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Bilateral Corpus Striatum Caudoputamen Hyperintensity: Wilson’s Disease

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This 64-year-old woman presents with

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cognitive dysfunction ataxia.

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There is cerebellar hemispheric atrophy and dystonia.

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She's got bilateral caudoputaminal hyperintensity

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with loss of putaminal volume,

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but preservation of caudate volume seen on FLAIR

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seen on T2,

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and preservation of the caudate,

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especially well seen on T1 axial imaging.

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There is subtle bithalamic involvement.

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The key to the diagnosis is the symmetry of the

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process and the fact that the cortex

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is symmetrically atrophic.

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There really aren't any areas of abnormal

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iron in the basal ganglia.

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There might be a little increased conspicuity in

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the back of the putamen where this

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process has not affected it yet.

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Let's go down to the brain stem where

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we see preservation.

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In fact, pronounced preservation of the

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zona compacta stripe,

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which you typically don't see in moderate to

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advanced cases of Alzheimer's disease.

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So in this patient with Dystonia,

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what type of Dystonia do they have?

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They had focal dystonia of one area of the body.

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Two areas that's segmental.

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Generalized everywhere,

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three or more that can be identified,

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specifically multifocal,

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and one side of the body,

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hemidystonia.

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When I have a history of Dystonia,

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I like to break it down into

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primary versus secondary,

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exogenous versus endogenous.

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So, what types of exogenous Dystonia can you get?

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Well, people that inject themselves with cut heroin

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can develop a Dystonic syndrome.

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There's usually some degree of necrosis,

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and it's almost always bilateral,

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but more severe on one side.

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This is spot-on,

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totally symmetrically bilateral,

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which should lead you to metabolic

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causes of Dystonia.

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What's another toxic cause

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of Dystonia that is endogenous?

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Lactate production.

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Where do you get lactate production?

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In disorders that affect the oxidative pathway?

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One example, Leigh's disease.

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But Leigh's disease doesn't occur

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in 50, 60, 70-year-old people.

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It occurs earlier.

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But it absolutely does involve the caudate,

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the putamen and thalamus.

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But there's a theme here,

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we're staying with ATP production,

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an oxidative capacity of the brain,

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another disorder that you're all familiar with

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that can do this, that likes the thalamus,

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but also affects the mammillary body,

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producing hemorrhage and atrophy of the

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mammillary body, not present here.

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The mammillary body is juicy,

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is Wernicke's encephalopathy,

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an acute deficiency C of vitamin B1.

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And what do you need vitamin B1 for?

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You need it to produce oxygen and ATP

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to supply the metabolism of the brain.

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So that would be another consideration.

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Another endogenous toxicity is copper,

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and that is Wilson's disease.

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And that's what this patient has,

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Wilson's disease.

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So what are some of the other manifestations

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of Wilson's disease?

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You can see signal intensity in the thalami and

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the thalami can get kind of swollen.

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Sometimes you'll see something called the big panda sign,

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which we're going to show you in another case.

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But here we have caudate involvement,

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putaminal involvement,

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some subtle thalamic involvement.

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Sometimes in Wilson's disease,

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you'll see accumulation of copper that leads

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to more focal areas of hyperintense

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signal in the basal ganglia,

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almost like basal ganglia necrosis.

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You will see accumulation of manganese

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and magnesium and iron.

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So you may see some very bizarre

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distribution in iron stores.

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So, the signal in Wilson's disease may be high or

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the signal in Wilson's disease may be low.

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This patient has proven Wilson's disease

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and we'll talk about the disorder in the next vignette.

Report

Description

Faculty

Stephen J Pomeranz, MD

Chief Medical Officer, ProScan Imaging. Founder, MRI Online

ProScan Imaging

Tags

Syndromes

Non-infectious Inflammatory

Neuroradiology

Metabolic

MRI

Idiopathic

Drug related

Brain

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