Three cases of chronic distal sensorimotor neuropathy are described in patients with neurofibromatosis. One had type 2 or central neurofibromatosis with a chromosome 22 deletion; the precise form of the disease was not established in the other two. A striking clinical feature was a diffuse nodular enlargement of the peripheral nerves. Nerve biopsies from all three cases demonstrated the presence of neurofibromatous pathology. Neurofibromatous neuropathy constitutes a rare manifestation of neurofibromatosis, related to diffuse neurofibromatous changes in the peripheral nerves.


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@article{Thomas1990NeurofibromatousN, title={Neurofibromatous neuropathy.}, author={Professor P. K. Thomas and Rosalind H. M. King and Theresa Chiang and Francesco Scaravilli and Atul Sharma and A. W. Downie}, journal={Muscle & nerve}, year={1990}, volume={13 2}, pages={93-101} }