Interactive Transcript
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Dr. Laser,
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we're back with our 59-year-old woman
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who's got profound frontal atrophy,
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left greater than right.
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As you mentioned before, knife blade atrophy,
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atrophy of the temporal region, pretty profound,
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but relative sparing of the parietal region.
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And then, we also said,
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in the coronal projection,
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that the patient had relative sparing of
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the entorhinal cortex,
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which militates heavily against the diagnosis
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of ALZ or Alzheimer's disease.
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And sparing of this is a cardinal
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feature of Pick's disease.
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We also pointed out that there is profound atrophy of
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the temporal lobe, may spare the front or the tip,
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but extends back, and also in this case,
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involves the left side or Broca's area.
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Broadmann areas 44 and 45 are affected,
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so it's not surprising if she has a speech disturbance.
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So, I want to take a moment to talk about
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the different types of Pick's disease.
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You can have frontotemporal dementia
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with a Parkinson-like syndrome,
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and this is one of several forms of Pick's disease
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that may be related to chromosome 17.
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In fact, FTDP-17T is the chromosomal linkage.
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Then you've got the frontotemporal lobar degeneration with
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ubiquitin-positive and tau-negative inclusion,
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so-called frontotemporal lobar dementia,
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FTLD-U.
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And then, you've got frontotemporal lobar dementia
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with motor neuron disease,
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FTLD-MND.
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And then finally, you have frontotemporal lobar
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dementia without ubiquitin or tau deposits,
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dementia lacking distinctive histology.
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So, there are at least four or more subtypes
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of Pick's disease.
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This is a little bit like multisystem atrophy,
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where you divide it up into autonomic dysfunction,
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Parkinson's disease,
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motor affectation, etc.
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Dr. Laser, this is a tauopathy,
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and tau inclusions occur
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in several neurodegenerative diseases.
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The classic one is Alzheimer's disease,
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but frontotemporal dementia, FTD,
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or frontotemporal lobar dementia is another one,
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progressive supranuclear palsy,
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and the fourth major one is corticobasal degeneration.
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Now,
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mutations in tau cause
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some familial frontotemporal dementias,
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of which Pick's disease is one.
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So, what percent of individuals have
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a heredofamilial predilection for Pick's?
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It's about 30% to 40%.
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That is extremely scary.
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Yeah.
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And the onset of Pick's is usually a little
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earlier than Alzheimer's disease.
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There is a familial type of Alzheimer's disease
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that we see in younger individuals.
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And the heredofamilial incidence
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is probably around 10% to 15%.
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So not as strong as it is with Pick's disease,
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which is a scary thought.
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Shall we move on?
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Let's.
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