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. Muscle Nerve 55: 350-358, 2017.
journal_name
Muscle Nervejournal_title
Muscle & nerveauthors
Farschtschi S,Gelderblom M,Buschbaum S,Bostock H,Grafe P,Mautner VFdoi
10.1002/mus.25256subject
Has Abstractpub_date
2017-03-01 00:00:00pages
350-358issue
3eissn
0148-639Xissn
1097-4598journal_volume
55pub_type
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