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Chronic inflammatory demyelinating polyradiculoneuropathy with tumefactive central demyelination
Lisa S Krivickas1, Fred H Hochberg, Stefanie Freeman
1Department of Physical Medicine and Rehabilitation, Harvard Medical School and Spaulding Rehabilitation Hospital, Boston, Massachusetts 02114, USA. lkrivickas@partners.org
Muscle & Nerve
|August 9, 2005
Summary
A patient with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) developed central nervous system demyelination and a brain mass. Steroid treatment improved both the mass and neurological symptoms.
Area of Science:
- Neurology
- Neuroimmunology
- Demyelinating Diseases
Background:
- Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an autoimmune disorder affecting peripheral nerves.
- Central nervous system involvement in CIDP is rare and its presentation can be varied.
Observation:
- A 46-year-old man presented with progressive hand paralysis and a history of peripheral neuropathy.
- Brain MRI revealed a contrast-enhancing mass with biopsy showing focal myelin loss and macrophage activity.
- The patient was diagnosed with CIDP and central demyelination.
Findings:
- Electrodiagnostic studies confirmed CIDP.
- Biopsy demonstrated central demyelination with axonal preservation.
- The brain mass and neurological deficits improved with steroid therapy.
Implications:
- This case expands the known spectrum of demyelinating diseases associated with CIDP.
- It highlights the importance of considering central nervous system involvement in CIDP patients with neurological changes.
- The findings suggest a potential link between CIDP and central demyelinating lesions with mass effect.