0:00

This 75-year-old woman

0:02

with gait disturbance

0:03

and a gaze palsy,

0:05

with basically a movement problem.

0:07

Parkinsonian-like has progressive supranuclear palsy

0:11

or Steele-Richardson-Olszewski syndrome.

0:14

In another vignette,

0:15

I pointed out the preservation

0:17

of the Zona Compacta stripe,

0:19

which goes against the diagnosis of

0:22

classic Parkinson's disease.

0:24

Now, let's look at the brain stem,

0:26

especially the midbrain.

0:27

Look at how small the midbrain is sagittally.

0:31

It's not as easy to appreciate in the axial

0:33

projection, although in an earlier vignette,

0:35

I gave you the Mickey Mouse sign and the morning

0:38

glory sign to help you decide whether

0:40

the midbrain is too small or not.

0:43

Now, there are three disorders that resemble

0:46

progressive supranuclear palsy that do not have

0:50

midbrain atrophy until the later stages of the disease.

0:53

These include MSA, multisystem atrophy,

0:57

corticobasal degeneration,

0:59

and Parkinson's disease.

1:00

So when midbrain atrophy is a prominent

1:03

or early component of the disease,

1:06

you've got to start thinking about PSP,

1:08

especially when the superior collicular plate is absent.

1:12

Now, there are secondary causes of a PSP-like syndrome

1:18

where there is vertical gaze palsy

1:21

and some of the other signs of this disorder,

1:23

which is basically dysfunction of the

1:27

nigrostriatal dopaminergic system,

1:29

as well as other movement disorders.

1:32

Ad these include,

1:34

let's go up to the thalamus.

1:36

Unfortunately, there is a gliotic spot here.

1:39

It's related to hypertension.

1:41

So the patient has had a gliotic vascular injury

1:44

along with État criblé lacunaire

1:48

from hypertension.

1:49

And this patient does not demonstrate the hockey

1:52

stick sign of one of the secondary causes of

1:56

progressive supranuclear palsy-like syndrome,

2:00

which is JC disease or Jakob-Creutzfeldt disease,

2:05

also known as prion-mediated disease.

2:08

So, the hockey stick sign will look a little bit like this.

2:11

It's often bilateral,

2:13

and it may involve both sides of the thalami.

2:16

So it may involve both pulvinars at the same time.

2:19

So you may see something that looks like this,

2:21

some signal in the pulvinaric region

2:24

posteriorly on both sides,

2:27

and this is known as the pulvinar sign.

2:30

Unfortunately,

2:31

you can see this with some metabolic disorders,

2:34

including Wernicke's encephalopathy

2:36

and Wilson's disease

2:37

and a few other disorders.

2:39

But chronic pulvinaric signal that persists over a

2:43

period of time, with diffusion restriction,

2:45

without enhancement, should alert you to the potential

2:48

diagnosis of Creutzfeldt-Jakob disease

2:51

or Jakob Creutzfeldt disease

2:53

as a potential secondary cause

2:56

of progressive supranuclear palsy.

2:58

Now, I want to turn your attention

2:59

back once again to the sagittal

3:01

just to highlight the hummingbird sign,

3:04

which, by the way, also has another name,

3:06

the King penguin sign,

3:08

if you are a bird lover.

3:10

Let's move on and talk a little bit more about

3:12

PSP or progressive supranuclear palsy.