that's, uh, run by our, our

60:11

FM Kirby Center, uh, headed by,

60:15

um, Peter Benzel.

60:17

Um, if, if a 60, 70-year-old

60:19

with multiple T two hypotensive foci in the subcortical,

60:22

can we exclude angio amyloid angiopathy?

60:24

No, not at all. Um, if we see small micro hemorrhages

60:29

in the periphery, subcortical white matter, whether it's

60:33

with or without a cortical involvement or low bar hemorrhage

60:36

or with or without hem cirrhosis,

60:39

definitely amyloid angiopathy is in the

60:41

differential diagnosis.

60:43

The first thing we do is we look at the atherosclerotic risk

60:46

factors and the EMR

60:48

and see whether the patient is hypertensive,

60:50

well controlled, not well controlled bullying

60:52

and hypertension, because frankly, hypertensive

60:57

patients are much more common than patients

60:59

with amyloid angiopathy.

61:01

So we would still include that

61:05

as more likely in the differential diagnosis.

61:07

If you then add a few more hemorrhages in the basal ganglia,

61:10

thalamus, brainstem,

61:12

that pushes us even more strongly towards hypertension

61:16

as opposed to amyloid angiopathy where

61:19

sometimes those deep gray matter structures are spared, um,

61:26

scrolling for routine intracranial RAs

61:29

of the circle of voice vessels.

61:30

Do you do post contrast imaging

61:31

or do you confined compliant cases

61:32

where vasculopathy is suspected?

61:35

So, um, we are doing non-contrast MRA,

61:40

where the vast majority of those patients who have, um,

61:46

who have circle of voice MRA, the instances

61:49

where we will give the post the contrast are those

61:53

specifically for vasculopath, these vasculitis cases.

61:57

And also in some of the cases

61:59

where we're looking at stented vessels, uh,

62:03

for residual aneurysm, we, we think that giving the contrast

62:07

actually helps us see whether stented vessels are patent

62:10

or not, and whether

62:12

or not there's little areas of contrast enhancement blebs

62:15

that you might not see on the non-contrast scan.

62:18

Certainly for anything out in the periphery of AVMs,

62:21

et cetera, a lot of times giving the GAD for the peripheral

62:27

MRA is very helpful.

62:29

And on the venous side, frankly, I I think

62:32

that doing non-contrast MR venography is worthless.

62:36

I mean, there's so many examples where we've seen the veins

62:41

that were thought to be narrowed on the non-contrast.

62:44

MRV just, you know, show normal enhancement

62:48

and normal caliber on the enhanced MRV.

62:53

So I would recommend if you're doing non-contrast, MRVI,

62:56

I would just completely eliminate it

62:58

unless the patient's, you know, GA allergic.

63:02

Okay. Uh, our CVS, which is the preferred modality, CTA

63:07

or MRI with vessel wall imaging, um,

63:10

because the patients usually are manifesting

63:13

with a thunder thunderclap headache,

63:15

the differential diagnosis is usually an aneurysm and,

63:18

and an aneurysm that bleeds.

63:21

So the vast majority of patients are seen in the ed,

63:24

they get a non-contrast CT to look

63:27

for subarachnoid hemorrhage

63:28

because of this severe thunderclap abrupt headache.

63:30

And in the scanner they are then lead to CTA.

63:35

So in my opinion, for the peripheral vessels

63:38

and for vasospasm in particular, I think CTA is superior

63:43

to MRA, um, it shows those peripheral vessels better,

63:46

in my opinion, which is more sensitive in temporal arteritis

63:51

PET CT or MRI

63:54

or better PET MRI don't have, uh, experience with that.

63:59

Um, if any of the people in the audience are using PET MRI

64:03

and to look at art arrays, by all means, uh, weigh in here.

64:07

I don't know whether elgin's on my, um, the head

64:10

of nuclear medicine now at, uh, Cleveland Clinic's on,

64:12

but, uh, maybe she can give an opinion,

64:14

don't have experience with that.

64:16

Sorry. Is time of flight imaging enough

64:19

to exclude stenosis?

