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Mild Cognitive Impairment Syndrome

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Dr. Laser,

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this 63-year-old's main symptoms

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are ataxia and memory loss.

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So, we're into that neurodegenerative

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group of disorders.

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Before we start down the differential of

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this case and point out some of the findings,

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if we take the main dementias,

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I use the clinical as well as the visual as

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tip-offs to segregate into various diagnoses.

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For instance, you know, movement disorders.

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Most patients with ALZ,

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at least in the early to mid-stage,

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don't have a lot of movement disorder

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associated with their syndrome.

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So if they've got tremor and rigidity,

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I'm in the Parkinsonian group,

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whether it's multisystem,

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atrophy or classic Parkinson's disease.

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If they've got hallucinations,

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granted hallucinations do occur with ALZ,

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but prominent visual hallucinations

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pretty early on in somnolence,

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seen with dementia with Lewy bodies.

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Then ALZ is really characterized,

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not just by cognitive decline

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and short-term memory loss.

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The patients have enormous capacity to remember

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back 30, 40, 50 years,

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but short-term memory,

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not so much.

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Their daily function is impaired.

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They can't remember what they said 30 seconds ago.

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They have aphasia, paraphasia,

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and they may even have semantic aphasia

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where the meaning of words is lost.

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Then you've got frontotemporal dementia syndromes,

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of which Pick's disease was included in that in the past.

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We don't use that name so much anymore.

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There are heredofamilial types

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and non-heredofamilial types.

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But this disorder is characterized,

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not just by frontal or frontotemporal involvement,

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but by disinhibition, disexecutive behavior,

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you know,

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eating with your feet at the dinner table,

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making weird and lascivious comments

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early on in the disease.

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So they lose their inhibitory capacity.

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They are, in other words, unfiltered.

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And then you've got vascular disease.

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That one's a little bit easier,

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that's kind of a stuttering decline.

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But because there's vascular disease,

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you may see macro infarcts or you may see,

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as you've described before,

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with the Fazekas scale,

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multiple confluent areas of white matter

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signal alteration,

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commensurate with Binswanger syndrome.

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So now let's scroll this case.

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And we've got left greater than right,

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both parietal and frontal cortical atrophy,

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at least moderate in severity.

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And then in the sagittal projection,

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we've got some parietal occipital atrophy,

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some occipital atrophy.

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Here is the precuneus right here.

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Also a little bit of atrophy,

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some atrophy of the cingulate sulcus

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going all the way forward.

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And there's also some brain stem involvement.

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You commented on this in the report,

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the midbrain is atrophic.

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It looks a little bit like a hummingbird.

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The superior colliculus off to the side.

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It's pretty good, actually.

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It's pretty juicy in the midline.

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It's a little small,

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but they always are in the midline.

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But that at least raised the possibility

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of progressive supranuclear palsy.

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So, ignoring the visual abnormalities that include

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lens replacement and the patient's

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been treated with a band

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for I think possibly retinal detachment or nearsightedness.

3:03

I'm not sure which.

3:05

Tell us about the differential diagnosis of

3:08

ALZ and other disorders, including MCI.

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And what is MCI?

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Mild Cognitive Impairment Syndrome

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is MCI.

3:18

Okay.

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And that term is used a lot,

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and we frequently use that term in place of ALZ

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because if you put the term ALZ in a report,

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it has emotional consequences,

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it has insurance consequences,

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and it has general relationship consequences.

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I once did that in a report to a neurologist's

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mother, and he came in.

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He said, my mother is not demented.

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He was absolutely incensed.

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Turned out she developed dementia about a year later,

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and she did have some cognitive impairment.

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So what is cognitive...

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Mild Cognitive Impairment Syndrome?

3:49

When do we use that term or pull that term out?

3:53

So, typically,

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the way I do it is I look at all the scales that

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we've discussed prior,

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the global cortical atrophy scale,

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the medial temporal lobe atrophy scale,

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the Fazekas scale,

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the codom scale.

