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This is a 42-year-old woman with

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tremors and poor wound healing.

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We've got an axial T2 on the left,

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a flair in the middle,

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and a coronal T1 on the far right.

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Let's scroll.

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We've got some pretty nice anatomy here,

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including the delimitation

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between the globus pallidus,

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the internal and external portions,

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and the putamen.

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Got a nice view of the caudate nucleus here anteriorly,

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the thalamus and the pulvinar.

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There's the external capsule,

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the claustrum and the extreme capsule.

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Now, we're talking about tremor.

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And one of the first things that might pop into

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your head, first things that pops in,

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is Parkinson's disease.

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But look at the clarity of the

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red nucleus on multiple cuts,

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certainly on this cut,

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between the nucleus ruber or red nucleus,

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and the substantia nigra.

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Look at the preservation of the lateral aspect of

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the substantia nigra or preservation of the tail

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of the swallow, in this case, laterally.

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So, this is one where you could say the findings

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are inconsistent with Parkinson's disease

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in a tremor patient.

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Now, as you continue scrolling,

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you see that for her age,

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she should have only maybe four or five gliotic foci.

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She's got more than that.

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So, odds are there is a vasculopathic insult

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at the microscopic level that's ongoing.

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But what's even more strange than

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that is as we get down low,

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there are these mesial temporal areas

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of hypointensity that represent hyalinization or

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hardening of the mesiotemporal region.

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And these can go on to calcify on CT.

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This has a very characteristic appearance of

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this disease known as lipoid proteinosis,

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also known as Erbach-Wheatley disease.

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I'm showing it as a nice comparison in somebody

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that has tremor that does not demonstrate the iron

2:09

distribution signs of Parkinson's disease.

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Now,

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this disorder is associated with dry wrinkly skin

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and papules, and beating around the eyelids.

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Mesial temporal brain hardening that is bilateral

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and symmetric is one of the cardinal signs,

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although you may also see basal ganglia

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and thalamic hypointensities.

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It's inherited as an autosomal recessive

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on chromosome 1q21,

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and it's caused by buildup of hyalin material.

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On the coronal T1, circled nicely in green are

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these areas of hyalinization of the mesial

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temporal region in a patient with lipoid

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proteinosis who presents with tremor.