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Chronic Inflammatory Demyelinating Polyradiculoneuropathy

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Although we described chronic inflammatory demyelinating

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polyradiculoneuropathy and the hypertrophic motor

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sensory neuropathy syndromes when we were talking

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about intradural extramedullary lesions,

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the truth is that these may be purely extradural

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diseases of the nerve roots.

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CIDP is an acquired disorder usually thought to be an

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autoimmune disorder in which the patients present

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with proximal weakness and paresthesias,

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they may affect the cranial nerves or the peripheral

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nerves, and it is usually a bilateral and symmetric

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process. When it's unilateral or asymmetric, it may be

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called MMN, which is Multifocal Motor Neuropathy.

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Here we have examples of a patient who has enlargement

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of the nerve roots which is predominantly in

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the extradural compartment. As you can see,

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this is going through the anterior scaling,

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middle scaling junction involving the brachial plexus.

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And this is on T2-weighted scan,

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T1-weighted scan, and you see that there are enlarged

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neuroforamina, as well as the involvement of the

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nerves as they go into the brachial plexus.

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So this is called CIDP and it is, again, an autoimmune

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disorder which is often treated with immunosuppressive

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medications and definitively with plasmapheresis.

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In the differential diagnosis with CIDP are the

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hereditary motor and sensory neuropathy

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syndrome, such as your Charcot-Marie-Tooth disease and Dejerine–Sottas disease,

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which may be purely extradural.

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Also in the differential diagnosis, one may find some of the mucopolysaccharidoses

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which also may be associated with enlarged

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nerve roots, as well as dural thickening,

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most commonly at the foramen magnum

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and C1-C2 junction.

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So these are causes of enlarged extradural nerve roots.

Report

Description

Faculty

David M Yousem, MD, MBA

Professor of Radiology, Vice Chairman and Associate Dean

Johns Hopkins University

Tags

Spine

Non-infectious Inflammatory

Neuroradiology

Musculoskeletal (MSK)

MRI

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