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Quantitative sudomotor test helps differentiate Transthyretin familial amyloid polyneuropathy from Chronic Inflammatory Demyelinating Polyneuropathy by Fortanier, E.; Delmont, E.; Verschueren, A; Attarian, S. is a Neuroscience article available to read on EtoBox.

What is Quantitative sudomotor test helps differentiate Transthyretin familial amyloid polyneuropathy from Chronic Inflammatory Demyelinating Polyneuropathy about?

## Objective: Transthyretin familial amyloid polyneuropathy (ttr-fap) is an aggressive hereditary neuropathy characterized by sensory and autonomic dysfunction. there are numerous reports of ttr-fap misdiagnosed and treated as chronic inflammatory demyelinating polyneuropathy (cidp), leading to delayed diagnosis, risk of iatrogenic adverse events and increased socio-economic costs. quantitative sudomotor function measured by electrochemical skin conductance (esc) appears to be a sensitive test in ttr-fap. we aimed to evaluate the performance of esc in differentiating ttr-fap from cidp. ## Methods: Thirty-eight patients with genetically confirmed hereditary ttr amyloidosis and 26 with definite cidp according to the efns/pns guidelines and negative ttr-fap genetic testing were involved in this study. we compared the esc for feet and hands measured by sudoscan for each patient. ## Results: Esc (μs) was significantly lower in ttr-fap for both hands (72 vs 45, p < 0.0001) and feet (77 vs 35, p < 0.0001). feet esc < 64 μs had a 89% sensitivity and a 96% specificity to differentiate between cidp and ttr-fap. ## Conclusion: Sudoscan is a fast, non-invasive and easy to perform test, able to

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Author

Fortanier, E.; Delmont, E.; Verschueren, A; Attarian, S.

Publisher

Elsevier Science; Elsevier ; Elsevier BV (ISSN 1388-2457)

Published

2020

Language

EN

Field

Neuroscience (Life Sciences)