0:00

I'm Dr. Stephen Pomeranz.

0:02

He's Dr. Malcolm Schupeck.

0:04

We've got a nerdy radiologist and a neurosurgeon

0:07

and neuroimager together, looking at an

0:09

interesting case. Believe it or not,

0:12

it's a 29-year-old woman presents with a

0:15

history of an MVA. Following the MVA,

0:17

she had severe upper and lower back pain.

0:21

And we're going to start out with

0:23

the series of sagittal images.

0:26

There is also a sagittal water-weighted lumbar,

0:29

but it really isn't contributory.

0:30

So we've got a sagittal T2,

0:32

or actually a PD-spur,

0:34

which we're going to scroll, not a T2.

0:36

So it's fat-suppressed with SPGR

0:39

spare or special in the middle,

0:41

another fat suppression proton density sequence.

0:45

Also sagittal in the thoracic,

0:48

and I've got my arrow placed over where some areas

0:51

of increased signal intensity in the cord,

0:53

so they are intramedullary.

0:56

And then in the axial projection,

0:58

I've got a water-weighted

1:00

or water-emphasized series with an abnormality

1:04

along the lateral and posterior column of the

1:08

cord corresponding to this area at C4.

1:12

You can see it crosses the midline and

1:17

it's almost involving the entire cord at

1:20

this level. So-called holo cord lesion.

1:23

So the question is, is this related to trauma?

1:27

I mean, you're a neurosurgeon.

1:28

You've seen a lot of trauma.

1:29

Does this look like trauma?

1:30

Yeah,

1:33

I would say that this is kind of a

1:35

common thing you run into where

1:38

the history turns up something else.

1:41

Patient has occasion to come to imaging,

1:44

we find something other than what we expect.

1:46

No sclerosis, for one thing.

1:48

Right. Meaning?

1:48

If you were thinking of should probably see some

1:51

signal abnormality consistent with a traumatic

1:54

lesion. Right, sure. A little blood, right?

1:56

Little sclerosis or some med hemoglobin stain.

2:00

Don't have it. Well, and how.

2:01

Right? Spinal cord wide open.

2:03

How would you get a lesion like that?

2:06

What traumatic mechanism?

2:08

Whenever I think of trauma,

2:09

you have to have an aetiology.

2:11

You say, well, what mechanism created that lesion?

2:14

And this is really important when you get

2:16

into spine fractures, dislocations,

2:18

but you just look at that, there's no spur there.

2:22

Maybe if you had a big spur or a traumatic disc,

2:25

you could explain it,

2:26

but we don't have a good explanation.

2:27

So I think we're looking for something else.

2:29

And in a 29-year-old with multiple lesions,

2:33

including a posterior lateral distribution,

2:36

skip areas. Right? Meaning short segment.

2:39

We're going to have to start thinking

2:40

along another line.

2:42

Although the history to me is always

2:44

really important, in this case,

2:46

we have some findings which may even override

2:48

that the history completely misleading.

2:51

And I hear you loud and clear.

2:52

I mean,

2:53

to get this kind of cord abnormality from trauma

2:55

flexion, teardrop, extension teardrop, some.

2:59

In the back, some hemorrhage posteriorly,

3:02

a bi columnar lesion, something unstable,

3:06

a burst fracture has none of it.

3:08

She has none of it.

3:09

So it totally is illogical that this

3:12

could be related to trauma.

3:14

Both the direct and the indirect

3:15

signs don't support it.

3:16

So you have to move in another direction right now.

3:19

Also, I would also say that you should.

3:21

The patient got in a car accident.

3:23

A lot of reasons maybe to go.

3:24

And seek medical care.

3:25

If you talked to her personally and asked her.

3:28

A few questions, you might be moving along.

3:30

A different track as well.

3:32

For example,

3:32

Have you had numbness,

3:34

Have you had visual disturbances?

3:35

Any of those things?

3:36

Those questions might not have been asked.

3:38

Depending on the setting that she sought care in.

3:41

Sure. So you asked about visual disturbances,

3:43

I think for a reason and we're getting.

3:45

Together on this case rather acutely.

3:48

We haven't really discussed this case in any scale.

3:50

Like you would out in the audience that's.

3:53

Watching, looking at it fresh.

3:55

The one thing you would think.

3:57

About is Devic's disease.

3:59

Which classically in the past was considered.

