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Chronic Inflammatory Demyelinating Polyradiculoneuropathy

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Although we described chronic inflammatory

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demyelinating polyradiculopathy and the

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hypertrophic motor sensory neuropathy syndromes,

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when we were talking about intradural extramedullary

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lesions, the truth is that these may be

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purely extradural diseases of the nerve roots.

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CIDP is an acquired disorder, usually thought to

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be an autoimmune disorder in which the patients

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present with proximal weakness and paresthesias.

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They may affect the cranial nerves or

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the peripheral nerves, and it is usually

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a bilateral and symmetric process.

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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

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enlargement of the nerve roots, which is

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predominantly in the extradural compartment.

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As you can see, this is going through the

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anterior scalene, middle scalene junction

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involving the brachial plexus, and this is on T2

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

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enlarged neuroforamina as well as the involvement

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of the nerves as they go into the brachial plexus.

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So this is called CIDP, and it is again,

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an autoimmune disorder, which is often treated with

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immunosuppressive medications and definitively with

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plasmapheresis. In the differential diagnosis with

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CIDP are the hereditary motor and sensory neuropathy

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syndromes, such as your Charcot-Marie-Tooth disease and

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Dejerine-Sottas disease, which may be purely extradural.

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Also, in the differential diagnosis, one may

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find some of the mucopolysaccharidoses, which

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also may be associated with enlarged nerve roots,

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as well as dural thickening, most commonly at

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the foramen magnum and C1–C2 junction.

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So these are causes of enlarged

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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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