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Immunologic Causes of Chorea

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Let's talk about other causes of Chorea,

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namely immunological causes of Chorea that include

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SLE, systemic lupus erythematosus,

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primary antiphospholipid antibody syndrome,

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seen more frequently in women,

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vasculitis,

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including primary intracranial leukocytoclastic vasculitis,

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and paraneoplasia.

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Now, most of these can be differentiated.

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People with lupus do not present simply with a

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putaminal abnormality like we might see here,

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either hemorrhage or an infarct.

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They frequently also have some element

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of vasculitis in the cortex,

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which shows up as a hazy area of hyperintensity

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in the cortical or subcortical area,

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which may or may not enhance

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and may or may not bleed.

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They may also have lacunar infarctions elsewhere

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and an angiogram or an MRA may provide

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a sustainable diagnosis.

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On the other hand,

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primary antiphospholipid antibody syndrome,

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which is seen more frequently in women and

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associated with premature miscarriage,

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is frequently seen with white matter abnormalities

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that are scattered throughout the brain,

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simulating, somewhat, very gentle punctate,

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stippled Binswanger's phenomenon.

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Vasculitis would look just like

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systemic lupus erythematosus,

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while paraneoplastic syndrome is often associated

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not with unilateral but bilateral putaminal abnormalities,

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and more frequently, if we scroll,

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it's not uncommon to see concomitant involvement

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of the cortical areas of the hippocampus and

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perihippocampus and paraneoplastic syndrome.

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So, let's talk a little bit further about these.

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SLE or systemic lupus erythematosus

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and / or primary antiphospholipid antibody syndromes,

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these are classically described

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as the prototypical disorders for autoimmune choreas.

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That doesn't mean they're the only ones.

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These have been reported in the context also of

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paraneoplastic syndromes. In other words,

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it's an immune-mediated phenomenon

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in paraneoplasia as well.

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It's usually associated with the anti-hu, H.U.

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more than the anti-mu antibody complex

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and anti-CRMP-5 antibodies in patients with small

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cell carcinoma of the lung.

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So, if you get a choreiform movement disorder

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in somebody with known small cell,

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you're going to go right to paraneoplasia.

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If you wish, let's move on to a quick discussion

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of infectious choreas.

Report

Description

Faculty

Stephen J Pomeranz, MD

Chief Medical Officer, ProScan Imaging. Founder, MRI Online

ProScan Imaging

Tags

Vascular

Non-infectious Inflammatory

Neuroradiology

Neoplastic

Metabolic

MRI

Infectious

Idiopathic

Congenital

Brain

Acquired/Developmental

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