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Massive nerve root enlargement in chronic inflammatory demyelinating polyneuropathy
W Schady1, P J Goulding, B R Lecky
1Department of Neurology, Manchester Royal Infirmary, UK.
Journal of Neurology, Neurosurgery, and Psychiatry
|December 1, 1996
Summary
Chronic inflammatory demyelinating polyneuropathy (CIDP) can affect nerve roots, mimicking other conditions. MRI of the cauda equina aids in diagnosing CIDP, especially when nerve thickening is subtle.
Area of Science:
- Neurology
- Neurophysiology
- Pathology
Background:
- Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare autoimmune disorder affecting peripheral nerves.
- Typical CIDP presentation involves symmetrical sensory and motor deficits, but atypical presentations can occur.
Observation:
- Three patients with CIDP presented with symptoms suggestive of cervical or lumbar root disease.
- Nerve roots appeared enlarged on MRI, CT myelography, and during surgery.
- Peripheral nerve thickening was minimal or absent clinically, but facial nerve hypertrophy occurred in one patient.
Findings:
- Neurophysiological tests indicated a demyelinating polyneuropathy.
- Sural nerve biopsies revealed myelinated fiber loss, inflammation, and onion bulbs.
- Posterior nerve root biopsies showed significant hypertrophic changes with Schwann cell proliferation.
Implications:
- MRI of the cauda equina can be a valuable diagnostic tool for CIDP, particularly in cases with atypical root involvement.
- Early diagnosis and appropriate treatment, such as steroids, are crucial for managing CIDP.
- Further research is needed to optimize treatment strategies for advanced CIDP cases.
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