Interactive Transcript
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This is an MRI of the brain
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in an eight-year-old child with headaches,
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with also a known history of neurofibromatosis Type 1.
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So, at first glance, we look at this,
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we see everything is symmetric
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on this axial T2-weighted image.
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Now, if we look at this axial FLAIR image,
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we see some hyperintense signal in the region
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of the pulvinar of the thalamus bilaterally.
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But otherwise,
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we're not seeing a lot of supratentorial
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signal abnormalities.
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That can be common because
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every patient with neurofibromatosis Type 1
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has a brain manifestation that
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looks a little bit different.
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Now,
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I want to make sure we discuss these areas of
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bright signal have sometimes, in the past,
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been referred to as UBO's
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or Unidentified Bright Objects,
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or hamartomas.
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Well, they're not hamartomas.
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We now know that they're actually
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areas of myelin vacuolization.
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We know this because they may appear
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as a child is growing,
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and they may disappear during adolescence
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and young adulthood.
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Hamartomas don't disappear.
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And since we know what they are,
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myelin vacuolization,
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they're not really unidentified bright objects.
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They're areas of myelin vacuolization
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in neurofibromatosis Type 1.
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If I scroll to the brainstem,
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we can actually see here
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in the right aspect of the pons,
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we see an area of signal abnormality.
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I'm going to zoom in and we can see this patchy
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area of T2 hyperintense signal.
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We can see an additional area
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of T2 hyperintense signal
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in the lateral aspect
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of the right middle cerebellar peduncle.
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And we can also see some bright signal in the
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deep cerebellar gray matter in the right
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cerebellar hemisphere,
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in the region of the dentate nucleus.
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These are all areas of myelin vacuolization
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in the setting of neurofibromatosis Type 1.
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If we look closely at the sagittal image
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and we identify the basion to the opisthion,
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if we draw a line connecting them,
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that line approximates the plane
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of the foramen magnum.
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The cerebellar tonsils extend 13 mm below
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the plane of the foramen magnum.
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So, while this patient has neurofibromatosis type 1,
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this patient also has a Chiari Type 1 malformation.
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So, remember,
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a patient that has one entity can have more
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than one disease process going on,
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more than one condition.
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So, this patient has neurofibromatosis type 1
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with a relatively mild pattern
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of myelin vacuolization,
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also with a Chiari Type 1 malformation.
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