64:22

Um, I'm not sure whether you're talking about in the neck

64:25

or intracranial.

64:27

Um, you know, our protocol is

64:31

to do a non-contrast 3D time of flight, uh,

64:35

through the carotid bifurcation,

64:38

just looking at the bifurcation with non-contrast.

64:41

And then we do the contrast enhanced, um, MRA

64:46

of the, uh, neck, uh, to look at those,

64:51

uh, blood vessels both at the aortic arch as well as

64:55

to re-look at the, uh, carotid bifurcations.

64:58

And I usually take whatever is worse between the enhanced

65:02

bifurcation or the non enhanced high resolution bifurcation.

65:06

I usually report the, the worst looking of it.

65:09

Um, there's reasons why one would be better than the other.

65:13

Um, um, which gets into the techniques

65:17

of MRA, the, um,

65:20

that I don't wanna necessarily get into at this point,

65:22

what is the most sensitive gold standard imaging

65:24

for the diagnosis of vasculitis?

65:27

Um, so conventional arteriography is,

65:31

I would say is still the gold standard, um,

65:35

with the caveat that the vessel wall imaging enhancement,

65:40

um, sometimes we'll find areas

65:44

of inflammation in the blood vessel where it has not

65:47

manifested as narrowing.

65:50

So that's sort of the argument for doing

65:53

black blood imaging, vessel wall imaging is that sometimes

65:57

as with, uh, atherosclerotic disease, it's not all

66:01

that narrowed, but there's a lot going on in the wall

66:04

and that's why we want to wouldn want to do it.

66:07

So, um, i I think that there's reasons to do both.

66:11

Uh, where, where in which segments is the sno located

66:15

in RCVS?

66:16

So usually it's out in the peripheral vessels.

66:19

It's not in the carotid or the proximal vessels.

66:22

Usually the manifestations that I've seen have been in the,

66:26

um, for the example I sh I showed you example

66:28

of the peric cosal art artery, the cosal marginal

66:30

and the, um, more peripheral

66:32

of the middle cerebral artery vessels rather than centrally.

66:36

Uh, which non-contrast MRV techniques do you commonly use

66:39

such as time of flight or, or phase contrast?

66:42

So, as I said, I'm not a big fan of non-contrast, MRV time

66:46

of flight, if there is a bright

66:51

clot, remember the time of flight

66:55

is a T one weighted sequence.

66:57

So if you have, for example,

67:00

superior sagittal sinus thrombosis with

67:03

met hemoglobin bright clot, when you do your time of flight,

67:07

MRV, it's gonna look like it's flowing

67:10

because the bright cot will be superimposed on flowing blood

67:16

and will look like it's open.

67:18

So in those settings is

67:20

where we use face contrast MRV biography when we have the

67:24

suspicion or we've seen on the pre

67:27

MRVA bright signal in the blood vessel

67:31

because you're gonna mistake it for flowing vessel when in,

67:34

in point of fact it's the me hemoglobin of the cut.

67:39

Do you find ultrasound better useful

67:41

and the initial modality for temporal rise workup

67:43

or proceed right with the MRI, with contrast,

67:48

not a person who does ultrasound

67:49

or interprets ultrasound, um,

67:54

I am not sure of the value of, um, ultrasound

67:58

for vasculitis or temporary rise workup.

68:01

I will defer to probably some of you, the European

68:05

and Indian and Asian individuals

68:08

who have a lot more experience with, uh, ultrasound of the,

68:11

of the vasculitis than I do.

68:13

Sorry, can't answer that.

68:15

Which sequences do you use

68:17

for extra cranial and vessel wall imaging?

68:19

So I don't wanna, um, you know, s um,

68:25

I, I don't wanna superimpose over Bruce's,

68:27

um, area of interest.

68:30

Just look up Bruce Wasserman

68:32

and, um, vessel wall imaging,

68:36

and you will see he has developed over the course of time

68:39

multiple post sequences that have evolved.

68:42

And, um, again, a lot of them revol re revolve

68:46

around inversion, reco,

68:47

multiple inversion recovery post sequences in order

68:50

to suppress the wall, the,

68:53

the flow in the wall in the, in the lumen in order

68:57

to best see the, um, the wall.

