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Clin Neurophysiol. 2018 Aug;129(8):1565-1569. doi: 10.1016/j.clinph.2018.05.005. Epub 2018 May 29.

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

Author information

1
Université Paris Est Créteil, Faculté de Médecine, EA 4391 Créteil, France; Assistance Publique - Hôpitaux de Paris, Hôpital Henri Mondor, Unité de Neurophysiologie Clinique, Service de Physiologie, Explorations Fonctionnelles, Créteil, France; Réseau Amylose Henri-Mondor, Hôpital Henri Mondor, Créteil, France. Electronic address: jean-pascal.lefaucheur@hmn.aphp.fr.
2
Université Paris Est Créteil, Faculté de Médecine, EA 4391 Créteil, France; CHU Habib Bourguiba, Service d'Explorations Fonctionnelles, Sfax, Tunisia.
3
Réseau Amylose Henri-Mondor, Hôpital Henri Mondor, Créteil, France; Inserm, IMRB, Hôpital Henri Mondor, Centre d'Investigation Clinique, 1430 Créteil, France.
4
Université Paris Est Créteil, Faculté de Médecine, EA 4391 Créteil, France; Assistance Publique - Hôpitaux de Paris, Hôpital Henri Mondor, Unité de Neurophysiologie Clinique, Service de Physiologie, Explorations Fonctionnelles, Créteil, France.
5
Réseau Amylose Henri-Mondor, Hôpital Henri Mondor, Créteil, France; Assistance Publique - Hôpitaux de Paris, Hôpital Henri Mondor, Unité d'Insuffisance Cardiaque, Service de Cardiologie, Créteil, France; Université Paris Est Créteil, Faculté de Médecine, GRC Institut de Recherche sur l'Amylose, Créteil, France.
6
Réseau Amylose Henri-Mondor, Hôpital Henri Mondor, Créteil, France; Université Paris Est Créteil, Faculté de Médecine, GRC Institut de Recherche sur l'Amylose, Créteil, France; Assistance Publique - Hôpitaux de Paris, Hôpital Henri Mondor, Service de Neurologie, Créteil, France.

Abstract

OBJECTIVE:

To 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.

METHODS:

ESC 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.

RESULTS:

ESC 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.

CONCLUSION:

ESC measures well correlate with the severity of TTR-FAP and could provide early marker of the disease.

SIGNIFICANCE:

ESC measures appear to be relevant to evaluate distal autonomic involvement in the context of amyloidosis.

KEYWORDS:

Amyloidosis; Autonomic nervous system; Early marker; Skin conductance; Small fiber neuropathy; Transthyretin

PMID:
29883834
DOI:
10.1016/j.clinph.2018.05.005
[Indexed for MEDLINE]

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