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It's a complex case.

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A 52-year-old man who carries a diagnosis

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of multiple sclerosis.

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And yes,

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he does have extraordinary atrophy and signal

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throughout the colossal

0:12

and the colossal septal interface.

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As you go off to the side,

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you can see some areas of right angle demyelination,

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but he presents now with episodes of falling down.

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And while it's true,

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you can get extensive atrophy.

0:25

We see that in the cingulate sulcus

0:27

and supermarginal sulcus going up.

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You wouldn't get this severity posterior

0:33

fossa atrophy from MS alone.

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I mean, look at the size of his pons.

0:39

It is extremely small.

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His cerebellar vermis with the individual

0:43

vermian lobes are also small.

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You can see the prominence of the patient's

0:48

primary fissure right here and the posterolateral fissure.

0:51

Then we go down to the medulla.

0:54

We see the medullary clava as a landmark,

0:56

and then go to the pons and the midbrain.

0:59

And of the two,

1:00

the pons looks a bit smaller than the midbrain.

1:03

But one of the differential diagnostic

1:05

considerations here would be progressive

1:07

supranuclear palsy.

1:09

And you look at the inferior colliculus,

1:11

you see it's got a pretty good bump.

1:12

And as you go off to the side,

1:14

the superior colliculus also has a pretty good bump.

1:17

They're almost equal.

1:19

So, PSP or progressive supranuclear palsy,

1:23

which, by the way,

1:24

is associated with gait disturbance

1:26

and paralysis of gaze,

1:28

which this patient doesn't have,

1:30

wouldn't be a favored diagnosis.

1:32

The severe pontine atrophy, though,

1:35

in the absence of posterolateral plaque formation,

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which is where you would get MS in the pons,

1:41

is a tip-off to the diagnosis.

1:43

And if we go down a little bit lower,

1:45

at the level of the brachium conjunctivum or

1:48

superior cerebellar peduncle,

1:50

it is very atrophic with this T-shaped area of

1:55

vertical high signal in the median pontine raphae

1:59

and a cross going from side to side,

2:02

from medial-lateral,

2:04

producing what's known as the hot cross bun sign

2:07

of this disorder, multisystem atrophy.

2:12

This patient does have atrophy,

2:14

and it's hard to tell what component of the

2:16

atrophy is related to chronic long-standing MS and

2:20

which component is related to this patient's MSA.

2:25

Now, MSA can be subdivided into three types.

2:29

The three types are MSAC,

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which affects the cerebellum,

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and the pons,

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also falling into the category

2:38

of olivopontocerebellar atrophy.

2:41

It's one of the olivopontocerebellar atrophies.

2:44

There are heredofamilial ones as well.

2:47

So, it's a subset of OPC disorders.

2:52

And these patients have severe ataxia and falling

2:55

down as their major manifestation.

2:58

The other types of Multisystem atrophy

3:01

are MSA, multisystem atrophy.

3:04

A, which stands for autonomic failure.

3:08

When they stand up, they get hypotension.

3:11

Sometimes when they stand,

3:12

they simply get tremors in their legs.

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They can't stand in one place, but they can walk,

3:17

which is very different than classic

3:19

Parkinson's disease,

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where walking is challenging due to the rigidity.

3:24

So when they stand still in Shy-Drager syndrome,

3:28

which is basically the autonomic type of MSA,

3:32

they have trouble staying in one place.

3:35

Then you've got the Parkinsonian variation, MSAP,

3:39

also known as Striatonigral variant of multisystem atrophy.

3:45

And this one may be very difficult to differentiate

3:48

from Parkinson's disease,

3:49

and doesn't respond very well

3:52

to the typical Sinemet and Parkinsonian preparations.

3:56

These patients often have a fair amount of

3:59

disturbance in their speech and laryngeal paresis.

4:04

Patients with MSA often have involvement

4:07

of the pyramids,

4:09

so there may be a combination of motor dysfunction

4:11

with some of these others.

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This patient's pyramids look pretty good.

4:16

Death is often related to bulbar dysfunction and dysphagia.

4:19

The patients often die from aspiration, pneumonia, or apnea.

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This patient manifests the hot cross bun sign,

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seen down low at the level of the pons of MSAC

4:31

with associated cerebellar atrophy,

4:34

atrophy of the brachium conjunctivum

4:37

or superior cerebellar peduncle,

4:39

and the cerebellar and pontine atrophy

4:42

conspicuous in the sagittal projection in

4:44

this patient who has concomitant MS.