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Muscle action potential scans and ultrasound imaging in neurofibromatosis type 2

Farschtschi, S; Gelderblom, M; Buschbaum, S; Bostock, H; Grafe, P; Mautner, VF; (2016) Muscle action potential scans and ultrasound imaging in neurofibromatosis type 2. Muscle & Nerve 10.1002/mus.25256. (In press). Green open access

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Abstract

INTRODUCTION: The neuropathy in patients with neurofibromatosis type 2 (NF2) is difficult to quantify and follow up. In this study we compared 3 methods that may help assess motor axon pathology in NF2 patients. METHODS: Nerve conduction studies in median nerves were supplemented by deriving motor unit number estimates (MUNEs) from compound muscle action potential (CMAP) scans and by high-resolution ultrasound (US) peripheral nerve imaging. RESULTS: CMAP amplitudes and nerve conduction velocity were normal in the vast majority of affected individuals, but CMAP scan MUNE revealed denervation and reinnervation in many peripheral nerves. In addition, nerve US imaging enabled monitoring of the size and number of schwannoma-like fascicular enlargements in median nerve trunks. CONCLUSION: In contrast to conventional nerve conduction studies, CMAP scan MUNE in combination with US nerve imaging can quantify the NF2-associated neuropathy and may help to monitor disease progression and drug treatments.

Type: Article
Title: Muscle action potential scans and ultrasound imaging in neurofibromatosis type 2
Location: United States
Open access status: An open access version is available from UCL Discovery
DOI: 10.1002/mus.25256
Publisher version: http://dx.doi.org/10.1002/mus.25256
Language: English
Additional information: Copyright © 2016 Wiley Periodicals, Inc. This is the pre-peer reviewed version of the following article: [Farschtschi, S., Gelderblom, M., Buschbaum, S., Bostock, H., Grafe, P. and Mautner, V. F. (2016), Muscle action potential scans and ultrasound imaging in neurofibromatosis type 2. Muscle Nerve. doi: 10.1002/mus.25256], which has been published in final form at http://dx.doi.org/10.1002/mus.25256. This article may be used for non-commercial purposes in accordance with Wiley Terms and Conditions for Self-Archiving.
Keywords: MUNE, denervation, neurofibromatosis type 2, neuropathy, reinnervation, ultrasound nerve imaging
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology
URI: https://discovery.ucl.ac.uk/id/eprint/1506026
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