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 CMT1A patients (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 ulnar motor nerve conduction velocity (β: -0.19) and fibular compound muscle action potential amplitude (-1.50) to significantly influence CMTNS2 and median forearm CSA to significantly influence CMTNS2 (β: 5.29) and CMTES2 (4.28). DISCUSSION: Nerve size is significantly associated with clinical scores in CMT1A, suggesting it might represent a potential biomarker of CMT damage and progression. This article is protected by copyright. All rights reserved.

Nerve size correlates with clinical severity in Charcot–Marie–Tooth disease 1A

Tamburin, Stefano
;
Taioli, Federica;Ferrarini, Moreno;Cavallaro, Tiziana;Fabrizi, Gian Maria
2019-01-01

Abstract

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 CMT1A patients (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 ulnar motor nerve conduction velocity (β: -0.19) and fibular compound muscle action potential amplitude (-1.50) to significantly influence CMTNS2 and median forearm CSA to significantly influence CMTNS2 (β: 5.29) and CMTES2 (4.28). DISCUSSION: Nerve size is significantly associated with clinical scores in CMT1A, suggesting it might represent a potential biomarker of CMT damage and progression. This article is protected by copyright. All rights reserved.
Clinical scores; cross sectional area (CSA); high resolution ultrasound (HRUS); inherited neuropathy; multivariate analysis; nerve conduction study (NCS)
File in questo prodotto:
Non ci sono file associati a questo prodotto.

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11562/999602
Citazioni
  • ???jsp.display-item.citation.pmc??? 7
  • Scopus 12
  • ???jsp.display-item.citation.isi??? 12
social impact