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Authors
P. Pytel, K. Rezania, B. Soliven, J. Frank, R. Wollmann

1Department of Pathology, MC 6106, University of Chicago Hospitals, 5841 S Maryland Ave., Chicago, IL 60637, USA
2Department of Neurology, University of Chicago, Chicago, USA

Abstract
This report illustrates a case of chronic inflammatory demyelinating

 polyradiculoneuropathy (CIDP) masquerading as neurofibromatosis due to multifocal

enlargements of spinal nerve roots. The patient initially complained of intermittent

 numbness of the hands and leg weakness at age 62. Nerve conduction velocities

 were reported to be abnormally slow, suggesting a diagnosis of demyelinating

 neuropathy. A complaint of progressive lower back pain 4 years later prompted a

 lumbar CT myelogram, which demonstrated bilateral nerve root enlargements. A

 biopsy of an enlarged lumbar root obtained during decompressive laminectomy was\

 interpreted as consistent with a plexiform neurofibroma. He suffered recurrent

 paraparesis, at times with a sensory level indicating spinal cord compression, which

responded to corticosteroid therapy. An autopsy 15 years after the onset of symptoms

 revealed hypertrophic radiculopathy and peripheral neuropathy due to CIDP with no

 evidence of neurofibromatosis. This case illustrates how the hypertrophic neuropathy

 accompanying CIDP can be mistaken for neurofibromatosis.

 

Keywords
Chronic inflammatory demyelinating polyneuropathy, Neurofibromatosis, Hypertrophic neuropathy

 

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