TY - JOUR
T1 - Progressive brachial plexus enlargement in hereditary transthyretin amyloidosis
AU - Salvalaggio, Alessandro
AU - Coraci, Daniele
AU - Obici, Laura
AU - Cacciavillani, Mario
AU - Luigetti, Marco
AU - Mazzeo, Anna
AU - Pastorelli, Francesca
AU - Grandis, Marina
AU - Cavallaro, Tiziana
AU - Bisogni, Giulia
AU - Lozza, Alessandro
AU - Gemelli, Chiara
AU - Gentile, Luca
AU - Russo, Massimo
AU - Ermani, Mario
AU - Fabrizi, Gian Maria
AU - Plasmati, Rosaria
AU - De Napoli, Federica
AU - Campagnolo, Marta
AU - Castellani, Francesca
AU - Salvi, Fabrizio
AU - Fenu, Silvia
AU - Devigili, Grazia
AU - Pareyson, Davide
AU - Gasparotti, Roberto
AU - Rapezzi, Claudio
AU - Martinoli, Carlo
AU - Padua, Luca
AU - Briani, Chiara
N1 - Funding Information:
Alessandro Salvalaggio reports travel grants from Akcea, Alnylam and Pfizer and consulting honoraria from Alnylam. Laura Obici reports speaker and consulting honoraria from Akcea, Alnylam and Pfizer. Marco Luigetti reports financial grants (honoraria and speaking) from Akcea, Alnylam and Pfizer, and travel grants from Pfizer, Kedrion and Grifols. Gulia Bisogni reports financial grants (honoraria and speaking) from Alnylam and travel grants from Pfizer and Grifols. Gian Maria Fabrizi reports consulting honoraria from Akcea and Alnylam, and travel grants from Kedrion and Alnylam. Chiara Gemelli reports travel grants from Akcea and Pfizer. Carlo Martinoli reports financial relationships (consultant, speaker fees, adv board) with Pfizer, Novartis, Sobi, Takeda and Novonordisk. Chiara Briani reports speaker and consulting honoraria from Akcea, Alnylam and Pfizer, and travel grants from Kedrion, Alnylam and CSL Behring. Davide Pareyson reports financial grants (honoraria and speaking) from Alnylam, Akcea, Pfizer, and Inflectis, and travel grants from Kedrion and Pfizer. Silvia Fenu received financial grants (honoraria and speaking) from Alnylam, Akcea, and Pfizer and travel grants from Alnylam and Akcea. Luca Gentile is sub-investigator in clinical trials of Alnylam, Ionis, Takeda and reports travel grants from Kedrion and CSL Behring financial grants (consulting and speaking) from Pfizer.
Funding Information:
Open access funding provided by Università degli Studi di Padova within the CRUI-CARE Agreement. The present study was supported by an Investigator-Initiated Research to Azienda Ospedale Università of Padova from Pfizer Inc. Pfizer Inc had no role in the study design, data analysis, and results interpretation of the present study.
Publisher Copyright:
© 2021, The Author(s).
PY - 2021
Y1 - 2021
N2 - Axonal polyneuropathy is the main feature of hereditary transthyretin amyloidosis (ATTRv). Nerve morphological abnormalities have been reported, but longitudinal changes have never been assessed. We performed a prospective widespread nerve ultrasound evaluation and nerve cross-sectional area (CSA) was compared with baseline data in both ATTRv patients and pre-symptomatic carriers. Thirty-eight subjects were evaluated (mean follow-up 17.1 months), among them 21 had polyneuropathy while 17 were pre-symptomatic carriers. CSA significantly increased at brachial plexus in both groups (p = 0.008 and p = 0.012) pointing to progressive brachial plexus enlargement as a longitudinal biomarker of both disease progression and disease occurrence in pre-symptomatic carriers.
AB - Axonal polyneuropathy is the main feature of hereditary transthyretin amyloidosis (ATTRv). Nerve morphological abnormalities have been reported, but longitudinal changes have never been assessed. We performed a prospective widespread nerve ultrasound evaluation and nerve cross-sectional area (CSA) was compared with baseline data in both ATTRv patients and pre-symptomatic carriers. Thirty-eight subjects were evaluated (mean follow-up 17.1 months), among them 21 had polyneuropathy while 17 were pre-symptomatic carriers. CSA significantly increased at brachial plexus in both groups (p = 0.008 and p = 0.012) pointing to progressive brachial plexus enlargement as a longitudinal biomarker of both disease progression and disease occurrence in pre-symptomatic carriers.
KW - Amyloidosis
KW - Brachial plexus
KW - Peripheral nerves
KW - Transthyretin
KW - Ultrasound
UR - http://www.scopus.com/inward/record.url?scp=85112798994&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85112798994&partnerID=8YFLogxK
U2 - 10.1007/s00415-021-10754-9
DO - 10.1007/s00415-021-10754-9
M3 - Article
AN - SCOPUS:85112798994
SN - 0340-5354
JO - Journal of Neurology
JF - Journal of Neurology
ER -