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And I try to form a picture of what's going on

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with the patient.

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I try to grade it subjectively,

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but with those scales in mind.

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So I look at the hippocampi,

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which is probably the most important scale

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to look at, and the degree of atrophy,

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the degree of volume loss of the hippocampi.

4:18

In this case,

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as Dr. Pomeranz is pointing out on the left,

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the medial temporal lobe atrophy scale would be

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a two to three, almost a three in this case.

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So, there's profound left hippocampal volume loss.

4:29

And let's redefine what that is.

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The choroidal fissure is big.

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The temporal horn is big,

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and there is at least moderate to

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severe hippocampal atrophy.

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All these structures that live in here,

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the dentate gyrus, the amygdala, and others,

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nice and juicy on the right,

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very small and lobulated on the left.

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And as you and I have discussed before,

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the number one biomarker for ALZ is hippocampal

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volume loss, and this patient has it.

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Now, in somebody where you want to suggest

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the diagnosis of ALZ,

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but they don't give you a strong

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cognitive decline history,

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one way to say it in code is findings are highly

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suggestive of Mild Cognitive Impairment Syndrome,

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or progression thereof.

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Which is your way of saying,

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I'm worried about MCI progressing to ALZ.

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Now, what percent of patients that don't have a florid

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ALZ go on to MCI that have perihippocampal

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or hippocampal involvement?

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It's about 12% per year.

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If they've got some cognitive decline that's mild,

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will go on to flat-out ALZ.

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And over five years,

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80% will end up with ALZ.

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So this patient, you know, is a big candidate,

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a strong candidate for ALZ

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if they don't have it already.

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We haven't physically examined this individual.

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And this patient also has a fair

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amount of frontal involvement,

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which makes the case a little more challenging.

5:55

Right?

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The left is involved more than the right,

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so you might consider a more

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atypical form of frontolobar dementia

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or frontotemporal dementia with parietal extension back

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rather than ALZ with extension forward,

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except there's no behavioral disturbance.

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The patient only has memory loss.

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So that really clinically favors ALZ over FLD,

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Frontal Lobar Dementia,

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But there is involvement of the olfactory area,

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which at least raises the possibility

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of a frontolobar dementia.

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So, we've got a pretty broad differential here.

6:27

Any other comments before we exit this case?

6:31

I would.

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One other thing that you could show is if

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you show the FLAIR sequence,

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that there is very little white matter disease

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for a 63-year-old.

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He has some punctate white matter lesions,

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but the degree of white matter is essentially

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normal for a patient of this age.

6:46

That's a very good point.

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You know, his Fazekas scale is one.

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And one of your charges, one of your jobs,

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is to try and tease out what kind of dementia

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you're dealing with.

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And if you can exclude vascular disease...

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And by the way,

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vascular disease and neurodegenerative disease combined,

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account for 15% of all dementia.

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So, it's pretty common.

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15% are purely vascular.

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So now you've got 30% are attributable

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to vascular disease.

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So if you've got vascular disease

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that's very prominent,

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you may want to treat that patient with, say,

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a statin,

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very aggressive statin therapy to try and halt the

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progression of microvascular

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or macrovascular dementia.

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So, very little white matter disease makes this

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unlikely to be a vasculopathic dementia and more

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likely to be a primary neurodegenerative disease.

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And some of the ones we were

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considering here were PSP,

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which we eventually excluded because

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of the collicular plate,

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even though there is a hummingbird sign,

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frontolobar dementia

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or related Picks disease and ALZ

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or MCI Alzheimer's disease

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or mild cognitive impairment syndrome.

7:48

Any other comments before we quit?

7:49

Nope.

7:49

Okay, P and Laser out.

Report

Description

Faculty

Stephen J Pomeranz, MD

Chief Medical Officer, ProScan Imaging. Founder, MRI Online

ProScan Imaging

Tags

Syndromes

Neuroradiology

MRI

Idiopathic

Brain

Acquired/Developmental

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