0:00

Dr. Laser,

0:01

we're back with our 59-year-old woman

0:03

who's got profound frontal atrophy,

0:05

left greater than right.

0:06

As you mentioned before, knife blade atrophy,

0:10

atrophy of the temporal region, pretty profound,

0:13

but relative sparing of the parietal region.

0:15

And then, we also said,

0:17

in the coronal projection,

0:19

that the patient had relative sparing of

0:21

the entorhinal cortex,

0:22

which militates heavily against the diagnosis

0:24

of ALZ or Alzheimer's disease.

0:26

And sparing of this is a cardinal

0:28

feature of Pick's disease.

0:30

We also pointed out that there is profound atrophy of

0:33

the temporal lobe, may spare the front or the tip,

0:36

but extends back, and also in this case,

0:39

involves the left side or Broca's area.

0:41

Broadmann areas 44 and 45 are affected,

0:45

so it's not surprising if she has a speech disturbance.

0:48

So, I want to take a moment to talk about

0:49

the different types of Pick's disease.

0:52

You can have frontotemporal dementia

0:55

with a Parkinson-like syndrome,

0:57

and this is one of several forms of Pick's disease

1:00

that may be related to chromosome 17.

1:03

In fact, FTDP-17T is the chromosomal linkage.

1:09

Then you've got the frontotemporal lobar degeneration with

1:13

ubiquitin-positive and tau-negative inclusion,

1:15

so-called frontotemporal lobar dementia,

1:18

FTLD-U.

1:20

And then, you've got frontotemporal lobar dementia

1:23

with motor neuron disease,

1:24

FTLD-MND.

1:28

And then finally, you have frontotemporal lobar

1:30

dementia without ubiquitin or tau deposits,

1:33

dementia lacking distinctive histology.

1:35

So, there are at least four or more subtypes

1:38

of Pick's disease.

1:39

This is a little bit like multisystem atrophy,

1:41

where you divide it up into autonomic dysfunction,

1:44

Parkinson's disease,

1:46

motor affectation, etc.

1:49

Dr. Laser, this is a tauopathy,

1:51

and tau inclusions occur

1:53

in several neurodegenerative diseases.

1:55

The classic one is Alzheimer's disease,

1:57

but frontotemporal dementia, FTD,

2:00

or frontotemporal lobar dementia is another one,

2:03

progressive supranuclear palsy,

2:05

and the fourth major one is corticobasal degeneration.

2:07

Now,

2:08

mutations in tau cause

2:10

some familial frontotemporal dementias,

2:13

of which Pick's disease is one.

2:15

So, what percent of individuals have

2:17

a heredofamilial predilection for Pick's?

2:19

It's about 30% to 40%.

2:21

That is extremely scary.

2:22

Yeah.

2:23

And the onset of Pick's is usually a little

2:24

earlier than Alzheimer's disease.

2:26

There is a familial type of Alzheimer's disease

2:29

that we see in younger individuals.

2:32

And the heredofamilial incidence

2:35

is probably around 10% to 15%.

2:36

So not as strong as it is with Pick's disease,

2:39

which is a scary thought.

2:40

Shall we move on?

2:41

Let's.