4:02

A subset of multiple sclerosis.

4:05

Also called neuromyelitis optica.

4:08

But you'd want to investigate to see if she has.

4:10

Had visual symptoms in the past or concurrently.

4:14

Has visual symptoms. And I suspect she has.

4:17

Although we have no proof of that.

4:19

And the patients get lesions in the.

4:21

Cord and in the optic tracts.

4:22

Which you do see if you produce a high quality.

4:25

Fat suppressed.

4:27

Contrast-enhanced study of the optic pathway.

4:32

And you can even see them without giving contrast.

4:34

As you can see these without giving contrast.

4:37

Now, do you like this for Devic's?

4:39

From what you're seeing here?

4:41

I don't love it for Devic's.

4:43

But apparently it's a proven Devic's case.

4:47

The patient has

4:49

positive water channel aquaporin-4 antibodies.

4:53

So it has been proven Devic's is usually

4:56

a pretty aggressive process.

4:59

You know,

5:00

initially in the early stages of describing Devic's,

5:03

lectures I went to when I was much younger,

5:06

they said it was a benign form of multiple

5:08

sclerosis. We now know this not only to be untrue.

5:12

Most people believe it's not even a form of MS.

5:15

If it's Devic's disease,

5:17

and we've got some laboratory data to support it,

5:21

these patients have a higher incidence of

5:24

autoimmune conditions such as systemic lupus,

5:27

erythematosus, and Sjögren's disease. Now,

5:30

these used to be associated with MS,

5:32

but that's because Devic's was lumped into MS.

5:35

And since this was associated with Devic's,

5:38

the connection was made, well,

5:39

SLE and Sjögren's more common with MS.

5:42

But in fact, what it's more common with is Devic's,

5:45

which is its own disease.

5:47

And you do see what we call Devic's variant,

5:51

where they have cord lesions,

5:53

they have parotid lesions.

5:54

And they also have another finding

5:56

which is interesting.

5:58

They have.

5:59

A very acute interferon response,

6:02

and they also have high levels of interferon,

6:05

unlike MS patients,

6:07

who have lower levels of interferon and don't

6:10

have as brisk an interferon response.

6:12

So there's a lot of evidence pointing towards the

6:15

fact that this is a condition separate

6:18

and distinct from MS. Well,

6:20

the reason I ask you whether you like because

6:22

I would get this wrong, okay?

6:24

Because you got a couple of different

6:26

differentials, right? Meaning skip areas,

6:29

posterior, lateral, okay?

6:31

Devic's you usually think of in a different

6:34

differential. That is your longitudinally,

6:37

extensive cord lesion greater than three segments.

6:41

And that brings you into a whole different

6:43

differential, includes neuromyel, myelitis,

6:46

optica, or Devic's, but also other things sarcoid,

6:49

Sjögren's, but also dural, AVF.

6:52

Okay? So to me,

6:53

I would have got it wrong because I didn't have

6:55

my aquaporin machine with me at that time.

6:58

You don't walk around.

6:59

With a transistor aquaporin machine.

7:02

It's on order, but it's in the car trunk.

7:06

But I think that

7:08

it's proven.

7:10

But it's a really great case because it shows

7:13

the difference between those two diseases.

7:15

Because I wouldn't have called it Devic's.

7:17

Now, we could have looked in the brain.

7:18

Meaning, if the brain is negative, okay,

7:22

then we might start moving along that mind,

7:25

because usually the findings in the brain

7:27

are much less impressive for Devic's.

7:29

They're usually mostly in the cord.

7:31

But usually this is not a typical lesion,

7:33

at least in my experience.

7:34

Should be a long segment, big, impressive lesion.

7:38

These Skip lesions really look more like MS to me.

7:42

And as I say, we got a good demographic.

7:45

Which speaks more to the fact that this

7:48

is considered a different disease,

7:49

the fact that it's confluent.

7:51

Because typically MS, real MS,

7:54

is Skip lesions all the time, all day long.

7:57

It likes the posterior cord, as this does.

8:00

It likes the posterolateral column, as this does.

8:04

And to be honest, when I looked at it too,

8:06

I thought the same thing.

8:09

And then I got the information that we had,

8:11

the laboratory support and the

8:13

visual symptomatology.

8:15

So when you have three levels of involvement,

8:18

it does take you down a different path.

8:20

But here's something I always do.

