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

Familial amyloid polyneuropathy (FAP) due to transthyretin (TTR) mutation results from an irreversible extracellular fibril protein deposits caused by mutated TTR and is a length-dependent small-fiber predominant neuropathy (Planté-Bordeneuve and Said, 2011). Because of this characteristic, the evaluation of the autonomic cutaneous innervation of the extremities by unmyelinated C fibers is particularly relevant in this disease. A few studies addressed this issue. At distal limb level, sudomotor innervation was assessed in patients with TTR-FAP by sympathetic skin reflex recording (Montagna et al., 1996; Alves et al., 1997; Shivji and Ashby, 1999; Conceição et al., 2008, 2014; Lefaucheur et al., 2013, 2015; Castro et al., 2016), quantitative sudomotor axon reflex testing (QSART) (Wang et al., 2008; Kim et al., 2009), and sweat gland quantitation in skin biopsy (Chao et al., 2015).

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