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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