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.
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) polyneuropathypatients and carriers. PATIENTS AND METHODS: We regularly performed different clinical scales, nerve conductions studies (NCS), and Sudoscan on a cohort of hATTR amyloidosispatients and carriers from a single centre of central Italy, a non-endemic area, in the last 2 years. RESULTS: About 18 hATTR amyloidosispatients 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 amyloidosispatients and we suggest its possible utility in early detection of disease in this population.
Entities:
Keywords:
Sudoscan; TTR; amyloid; carrier; neuropathy; patient; small fibre
Authors: Marco Luigetti; Valeria Guglielmino; Marina Romozzi; Angela Romano; Andrea Di Paolantonio; Giulia Bisogni; Eleonora Sabatelli; Anna Modoni; Mario Sabatelli; Serenella Servidei; Mauro Lo Monaco Journal: Brain Sci Date: 2022-08-04
Authors: Simone Bellavia; Irene Scala; Marco Luigetti; Valerio Brunetti; Maurizio Gabrielli; Lorenzo Zileri Dal Verme; Serenella Servidei; Paolo Calabresi; Giovanni Frisullo; Giacomo Della Marca Journal: J Clin Med Date: 2021-12-14 Impact factor: 4.241