8:22

If I've got multiple cord lesions,

8:25

one long cord lesion and a second lesion,

8:27

I still always think Devic's.

8:29

And I still go upstairs, I check the brain,

8:31

and I check the visual pathway anyway,

8:34

because everything doesn't always obey the rules.

8:37

So that's still in the back of my mind.

8:39

But I completely agree with you that this

8:41

is not a classic case of Devic's,

8:44

but yet we have laboratory proof.

8:46

And this condition of Devic's disease occurs more

8:50

frequently in people of Asian descent,

8:53

especially in Japanese.

8:55

And unlike its original description,

8:57

where it was thought to be kind

8:58

of a low grade version.

9:00

Of a demyelinating disease.

9:02

It's actually more frequently acute and severe.

9:05

It may be associated with necrosis and hemorrhage,

9:09

which may produce an overlap with ADEM,

9:11

acute disseminated encephalomyelitis,

9:14

which sometimes is hemorrhagic.

9:16

And it can occur both ways in the spine,

9:19

it can have a long segment of involvement

9:21

or it can have skip lesions like MS.

9:23

But when you get up into the brain,

9:25

the lesions are usually they're pretty big.

9:28

I'll use my drawing pen.

9:30

Even though we're not in the brain, they can be,

9:32

like this big and they often have a halo

9:36

around them when it's acute ADEM.

9:37

Now, granted, you get a halo sign, in other words,

9:40

a fried egg or halo sign with MS too.

9:43

But in my experience in acute disease,

9:45

I see it with greater frequency with

9:48

larger lesions. Ah, with ADEM.

9:50

Now,

9:51

one nice thing about ADEM maybe it's not so nice

9:54

is that it is almost always preceded by either

9:56

a vaccination or an upper respiratory

9:59

tract infection.

10:00

That is not true for multiple sclerosis.

10:04

What else would you consider a three level

10:07

involvement of the cord? Well,

10:09

there is

10:10

I mean,

10:12

I think the first thing is that comes to my mind

10:15

in these is how can the history help you?

10:17

And that in this case,

10:20

the trauma thing kind of overrides everything,

10:22

at least in the medical legal environment

10:24

many of us are in.

10:26

But

10:27

waxing and waning history,

10:30

if you had that meaning,

10:32

if you got on your history form,

10:33

waxing and waning getting worse, better,

10:35

you'd say MS. Okay?

10:36

Whereas Devic's is going to be a more abrupt,

10:40

acute progressive. Okay? Now,

10:42

other things in that differential, that is,

10:44

the longitudinal differential,

10:46

there are things like Sjögren's that you spoke

10:49

about and the Sjögren's variation

10:51

sorry to interrupt you,

10:52

but the Sjögren's variation lesions are very long,

10:55

just like you described with Devic's.

10:56

And some people consider this CNS Sjögren's.

11:00

Phenomenon to be a subset of Devic's disease.

11:03

And people with SLE get very long lesions.

11:05

So I'm sorry. Go ahead.

11:07

Well, the other thing is a cord lesion.

11:10

Dural AVF is in that long segment, and that is,

11:15

to me,

11:15

is the scariest one because it's something

11:19

that nobody knows about.

11:21

You have people walking around that are

11:22

getting progressively myelopathic.

11:24

Figure it out can be very subtle.

11:27

Now, this person here in this case,

11:29

so you could have a chronic progressive problem.

11:32

Middle-aged male would be the typical patient.

11:34

So that wouldn't help us here.

11:35

Yeah. More commonly males for Dural AVF,

11:38

more commonly females for Demyelination.

11:40

So that's helpful.

11:40

But a progressive lower extremity myelopathy.

11:44

And sometimes they'll even have back pain.

11:47

This patient had pain.

11:49

The demographic is different,

11:51

but Dural AVF is a cause of progressive

11:55

myelopathy that is missed.

11:57

And the big problem with that and the reason I

11:59

say. It's scary is because people sit on it,

12:04

and it's irreversible.

12:05

Meaning by the time the diagnosis often made,

12:07

there's nothing you can do because they undergo a

12:09

cord necrosis because of the AVF and

12:12

the increased venous pressure.

12:13

So when you see a long segment like

12:15

that often will enhance that's.

12:17

Definitely in the differential,

12:18

particularly if you're in that middle-aged male

12:21

to the lesser degree female demographic.

