The value of electrochemical skin conductance measurement using Sudoscan® in the assessment of patients with familial amyloid polyneuropathy

Publication date: August 2018
Source:Clinical Neurophysiology, Volume 129, Issue 8
Author(s): Jean-Pascal Lefaucheur, Hela G. Zouari, Farida Gorram, Tarik Nordine, Thibaud Damy, Violaine Planté-Bordeneuve
ObjectiveTo reappraise the value of electrochemical skin conductance (ESC) measurement by Sudoscan® to assess the distal involvement of small autonomic fibers in familial amyloid polyneuropathy (FAP) due to various transthyretin (TTR) mutations.MethodsESC was measured at both hands and feet in 126 patients with either Val30Met (n = 65) or non-Val30Met (n = 61) TTR mutation. This series included clinically asymptomatic (n = 21) and paucisymptomatic (n = 30) patients, as well as patients with moderate (n = 37) or advanced (n = 38) TTR-FAP.ResultsESC measures did not differ between patients according to the type of TTR variant and were reduced in 24% of clinically asymptomatic patients, 40% of paucisymptomatic patients, 65% of patients with moderate TTR-FAP, and 92% of patients with advanced TTR-FAP. ESC measures were found to correlate with patients' clinical status, especially assessed by the Neuropathy Impairment Score and Karnofsky Performance Status.ConclusionESC measures well correlate with the severity of TTR-FAP and could provide early marker of the disease.SignificanceESC measures appear to be relevant to evaluate distal autonomic involvement in the context of amyloidosis.



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