Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with hypertrophic spinal radiculopathy mimicking neurofibromatosis

Acta Neuropathol. 2003 Feb;105(2):185-8. doi: 10.1007/s00401-002-0616-7. Epub 2002 Sep 28.

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.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Diagnosis, Differential
  • Humans
  • Hypertrophy / diagnostic imaging
  • Hypertrophy / etiology
  • Hypertrophy / pathology
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Neural Conduction / physiology
  • Neurofibromatoses / pathology*
  • Paraparesis / etiology
  • Paraparesis / pathology
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / complications
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / diagnostic imaging
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / pathology*
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / physiopathology
  • Radiculopathy / diagnostic imaging
  • Radiculopathy / etiology
  • Radiculopathy / pathology
  • Radiography
  • Spinal Nerve Roots / diagnostic imaging
  • Spinal Nerve Roots / pathology*