Nerve size correlates with clinical severity in Charcot–Marie–Tooth disease 1A
Giampietro Zanette MD
Neurology Division, Pederzoli Hospital, Peschiera del Garda, Verona, Italy
Search for more papers by this authorCorresponding Author
Stefano Tamburin MD
Department of Neurosciences, Biomedicine and Movement Sciences, University of Verona, Verona, Italy
Neurology Division, Department of Neuroscience AOUI Verona, Verona, Italy
Correspondence
Stefano Tamburin, Department of Neurosciences, Biomedicine and Movement Sciences, University of Verona, Piazzale Scuro 10, I-37134 Verona, Italy.
Email: [email protected]
Search for more papers by this authorFederica Taioli PhD
Department of Neurosciences, Biomedicine and Movement Sciences, University of Verona, Verona, Italy
Neurology Division, Department of Neuroscience AOUI Verona, Verona, Italy
Search for more papers by this authorMatteo Francesco Lauriola BSc
Neurology Division, Pederzoli Hospital, Peschiera del Garda, Verona, Italy
Search for more papers by this authorAndrea Badari BSc
Neurology Division, Pederzoli Hospital, Peschiera del Garda, Verona, Italy
Search for more papers by this authorMoreno Ferrarini PhD
Department of Neurosciences, Biomedicine and Movement Sciences, University of Verona, Verona, Italy
Neurology Division, Department of Neuroscience AOUI Verona, Verona, Italy
Search for more papers by this authorTiziana Cavallaro MD
Neurology Division, Department of Neuroscience AOUI Verona, Verona, Italy
Search for more papers by this authorGian Maria Fabrizi MD
Department of Neurosciences, Biomedicine and Movement Sciences, University of Verona, Verona, Italy
Neurology Division, Department of Neuroscience AOUI Verona, Verona, Italy
Search for more papers by this authorGiampietro Zanette MD
Neurology Division, Pederzoli Hospital, Peschiera del Garda, Verona, Italy
Search for more papers by this authorCorresponding Author
Stefano Tamburin MD
Department of Neurosciences, Biomedicine and Movement Sciences, University of Verona, Verona, Italy
Neurology Division, Department of Neuroscience AOUI Verona, Verona, Italy
Correspondence
Stefano Tamburin, Department of Neurosciences, Biomedicine and Movement Sciences, University of Verona, Piazzale Scuro 10, I-37134 Verona, Italy.
Email: [email protected]
Search for more papers by this authorFederica Taioli PhD
Department of Neurosciences, Biomedicine and Movement Sciences, University of Verona, Verona, Italy
Neurology Division, Department of Neuroscience AOUI Verona, Verona, Italy
Search for more papers by this authorMatteo Francesco Lauriola BSc
Neurology Division, Pederzoli Hospital, Peschiera del Garda, Verona, Italy
Search for more papers by this authorAndrea Badari BSc
Neurology Division, Pederzoli Hospital, Peschiera del Garda, Verona, Italy
Search for more papers by this authorMoreno Ferrarini PhD
Department of Neurosciences, Biomedicine and Movement Sciences, University of Verona, Verona, Italy
Neurology Division, Department of Neuroscience AOUI Verona, Verona, Italy
Search for more papers by this authorTiziana Cavallaro MD
Neurology Division, Department of Neuroscience AOUI Verona, Verona, Italy
Search for more papers by this authorGian Maria Fabrizi MD
Department of Neurosciences, Biomedicine and Movement Sciences, University of Verona, Verona, Italy
Neurology Division, Department of Neuroscience AOUI Verona, Verona, Italy
Search for more papers by this authorAbstract
Introduction
Nerve cross-sectional area (CSA) is larger than normal in Charcot–Marie–Tooth disease 1A (CMT1A), although to a variable extent. We explored whether CSA is correlated with CMT clinical severity measured with neuropathy score version 2 (CMTNS2) and its examination subscore (CMTES2) in CMT1A.
Methods
We assessed 56 patients with CMT1A (42 families). They underwent nerve conduction study (NCS) and nerve high-resolution ultrasound (HRUS) of the left median, ulnar, and fibular nerves.
Results
Univariate analysis showed NCS and HRUS variables to be significantly correlated with CMTNS2 and CMTES2 and with each other. Multivariate analysis showed that ulnar motor nerve conduction velocity (β: −0.19) and fibular compound muscle action potential amplitude (−1.50) significantly influenced CMTNS2 and that median forearm CSA significantly influenced CMTNS2 (β: 5.29) and CMTES2 (4.28).
Discussion
Nerve size is significantly associated with clinical scores in CMT1A, which suggests that it might represent a potential biomarker of CMT damage and progression.
CONFLICT OF INTEREST
The authors declare no conflicts of interest related to this report.
Supporting Information
| Filename | Description |
|---|---|
| mus26688-sup-0001-FigureS1.jpegJPEG image, 1.3 MB | Supplementary Figure 1 Scatterplots showing the correlation between CMT neuropathy score version 2 examination subscore (CMTES2; range = 0-28) and median nerve conduction (panels A–B) and high-resolution ultrasound measures (panels C–F). CMAP = compound muscle action potential. CMT = Charcot–Marie–Tooth disease. CSA = cross-sectional area. MNCV = motor nerve conduction. |
| mus26688-sup-0002-FigureS2.jpegJPEG image, 1.2 MB | Supplementary Figure 2 Scatterplots showing the correlation between CMT neuropathy score version 2 examination subscore (CMTES2; range = 0-28) and ulnar nerve conduction (panels A–B) and high-resolution ultrasound measures (panels C–F). CMAP = compound muscle action potential. CMT = Charcot–Marie–Tooth disease. CSA = cross-sectional area. MNCV = motor nerve conduction. |
| mus26688-sup-0003-TableS1.docxWord 2007 document , 13.9 KB | Supplementary Table 1 Cross-correlation between nerve conduction study and high-resolution ultrasound (HRUS) variables in the left median nerve |
| mus26688-sup-0004-TableS2.docxWord 2007 document , 14.1 KB | Supplementary Table 2 Cross-correlation between nerve conduction study and high-resolution ultrasound (HRUS) variables in the left ulnar nerve |
Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.
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