Generalized nerve sheath tumors in neurofibromatosis type 1 (NF1) - A casereport

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