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Creutzfeldt-Jakob Disease: Part 1

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Dr. Laser,

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we've got a 64-year-old woman with memory loss

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for one year, you know, rather profound.

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And thus she was unable to hold

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still in the scanner.

0:09

But we are still able to get a study,

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T2 axial fast spin echo FLAIR in the middle,

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and diffusion study on the far right.

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B value approximately 1000.

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And I sense that there's going to be some hockey.

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I don't know if you were a nice hockey player,

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but I sensed some hockey coming on here

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and a hockey stick sign,

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which I'll leave to you in a moment.

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But it's sort of weird.

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You have bilateral caudoputaminal hyperintensity.

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And, by the way, these won't enhance at all.

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They're just kind of sitting there.

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And they do sit there.

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You've got bilateral thalamic signal alteration.

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You've also got some signal alteration in the

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cortex, which is hard to see on the conventional

0:49

portion of the examination.

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But when you go over to the diffusion restriction

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portion of the study, caudate involved,

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putamen involved, thalamus involved,

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and how would you describe

1:00

that thalamic involvement?

1:01

So you have medial thalamic involvement and

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you have pulvinar thalamic involvement.

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And when you put the two together,

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it almost looks like a hockey stick.

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Your long shaft is the medial aspect of the

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thalamus, and then your pulvinar is your...

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the short end of the stick.

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So we have both.

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We also have another sign in the cortex.

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If we go up a little bit higher on the diffusion

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restriction portion of the study,

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I'll zoom out a little bit.

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What do you think of the cortex?

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You have this intrinsic diffusion-weighted

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cortical ribbon hyperintensity on the diffusion

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weighted sequence,

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and it's the cortical ribbon sign.

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And it's bilateral, too.

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It's in the right frontal as well as

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the mesial left frontal area.

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Now,

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this is typical of somebody with

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CJD or Creutzfeldt-Jakob disease.

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There is one other disorder that is a slow-acting

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viral-type infection that affects humans and

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that is transmissible mink encephalopathies.

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You see it in people that strip the...

2:01

strip the coats off minks in Russia.

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We don't see it much in this country.

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And then there are others that affect animals and

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other syndromes that are not affecting humans,

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like Gerstmann-Sträussler-Scheinker syndrome,

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Kuru, and Scrapie.

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So the one that you have to be

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most concerned about is CJD.

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You've described the hockey stick sign,

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the pulvinar sign, the ribbon sign,

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the fact that it's diffusion-restricted,

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the fact that it's on the FLAIR, and the T2,

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that it changes very, very slowly,

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if at all, over time.

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The fact that it doesn't enhance all these things

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along with the symmetry of it,

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go on to support the diagnosis

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of a prion-mediated disorder.

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So in the next vignette,

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we'll talk a little bit about CJD.

2:43

Do you have any other comments about the

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MRI findings that we see in this case?

2:46

Okay, let's move on then.

2:47

Laser and Pomeranz out.

Report

Description

Faculty

Stephen J Pomeranz, MD

Chief Medical Officer, ProScan Imaging. Founder, MRI Online

ProScan Imaging

Tags

Syndromes

Neuroradiology

MRI

Infectious

Brain

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