PMID- 27422240 OWN - NLM STAT- MEDLINE DCOM- 20170703 LR - 20180307 IS - 1097-4598 (Electronic) IS - 0148-639X (Linking) VI - 55 IP - 3 DP - 2017 Mar TI - Muscle action potential scans and ultrasound imaging in neurofibromatosis type 2. PG - 350-358 LID - 10.1002/mus.25256 [doi] AB - 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. Muscle Nerve 55: 350-358, 2017. CI - (c) 2016 Wiley Periodicals, Inc. FAU - Farschtschi, Said AU - Farschtschi S AD - Department of Neurology, University Medical Centre Hamburg-Eppendorf Hospital, Hamburg, Germany. FAU - Gelderblom, Mathias AU - Gelderblom M AD - Department of Neurology, University Medical Centre Hamburg-Eppendorf Hospital, Hamburg, Germany. FAU - Buschbaum, Sabriena AU - Buschbaum S AD - Department of Neurology, University Medical Centre Hamburg-Eppendorf Hospital, Hamburg, Germany. FAU - Bostock, Hugh AU - Bostock H AD - Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, University College London, London, UK. FAU - Grafe, Peter AU - Grafe P AD - Physiological Genomics, Institute of Physiology, Ludwig-Maximilians University Munich, Pettenkoferstrasse 12, 80336, Munich, Germany. FAU - Mautner, Victor F AU - Mautner VF AD - Department of Neurology, University Medical Centre Hamburg-Eppendorf Hospital, Hamburg, Germany. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20161216 PL - United States TA - Muscle Nerve JT - Muscle & nerve JID - 7803146 SB - IM MH - Action Potentials/*physiology MH - Adolescent MH - Adult MH - Aged MH - Child MH - Electrophysiology MH - Female MH - Humans MH - Male MH - Middle Aged MH - Motor Neurons/physiology MH - Muscle, Skeletal/*diagnostic imaging/*physiopathology MH - Neural Conduction/physiology MH - Neurofibromatosis 2/complications/*diagnostic imaging/*pathology MH - Peripheral Nerves/diagnostic imaging MH - *Ultrasonography MH - Young Adult OTO - NOTNLM OT - MUNE OT - denervation OT - neurofibromatosis type 2 OT - neuropathy OT - reinnervation OT - ultrasound nerve imaging EDAT- 2016/07/17 06:00 MHDA- 2017/07/04 06:00 CRDT- 2016/07/17 06:00 PHST- 2016/07/12 00:00 [accepted] PHST- 2016/07/17 06:00 [pubmed] PHST- 2017/07/04 06:00 [medline] PHST- 2016/07/17 06:00 [entrez] AID - 10.1002/mus.25256 [doi] PST - ppublish SO - Muscle Nerve. 2017 Mar;55(3):350-358. doi: 10.1002/mus.25256. Epub 2016 Dec 16.