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Nerve conduction velocity in CMT1A: what else can we tell?

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Title: Nerve conduction velocity in CMT1A: what else can we tell?
Authors: Manganelli, F., Pisciotta, C., Reilly, M. M., Tozza, S., Schenone, A., Fabrizi, G. M., Cavallaro, T., Vita, G., Padua, L., Gemignani, F., Laurà, M., Hughes, R. A. C., Solari, A., Pareyson, D., Santoro, L., Nolano, Maria, Iodice, Rosa, Grandis, Marina, Cabrini, Ilaria, Bertolasi, Laura
Source: European Journal of Neurology. Oct2016, Vol. 23 Issue 10, p1566-1571. 6p. 1 Chart, 1 Graph.
Subjects: Neural conduction, Charcot-Marie-Tooth disease, Pathological physiology, Electrophysiology, Ulnar nerve
Abstract: Background and purpose Charcot-Marie-Tooth disease (CMT) type 1A is characterized by uniformly reduced nerve conduction velocity (NCV) that is fully penetrant since the first years of life, remains fairly stable through the life and does not correlate with disability whereas compound muscular action potential (CMAP) amplitude does. The aim of the present study was to analyze the large amount of electrophysiological data collected in the ascorbic acid trial in Italy and the UK (CMT-TRIAAL/CMT-TRAUK) and to use these data to gain insights into the pathophysiology of NCV in CMT1A. Methods Baseline electrophysiological data from 271 patients were analysed. Electrophysiological recordings were taken from the motor ulnar, median and peroneal nerves and the sensory ulnar nerve. Distal motor latency ( DML), motor ( MNCV) and sensory ( SNCV) nerve conduction velocity, and amplitudes of CMAPs and sensory action potentials were assessed. Electrophysiological findings were correlated with age of patients at examination and the Charcot-Marie−Tooth Examination Score ( CMTES). Results NCV was markedly and uniformly reduced. CMAP amplitudes were overall reduced but more severely in lower limbs. DML decreased and MNCV and SNCV increased with age of the patients, whereas CMAP amplitudes worsened with age and also correlated with CMTES. Conclusions This is the largest sample of electrophysiological data obtained so far from CMT1A patients. Axonal degeneration as assessed by means of CMAP amplitude reflected clinical impairment and was consistent with a slowly progressive length-dependent neuropathy. All patients typically had markedly slowed NCV that did, however, slightly increase with age of the patients. The improvement of NCV might depend on myelin thickness remodelling that occurs during the adult life of CMT1A patients. [ABSTRACT FROM AUTHOR]
Copyright of European Journal of Neurology is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
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  Data: Nerve conduction velocity in CMT1A: what else can we tell?
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  Data: <searchLink fieldCode="AR" term="%22Manganelli%2C+F%2E%22">Manganelli, F.</searchLink><br /><searchLink fieldCode="AR" term="%22Pisciotta%2C+C%2E%22">Pisciotta, C.</searchLink><br /><searchLink fieldCode="AR" term="%22Reilly%2C+M%2E+M%2E%22">Reilly, M. M.</searchLink><br /><searchLink fieldCode="AR" term="%22Tozza%2C+S%2E%22">Tozza, S.</searchLink><br /><searchLink fieldCode="AR" term="%22Schenone%2C+A%2E%22">Schenone, A.</searchLink><br /><searchLink fieldCode="AR" term="%22Fabrizi%2C+G%2E+M%2E%22">Fabrizi, G. M.</searchLink><br /><searchLink fieldCode="AR" term="%22Cavallaro%2C+T%2E%22">Cavallaro, T.</searchLink><br /><searchLink fieldCode="AR" term="%22Vita%2C+G%2E%22">Vita, G.</searchLink><br /><searchLink fieldCode="AR" term="%22Padua%2C+L%2E%22">Padua, L.</searchLink><br /><searchLink fieldCode="AR" term="%22Gemignani%2C+F%2E%22">Gemignani, F.</searchLink><br /><searchLink fieldCode="AR" term="%22Laurà%2C+M%2E%22">Laurà, M.</searchLink><br /><searchLink fieldCode="AR" term="%22Hughes%2C+R%2E+A%2E+C%2E%22">Hughes, R. A. C.</searchLink><br /><searchLink fieldCode="AR" term="%22Solari%2C+A%2E%22">Solari, A.</searchLink><br /><searchLink fieldCode="AR" term="%22Pareyson%2C+D%2E%22">Pareyson, D.</searchLink><br /><searchLink fieldCode="AR" term="%22Santoro%2C+L%2E%22">Santoro, L.</searchLink><br /><searchLink fieldCode="AR" term="%22Nolano%2C+Maria%22">Nolano, Maria</searchLink><br /><searchLink fieldCode="AR" term="%22Iodice%2C+Rosa%22">Iodice, Rosa</searchLink><br /><searchLink fieldCode="AR" term="%22Grandis%2C+Marina%22">Grandis, Marina</searchLink><br /><searchLink fieldCode="AR" term="%22Cabrini%2C+Ilaria%22">Cabrini, Ilaria</searchLink><br /><searchLink fieldCode="AR" term="%22Bertolasi%2C+Laura%22">Bertolasi, Laura</searchLink>
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  Data: <searchLink fieldCode="JN" term="%22European+Journal+of+Neurology%22">European Journal of Neurology</searchLink>. Oct2016, Vol. 23 Issue 10, p1566-1571. 6p. 1 Chart, 1 Graph.
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  Data: <searchLink fieldCode="DE" term="%22Neural+conduction%22">Neural conduction</searchLink><br /><searchLink fieldCode="DE" term="%22Charcot-Marie-Tooth+disease%22">Charcot-Marie-Tooth disease</searchLink><br /><searchLink fieldCode="DE" term="%22Pathological+physiology%22">Pathological physiology</searchLink><br /><searchLink fieldCode="DE" term="%22Electrophysiology%22">Electrophysiology</searchLink><br /><searchLink fieldCode="DE" term="%22Ulnar+nerve%22">Ulnar nerve</searchLink>
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  Data: Background and purpose Charcot-Marie-Tooth disease (CMT) type 1A is characterized by uniformly reduced nerve conduction velocity (NCV) that is fully penetrant since the first years of life, remains fairly stable through the life and does not correlate with disability whereas compound muscular action potential (CMAP) amplitude does. The aim of the present study was to analyze the large amount of electrophysiological data collected in the ascorbic acid trial in Italy and the UK (CMT-TRIAAL/CMT-TRAUK) and to use these data to gain insights into the pathophysiology of NCV in CMT1A. Methods Baseline electrophysiological data from 271 patients were analysed. Electrophysiological recordings were taken from the motor ulnar, median and peroneal nerves and the sensory ulnar nerve. Distal motor latency ( DML), motor ( MNCV) and sensory ( SNCV) nerve conduction velocity, and amplitudes of CMAPs and sensory action potentials were assessed. Electrophysiological findings were correlated with age of patients at examination and the Charcot-Marie−Tooth Examination Score ( CMTES). Results NCV was markedly and uniformly reduced. CMAP amplitudes were overall reduced but more severely in lower limbs. DML decreased and MNCV and SNCV increased with age of the patients, whereas CMAP amplitudes worsened with age and also correlated with CMTES. Conclusions This is the largest sample of electrophysiological data obtained so far from CMT1A patients. Axonal degeneration as assessed by means of CMAP amplitude reflected clinical impairment and was consistent with a slowly progressive length-dependent neuropathy. All patients typically had markedly slowed NCV that did, however, slightly increase with age of the patients. The improvement of NCV might depend on myelin thickness remodelling that occurs during the adult life of CMT1A patients. [ABSTRACT FROM AUTHOR]
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  Data: <i>Copyright of European Journal of Neurology is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract.</i> (Copyright applies to all Abstracts.)
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