0:00

Dr. Laser,

0:00

this is a 59-year-old woman with severe progressive

0:04

mental status change that they said began one year ago,

0:07

worsen over the last three months.

0:09

The timing, you know, is in question

0:10

because, of course, patients don't often report

0:12

that they have cognitive decline.

0:14

People from the outside recognize it.

0:16

So, probably been going on longer than that.

0:18

But we've got an axial T2 with some

0:21

rather profound frontal atrophy.

0:23

Not so much parietal involvement this time.

0:25

The ventricles aren't that big.

0:27

Here's a coronal FLAIR demonstrating some

0:29

periventricular disease and some white matter

0:32

scattered gliosis throughout the brain,

0:34

but not a prominent finding in this case.

0:36

What is a prominent finding is the profound frontal

0:39

atrophy that is worse on the left and right with

0:42

extensive prominence of the Sylvian fissures.

0:45

And then as we come forward,

0:47

all the way into the olfactory area,

0:50

that's atrophic too,

0:51

although not quite as severely affected.

0:53

But look at this pattern of atrophy,

0:54

which you would call what?

0:56

It has the...

0:57

The gyri have actually shrunken down and atrophied

1:00

in terms of like a sharp knife.

1:02

So it's called the knife blade.

1:03

Knife blade atrophy.

1:04

Atrophy.

1:05

All right.

1:05

And then we go over to the sagittal.

1:07

We go over into Broca's area,

1:09

and the normal letter M for Broca's area is distorted.

1:14

And the typical Broca area, Broadmann 44 and 45,

1:18

affected as well in this ascending portion, or ramus,

1:22

of the insula, is affected anteriorly.

1:25

And then there is extensive temporal atrophy,

1:29

although the tip is spared.

1:30

And we're going to look at the entorhinal cortex,

1:34

the meat of the hippocampus,

1:36

and that's not much affected either.

1:38

Look how good the choroidal fissures and temporal

1:40

horns look relative to everything else.

1:42

Granted they're not perfect,

1:43

but they're pretty good compared to the severity of

1:46

atrophy you have elsewhere.

1:47

So all of those findings,

1:48

hippocampal sparing, knife blade atrophy,

1:51

frontal predilection, frontotemporal involvement,

1:55

a little bit of olfactory involvement.

1:57

What does that suggest to you as a dementia?

2:00

Oh, with her symptoms,

2:01

frontotemporal,

2:02

low bar degeneration.

2:03

Yeah.

2:04

Formerly known as the artist pick's disease.

2:06

So, let's talk a little bit about Pick's disease.

2:09

It may involve the basal ganglia.

2:11

In this case, the basal ganglia,

2:12

when we scroll through them, looks pretty good, actually.

2:16

And pretty juicy-looking

2:18

globus pallidus and putamen.

2:20

We go over to the caudate nuclei.

2:23

They look pretty good, too,

2:24

so they're not really all that atrophic.

2:26

And so, basal ganglion involvement here scanned.

2:30

And then, what defines this disease is the presence

2:33

of specific inclusions containing tau,

2:37

such as TDP 43 or ubiquitin,

2:41

which allows biochemical classification of this disease.

2:45

It's noted for being an asymmetric disease,

2:47

and even though there's a fair amount of symmetry

2:49

in the temple region, as discussed previously,

2:52

there's quite a bit of asymmetry in the frontal region,

2:55

left greater than right.

2:56

Now, when the left is more affected,

2:58

what is the primary presentation?

2:59

Or what can be one of the major symptoms?

3:02

Typically, it's aphasia.

3:04

Semantic aphasia.

3:05

A semantic aphasia.

3:06

The meaning of words is lost.

3:08

And we've already seen that the Broca area is involved too,

3:11

so that's problematic.

3:13

When it's on the right side,

3:14

predominantly, they present quite a bit later.

3:17

They may complain of difficulty with facial recognition.

3:22

So this is an individual with Pick's disease,

3:24

demonstrating all of the cardinal findings.

3:28

In the next vignette, companion vignette,

3:30

we're going to talk about the subtypes of Pick's disease

3:32

and a little bit more about their presentation,

3:34

which includes something called the disexecutive syndrome.

3:37

You know,

3:38

when you eat with your feet at the dinner table?

3:39

Yeah. Loss of filter. Yeah.

3:40

But we both know what that's about.

3:42

Yeah.

3:42

Pomeranz and Laser out.