T2-relaxometry in a large cohort of hereditary transthyretin amyloidosis with polyneuropathy

• Verfasst von: Poncelet, Anysia [VerfasserIn]
• Verfasst von: Hegenbart, Ute [VerfasserIn]
• Verfasst von: Schönland, Stefan [VerfasserIn]
• Verfasst von: Sam, Georges [VerfasserIn]
• Verfasst von: Purrucker, Jan [VerfasserIn]
• Verfasst von: Hund, Ernst [VerfasserIn]
• Verfasst von: Siepen, Fabian aus dem [VerfasserIn]
• Verfasst von: Göldner, Kira [VerfasserIn]
• Verfasst von: Hayes, John M. [VerfasserIn]
• Verfasst von: Heiland, Sabine [VerfasserIn]
• Verfasst von: Bendszus, Martin [VerfasserIn]
• