Generalized nerve sheath tumors in neurofibromatosis type 1 (NF1). A case report

Neuropediatrics. 2000 Aug;31(4):211-3. doi: 10.1055/s-2000-7458.

Abstract

We describe a case of chronic distal sensorimotor neuropathy associated with neurofibromatosis type 1 (NF1) in a 15-year old girl. The patient showed a striking clinical picture consisting of diffuse nodular enlargements of peripheral nerves. Motor conduction velocities were decreased and sensory responses were absent after maximal stimulation. Magnetic resonance imaging (MRI) was performed throughout the body and disclosed generalized nerve sheath neurofibromas affecting all peripheral nerves. Intracranially, the patient had a glioma of the left optic nerve, but no other cranial nerve involvement. These results demonstrate the value of MRI for visualization of the peripheral nervous system in neurofibromatosis.

Publication types

  • Case Reports

MeSH terms

  • Child, Preschool
  • Female
  • Humans
  • Magnetic Resonance Imaging
  • Median Nerve / pathology
  • Nerve Sheath Neoplasms / complications*
  • Nerve Sheath Neoplasms / diagnosis
  • Neural Conduction / physiology
  • Neurofibromatosis 1 / complications*
  • Neurofibromatosis 1 / diagnosis
  • Optic Nerve / pathology
  • Radial Nerve / pathology
  • Spinal Nerves / pathology
  • Spinal Nerves / physiopathology
  • Ulnar Nerve / pathology