0:00

This was a patient who had AIDS and was

0:05

initially diagnosed with progressive

0:08

multifocal leukoencephalopathy.

0:10

The initial scan is from August 19, 2013.

0:16

As we look at the FLAIR scan,

0:18

we can see that there is abnormal signal

0:21

intensity in the splenium of the corpus callosum,

0:24

which crosses from right to left.

0:26

We note the involvement of the subcortical U fibers.

0:32

So on this FLAIR scan,

0:34

you can see the involvement going all

0:36

the way out to the periphery,

0:38

which is typical of U fiber involvement with PML.

0:48

Now, there are additional smaller lesions,

0:52

such as this one seen in the subcortical

0:55

subinsular white matter and external

0:58

capsule region on the right side,

1:00

as well as more peripherally

1:03

in the parietal lobe.

1:08

The T2-weighted scan also shows similar

1:11

findings and you note that the lesion does not

1:14

show contrast enhancement on the postgad

1:18

T1-weighted scan here below the FLAIR scan,

1:22

and there is no restriction of

1:24

diffusion on the ADC map.

1:27

The next scan that the patient

1:29

had was from October.

1:31

I'm just going to pull down the

1:35

postgadolinium and FLAIR scans.

1:40

On this October scan,

1:43

you can see that, if anything,

1:46

things have progressed.

1:48

The lesion is larger.

1:50

It remains

1:54

extending to the subcortical U fibers,

1:58

out to the periphery,

2:01

in fact, bilaterally,

2:03

and now is extending even to the optic

2:06

radiations of the temporal lobe.

2:10

The splenium of the corpus callosum actually looks

2:13

a little bit wider and there is

2:16

a little bit of mass effect.

2:18

On postgadolinium-enhanced imaging,

2:22

you can see that there is just faint

2:25

areas of contrast enhancement.

2:29

Here is a demonstration of minimal contrast

2:33

enhancement within the demyelinating process.

2:36

However,

2:37

it can be seen on other such images

2:42

further inferiorly in the periatrial

2:46

region on the right side.

2:53

So, this is a patient whose

2:55

disease is progressive.

3:00

Let's continue to watch this patient over time.

3:02

The patient next gets a scan on December 13, 2013.

3:10

Oh, my goodness.

3:11

This is enlarging into the frontal

3:16

lobe.

3:17

It's extending via the white matter

3:19

tracts into the brain stem.

3:22

The left side is much more

3:25

involved than previously.

3:28

What's going on?

3:30

Let's see what happened on

3:31

the post-contrast scan.

3:35

Oh, my goodness.

3:36

You have avid contrast enhancement.

3:39

The patient's obviously not cooperating,

3:41

and all of a sudden,

3:42

your PML is showing dramatic contrast enhancement.

3:46

What happened?

3:48

Well, what happened between the August study

3:50

and the December study

3:52

was that the patient had

3:53

initiated the HAART,

3:56

the antiretroviral therapy.

4:00

And for whatever reason,

4:02

the patient's PML has dramatically gotten worse,

4:07

rather than what one would have expected

4:09

with it getting better.

4:11

This is the entity known as IRIS,

4:16

Immune Reconstitution Inflammatory Syndrome.

4:21

It is effectively

4:24

PML on steroids, only not really.

4:27

This is PML with marked inflammation,

4:30

which is aggravated by the institution

4:33

of the HAART therapy,

4:35

which leads to jazzing up of the immune response

4:40

such that it overreacts to the PML.

4:43

And in point effect,

4:44

you get abnormal enhancement, mass effect,

4:47

growth in the lesion.

4:49

What can you do?

4:51

Well, at this point,

4:52

the best alternative is to stop the HAART therapy

4:57

and try other medications to relieve

5:01

the patient's AIDS, which was, in fact,

5:03

what was done.

5:04

And we have a follow-up examination,

5:08

somewhat more remote,

5:10

but showing you the residual of what happened.

5:13

So here we have a study from March 5, 2017,

5:17

and you see the remainder of what was the IRIS,

5:24

that is, atrophy,

5:27

enlargement of the ventricles,

5:30

gliosis in the white matter,

5:33

sulcal enlargement showing the volume loss,

5:36

but no mass effect,

5:39

decreased volume of disease,

5:41

and in point of fact,

5:43

absence of contrast enhancement.

5:46

So, the patient actually responded well to the

5:51

withdrawal of the HAART therapy,

5:55

leading to

5:57

still residual disease,

6:00

as far as the sequela of IRIS,

6:04

Immune Reconstitution Inflammatory Syndrome

6:08

in a patient who has PML

6:11

with HAART institution

6:12

or other medications that jazz up and

6:16

reenergizes, if you will,

6:18

the inflammatory system,

6:20

the immune system in the body,

6:22

but it leads to overreaction and more

6:26

disease than the original PML.