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Sonography of the median nerve in CMT1A,CMT2A,CMTX, and HNPP
Authors:Stefanie Schreiber MD  Andreas Oldag MD  Cornelia Kornblum MD  Katja Kollewe MD  Siegfried Kropf PhD  Ariel Schoenfeld MD  Helmut Feistner MD  Sibylle Jakubiczka MD  Wolfram S Kunz PhD  Cordula Scherlach MD  Claus Tempelmann PhD  Christian Mawrin MD  Reinhard Dengler MD  Frank Schreiber MSc  Michael Goertler MD  Stefan Vielhaber MD
Institution:1. Department of Neurology, Otto‐von‐Guericke University, , Magdeburg, Germany;2. Department of Neurology, University of Bonn Medical Center, , Bonn, Germany;3. Department of Neurology, Hannover Medical School, , Hannover, Germany;4. Institute of Biometry and Medical Informatics, Otto‐von‐Guericke University, , Magdeburg, Germany;5. Institute of Human Genetics, Otto‐von‐Guericke University, , Magdeburg, Germany;6. Department of Epileptology and Life and Brain Centre, University of Bonn Medical Center, , Bonn, Germany;7. Institute of Neuroradiology, Otto‐von‐Guericke University, , Magdeburg, Germany;8. Institute of Neuropathology, Otto‐von‐Guericke University, , Magdeburg, Germany;9. Institute of Control Engineering, Technical University Braunschweig, , Braunschweig, Germany;10. German Centre of Neurodegenerative Diseases, , Magdeburg, Germany
Abstract:Introduction: In this study we compare the ultrasound features in the median nerve in patients with different types of Charcot–Marie–Tooth (CMT) disease and hereditary neuropathies with liability to pressure palsies (HNPP) as a typical entrapment neuropathy. Methods: Median nerve ultrasound and conduction studies were performed in patients with CMT1A (n = 12), MFN2‐associated CMT2A (n = 7), CMTX (n = 5), and HNPP (n = 5), and in controls (n = 28). Results: Median nerve cross‐sectional area (CSA) was significantly increased in CMT1A, whereas, in axonal CMT2A, fascicle diameter (FD) was enlarged. CSA correlated with nerve conduction slowing in CMT1A and with axonal loss, as shown by motor and sensory nerve amplitudes in both CMT1A and CMT2A. A relatively low wrist‐to‐forearm‐ratio (WFR <0.8) or a relatively high WFR (>1.8) appeared to be unlikely in MFN2 and Cx32 mutations of CMT2A and CMTX, respectively. Conclusion: Differences in CSA, FD, and WFR of the median nerve can be helpful in defining subtypes of hereditary neuropathies. Muscle Nerve 47:385‐395, 2013
Keywords:CMT1A  CMT2A  HNPP  MFN2  ultrasound
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