0:00

This is a 52-year-old woman with intractable

0:04

Parkinson's disease that did not respond to

0:07

typical dopaminergic medications like Sinemet.

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Let's scroll her in the axial projection.

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She's got a known Parkinson syndrome.

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Going to make it a little bigger for you

0:21

so you can see the midbrain.

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And let's see if she's got a preserved

0:26

compacta stripe.

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And she does.

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You see the red nucleus separate from the iron

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stores in the rest of the substantia nigra

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with a preserved compacta stripe in between

0:38

with the two not bleeding together

0:41

as they would in somebody with

0:42

a severe Parkinsonian syndrome.

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So what are we going to do?

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How are we going to look at this image?

0:50

How are we going to attack this case?

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Well, first, let's go up and look at the cortex.

0:55

Let's see what kind of atrophy she has.

0:57

Not much.

0:59

In fact, very little atrophy.

1:01

And the reason I want to go upstairs and look there

1:04

is because I'm going down my checklist

1:06

of Parkinson-like syndromes,

1:08

assuming that there's no medication

1:12

that's causing this,

1:12

because medication can cause

1:14

a Parkinson-like syndrome too.

1:15

So what am I looking for up high?

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Atrophy?

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Especially asymmetric atrophy as might occur in

1:23

corticobasal degeneration doesn't have it.

1:26

So, now, let's go down low.

1:28

Let's look at the temporal horns.

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They're a little bit dilated,

1:33

but the parahippocampi look pretty juicy.

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Let's pull down a coronal

1:39

and see just how juicy they do look.

1:42

They're a little bit atrophic.

1:44

The choroidal fissures are a little big.

1:46

The horns, the temporal horns are a little big.

1:49

So I would include,

1:50

in my differential diagnosis here,

1:53

Lewy body dementia.

1:54

However, I would not include Alzheimer's disease,

1:57

even though the entorhinal cortex

1:59

is somewhat atrophic,

2:01

because this patient is presenting with a severe

2:03

intractable Parkinson's-like syndrome.

2:06

So it doesn't fit historically.

2:09

In addition,

2:10

I don't really have a lot of parietal atrophy,

2:12

which you see in patients with

2:15

Alzheimer's disease.

2:16

Most of the atrophy that I'm seeing that is

2:18

cortically based is sylvian and frontal.

2:21

So that doesn't fit very well.

2:23

Another condition associated with a Parkinson-like

2:27

movement disorder is frontotemporal dementia.

2:30

Now, that one would remain in the

2:32

differential diagnosis,

2:34

except that these patients have a lot of

2:36

behavioral disturbance, dysexecutive function.

2:39

In other words,

2:40

they do things that are ill-mannered.

2:43

They lose their inhibition.

2:44

They do have frontal atrophy,

2:46

which this patient has.

2:47

They do have temporal atrophy,

2:49

which this patient has.

2:50

And they do have a Parkinsonian-like syndrome.

2:53

So that remains of the differential diagnosis,

2:56

but the history goes against it.

2:58

In other words, the patient doesn't have

3:00

this detached, dysexecutive, ill-mannered behavior.

3:04

So clinically, this helps you a lot.

3:06

Another aspect of this case that goes

3:09

against Lewy Body Dementia,

3:11

which is strongly associated with

3:12

a Parkinson-like presentation,

3:14

is the absence of visual hallucinations.

3:17

Now, another disorder that you would consider is

3:21

progressive supranuclear palsy.

3:22

So I'm listing,

3:23

I'm going right down the list

3:24

of Parkinson's plus syndromes.

3:27

So, let's talk about PSP.

3:28

I'm going to blow up the brainstem for a minute.

3:31

The cerebellum is not very atrophic.

3:34

If we look at the brainstem in the midline,

3:37

it looks like the colliculus is atrophic,

3:40

but it's not.

3:41

You're just in the sulcus.

3:42

If you go off to the side, it's nice and juicy.

3:45

If you go off to the other side,

3:47

it's nice and juicy.

3:48

And the superior colliculus

3:49

is as big as the inferior colliculus.

3:52

Therefore, you do not have the diagnosis of PSP,

3:57

Steele Richardson-Olszewski syndrome

4:00

or progressive supranuclear palsy.

4:03

So, now, I'm down on my luck.

4:05

I'm down on my list.

4:06

I'm down to MSAP,

4:08

multi-system atrophy Parkinson's type.

4:12

There are three basic types of MSA.

4:15

There's MSAA,

4:17

autonomic dysfunction.

4:18

Patients have trouble standing in one place.

4:20

They get hypotensive.

4:22

Also known as the shy-drager variant.

4:26

You have MSAC

4:28

where there is severe pontocerebellar atrophy

4:32

associated with the hot cross bun sign,

4:35

and then you have individuals that have MSAP,

4:39

where they have a Parkinson's-like syndrome.

4:42

The atrophy is very nonspecific,

4:44

although it can involve the pyramidal and / or motor system,

4:48

it can involve the frontotemporal region.

4:51

In fact, it can be just about anywhere.

4:53

So your differential diagnosis would come down to,

4:57

in this case,

4:58

frontolobar dementia or frontal lobar disease,

5:02

also known as Pick's disease, and MSAP,

5:06

with Lewy Body Dementia being a less likely

5:08

consideration due to the history.

5:11

And unfortunately, in this case,

5:14

the patient succumbed and was found to have MSAP,

5:18

Multi-system atrophy Parkinson's type.