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Muscle action potential scans and ultrasound imaging in neurofibromatosis type 2
Author(s) -
Farschtschi Said,
Gelderblom Mathias,
Buschbaum Sabriena,
Bostock Hugh,
Grafe Peter,
Mautner Victor F.
Publication year - 2017
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.25256
Subject(s) - medicine , compound muscle action potential , denervation , reinnervation , median nerve , nerve conduction velocity , neurofibromatosis , nerve conduction study , radiology , anatomy , nerve conduction , electrophysiology
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