title: Mononeuropathy multiplex as the initial manifestation of neurofibromatosis type 2.
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.
authors: Department of Neurology, Neuromuscular Center Nijmegen, the Netherlands. j.gijtenbeek@czzneu.azn.nl
authors: J M
authors: JM
authors: Gijtenbeek
authors: A A
authors: AA
authors: Gabreëls-Festen
authors: M
authors: M
authors: Lammens
authors: M J
authors: MJ
authors: Zwarts
authors: B G
authors: BG
authors: van Engelen
mesh_terms: D001369:Axons / Q000473:pathology / Q000648:ultrastructure; D005260:Female; D006801:Humans; D008854:Microscopy, Electron; D008875:Middle Aged; D016518:Neurofibromatosis 2 / Q000473:pathology*; D010523:Peripheral Nervous System Diseases / Q000473:pathology*; D012583:Schwann Cells / Q000648:ultrastructure; D013497:Sural Nerve / Q000473:pathology