69:01

All right. There's some chat stuff. I'm not, oh, okay.

69:05

So there's some chat questions.

69:06

I'm, uh, I'm willing to run over if people don't mind it.

69:09

Let me see where the check goes.

69:12

Uh, looking to, okay.

69:17

Vasculitis, in your experience, how sensitive

69:19

and specific is vest wall imaging in the diagnosis

69:21

of vasculitis?

69:23

Any tips on how to improve the specificity?

69:26

Um, you know, a lot of these all look alike.

69:28

I'm not sure that other than the size of the vessel

69:33

and looking at that nomenclature,

69:34

whether we're gonna be able to say that this represents,

69:38

you know, um, polyangiitis, um,

69:43

you know, granulomatous polyangiitis as opposed to, um,

69:47

primary S vasculitis.

69:49

So, um, not that specific.

69:52

Um, with regard to the different vasculitis, big sale,

69:57

remember to check your thing.

69:58

How angio centric lymphoma is different than

70:01

small vessel vasculitis.

70:02

Uh, the cases that I've seen, the, the,

70:05

the enhancement in lymphoma is, is much more dramatic.

70:08

You have much more parenchymal involvement

70:11

that may also show contrast enhancement in addition

70:14

to the vascular involvement as opposed

70:16

to small vessel vasculitis.

70:18

Where, where frankly we don't see all that often, uh,

70:21

the central, um, brain involvement.

70:25

Good event brain talk aside

70:27

to assess the cerebral venous sciences,

70:29

what would you recommend MRV

70:31

or CTV, uh, depending upon where you are,

70:35

um, I would not move an ED patient that you're evaluating

70:39

with CT to, uh, to move them to,

70:43

in order to do the MRV.

70:45

If you're doing the CT non-contrast

70:48

and there's a concern for, you know,

70:51

be it idiopathic intracranial hypertension,

70:53

just do the CTV in the same setting rather

70:56

than moving the patient around.

70:57

And I think that they are pretty much equivalent CTV

71:01

and MRV as long as you're doing untrust

71:04

and an M rv, don't do the non-contrast.

71:07

Uh, is angio and intravascular lymphoma different or same?

71:11

The same thing. Where do I find stenosis in SVCR?

71:18

Not sure I know what SVCR stands for.

71:20

Um, gimme another chance.

71:22

Is diffusion imaging reliable within an area

71:24

of microvascular change in the white matter?

71:28

It's the best thing we have.

71:29

If you're looking for white matter ischemic in injury,

71:33

it's the best thing we have.

71:34

So we do it. Um, are there patients

71:36

who have white matter disease without DWI findings

71:39

every single day?

71:40

Almost every single 65 and over person. All right.

71:45

I think that I,

71:48

I nailed 'em all in 11 minutes and I think

71:51

You got through like 20 questions

71:53

in that, that time period. So that was amazing.

71:56

Okay. Um, I am available by email,

71:59

do you someone at JU

72:01

for johns hopkins university.edu if you have

72:03

additional questions.

72:05

And, um, I do recommend the absolutely wonderful course

72:09

that Pam Schafer did on stroke in which she does cover some

72:12

of the vasculopaths

72:14

and also the beautiful course that, uh,

72:16

Francis t did does on profusion imaging and stroke.

72:20

So absolutely fantastic lecturers, great educators,

72:24

and, uh, probably the next step after this, uh,

72:28

after this, uh, noon conference.

72:30

Thanks for that plug on those awesome courses, Dr.

72:33

Ssim, and thanks for such a great lecture today.

72:35

We really appreciate you being here.

72:37

My pleasure. Happy Thanksgiving to everybody.

72:41

Um, and thank you so much for the learners

72:43

for participating in our noon conference

72:44

and asking such great questions.

72:47

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72:49

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72:50

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72:52

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72:55

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72:57

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72:59

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73:02

Strategies to Optimize Workflow Efficiency

73:04

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73:06

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73:09

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73:12

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