Mononeuropathy multiplex as the initial manifestation of neurofibromatosis type 2.

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Title:	Mononeuropathy multiplex as the initial manifestation of neurofibromatosis type 2.
Author(s):	Gijtenbeek, J.M.M. ; Gabreëls-Festen, A.A.W.M.; Lammens, M.M.Y. ; Zwarts, M.J. ; Engelen, B.G.M. van
Publication year:	2001
Source:	Neurology, vol. 56, iss. 12, (2001), pp. 1766-1768
ISSN:	0028-3878
Related links:	http://www.neurology.org/cgi/content/abstract/56/12/1766
DOI:	https://doi.org/10.1212/WNL.56.12.1766
Publication type:	Article / Letter to editor
Please use this identifier to cite or link to this item : http://hdl.handle.net/2066/122188
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Subject:	Neuromuscular and neurometabolic disorders Tumor pathology Neuromusculaire en neurometabole aandoeningen Tumor pathologie
Organization:	Neurology
Journal title:	Neurology
Volume:	vol. 56
Issue:	iss. 12
Page start:	p. 1766
Page end:	p. 1768
Abstract:	The authors report a patient with neurofibromatosis type 2 (NF2) presenting with an axonal mononeuropathy multiplex. Sural nerve biopsy showed small scattered groups of Schwann cells transformed into irregular branching cells with abnormal cell-cell contacts. The authors hypothesize that defective Schwann cell function, due to inactivation of the NF2 gene product merlin, leads to changes in morphology, cell-cell contact, and growth, and finally to degeneration of axons.