Sudoscan in the evaluation and follow-up of patients and carriers with TTR mutations: experience from an Italian Centre

Andrea Di Paolantonio, Serenella Servidei, Mario Sabatelli, Marco Luigetti, Angela Romano, Francesco Barbato, Paolo Maria Rossini, Giulia Bisogni

Risultato della ricerca: Contributo in rivistaArticolo in rivistapeer review

8 Citazioni (Scopus)

Abstract

Objective: To evaluate the utility of Sudoscan as possible marker of disease progression and disease onset in a cohort of hereditary ATTR amyloidosis (hATTR amyloidosis) polyneuropathy patients and carriers. Patients and methods: We regularly performed different clinical scales, nerve conductions studies (NCS), and Sudoscan on a cohort of hATTR amyloidosis patients and carriers from a single centre of central Italy, a non-endemic area, in the last 2 years. Results: About 18 hATTR amyloidosis patients and 8 asymptomatic carriers were enrolled. All patients had a neuropathy affecting large fibres, small fibres or both. Two subjects developed symptoms and neurophysiological alterations during follow-up. Sudoscan data from hand and feet inversely correlated with neuropathy severity and with disease duration. Moreover, global disease status, expressed by Kumamoto scale also inversely correlated with Sudoscan values. Conclusions: We confirmed that Sudoscan is a reliable marker of disease progression in late-onset hATTR amyloidosis patients and we suggest its possible utility in early detection of disease in this population.
Lingua originaleEnglish
pagine (da-a)242-246
Numero di pagine5
RivistaAMYLOID
Volume25
DOI
Stato di pubblicazionePubblicato - 2018

Keywords

  • Internal Medicine
  • Sudoscan
  • TTR
  • amyloid
  • carrier
  • neuropathy
  • patient
  • small fibre

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