12:23

But progressive weakness,

12:25

because progressive weakness,

12:26

people have all kinds of ideas about it.

12:28

But when you see a report that history

12:30

that says progressive weakness,

12:32

do not attribute it to lumbar spinal stenosis,

12:34

hardly ever happens, right?

12:36

Meaning I've seen it once or twice.

12:37

That's a great claim. But, I mean,

12:38

if you see really tight lumbar spinal stenosis,

12:41

the history is gait disturbance weakness.

12:43

You got a problem. You have not explained it.

12:45

It's usually pain, right? That's pain.

12:47

And they're fine when they're laying down, okay?

12:49

It's when they stand and walk that they get pain.

12:52

They do not get weakness.

12:53

They do not get gait disturbance.

12:55

I mean, I might have seen it a couple of times,

12:57

but you got to exclude everything else.

12:59

You got to look at the rest.

13:00

Of the spine.

13:01

Let's talk about Dural AVF for a minute.

13:03

It comes in around T nine as the major,

13:05

major arterial feeder. So if you say okay,

13:08

that's T nine.

13:09

What you end up seeing is this very sort of hazy.

13:13

I'm even going to use the color gray because it's

13:16

so subtle. The cord is gray, the signal is gray,

13:19

but it's a slightly lighter gray.

13:21

And what happens is it progressively,

13:23

slowly you get this filmy, ill-defined,

13:27

progressively ascending.

13:28

And when I say progressively,

13:29

I mean over months to years,

13:31

progressively ascending signal within

13:34

the cord due to venous congestion.

13:36

And the actual Dural AVF shows up as tiny little

13:40

dots the size of a pinhead or smaller.

13:43

And unless you've done a small field of view MRI,

13:46

you will absolutely miss these or attribute them

13:49

to normal peel vessels in a published paper.

13:52

This diagnosis was missed on an average

13:55

five times. And in my experience,

13:57

that is absolutely true when I see them.

13:59

They've had.

14:00

Four or five prior MRIs that were read as

14:03

negative or were called other things.

14:06

So if, you know, if it goes on for years now,

14:09

you have a permanently damaged cord,

14:11

you basically have venous congestion,

14:13

a venous infarction,

14:14

and then sometimes they may go suddenly downhill.

14:17

If one of these veins I've blown it up.

14:19

One of these veins thrombosis and now

14:22

the venous congestion accelerates.

14:25

That's called subacute necrotizing myelopathy

14:28

or Foix-Alajouanine syndrome.

14:31

So they get acutely worse.

14:32

And what's the diagnosis that's given by the

14:34

radiologist myelitis secondary to an infection

14:38

because it comes on rather suddenly,

14:39

even though the patient has had progressive

14:42

ascending weakness and eventually

14:44

develops a sensory level.

14:46

So when you really drill into the history,

14:48

it doesn't fit.

14:49

But that that is really the most

14:51

important diagnosis and also

14:55

one of the few things that we have a curative

14:57

treatment for. Meaning if you make the.

14:59

Diagnosis and they have not gone too far.

15:02

You can stop it right there.

15:03

I mean, little laminectomy. Identify the fistula,

15:07

put an aneurysm clip on and you're done.

15:10

So there aren't too many things in neurosurgery

15:12

where we have that kind of a great treatment

15:14

option. And now there's endovascular options, too.

15:16

So a really important diagnosis.

15:18

And

15:19

in that pile of spine films you're going

15:22

to be reading over the next few years.

15:24

It's in there somewhere. Yeah,

15:26

the radiologist can really save the day

15:28

in that condition. And so, in summary,

15:31

then we've got a patient with a more classic

15:35

looking MS appearance with skip lesions.

15:38

At least one of the lesions is posterolateral and

15:40

posterior, although we do have an anterior lesion,

15:43

which is slightly atypical. We have skip lesions.

15:46

It's a woman. All of those things are good for MS,

15:50

but in this case,

15:51

the patient had the proper antibody profile.

15:55

Aquaporin four. She had visual symptomatology.

15:59

So this is Devic's disease.

16:01

It probably is its own classified entity.

16:04

And there is an autoimmune component that is

16:08

very closely linked with Sjogren's disease.

16:12

And those patients will have chronic parotitis.

16:15

And there is a very close linkage with systemic

16:17

lupus erythematosus. Let's move on, shall we?