Generalized Nerve Sheath Tumors in Neurofibromatosis Type 1 (NF1) A Case Report

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.

Key words

Generalized nerve sheath neurofibromas - Neurofibromatosis type 1 (NF1) - Peripheral neurofibromas - Plexiform neurofibromas - Magnetic resonance imaging (MRI)

References

• 1 Halliday A L, Sobel R A, Martuza R L. Benign spinal nerve sheath tumors: their occurrence sporadically and in neurofibromatosis types 1 and 2.  J Neurosurg. 1991;  74 248-253
• 2 Needle M N, Cnaan A, Dattilo J, Chatten J, Phillips P C, Shoochat S. et al . Prognostic signs in the surgical management of plexiform neurofibroma: The Children's Hospital of Philadelphia experience, 1974 - 1994.  J Pediatr. 1997;  131 678-682
• 3 Rubenstein L. The malformative central nervous system lesions in the central and peripheral forms of neurofibromatosis: A neuropathological study of 22 cases.  Ann NY Acad Sci. 1986;  486 14-29
• 4 Spinal J B, Indakoetxea B, López de Muniain A, Martí-Massó J F, Riu I. Tumores radiculares múltiples y neurofibromatosis: aportación de la resonancia magnética.  Neurología. 1991;  6 142-147
• 5 Such J S, Abenoza P, Galloway H R, Everson L I, Griffiths H J. Peripheral (extracranial) nerve tumors: Correlation of MR imaging and histologic findings.  Radiology. 1992;  183 341-346
• 6 Thomas P K, King R HM, Chiang T R, Scaravilli F, Sharma A K, Downie A W. Neurofibromatosis neuropathy.  Muscle Nerve. 1990;  13 93-101