Neurofibromatous neuropathy.
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.
Thomas
P K
PK
Royal Free Hospital School of Medicine, Queen Square, London, UK.
King
R H
RH
Chiang
T R
TR
Scaravilli
F
F
Sharma
A K
AK
Downie
A W
AW
D000328:Adult; D002872:Chromosome Deletion; D002892:Chromosomes, Human, Pair 22; D006801:Humans; D008297:Male; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000235:genetics / Q000473:pathology; D009464:Neuroma, Acoustic / Q000150:complications; D010523:Peripheral Nervous System Diseases / Q000150:complications* / Q000473:pathology