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.
Nerve conduction velocity in CMT1A: what else can we tell? / Manganelli, Fiore; Pisciotta, Chiara; Reilly, Mm; Tozza, S; Schenone, A; Fabrizi, Gm; Cavallaro, T; Vita, G; Padua, L; Gemignani, F; Laurà, M; Hughes, Ra; Solari, A; Pareyson, D; Santoro, Lucio; Nolano, M; Iodice, Rosa; Grandis, M; Cabrini, I; Bertolasi, L; Mazzeo, A; Majorana, G; Vitetta, F; Scaioli, V; Ciano, C; Calabrese, D; Quattrone, A; Blake, J.. - In: EUROPEAN JOURNAL OF NEUROLOGY. - ISSN 1351-5101. - (2016). [10.1111/ene.13079]
Nerve conduction velocity in CMT1A: what else can we tell?
MANGANELLI, FIORE;PISCIOTTA, CHIARA;Tozza, S;SANTORO, LUCIO;Nolano, M;IODICE, ROSA;
2016
Abstract
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.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
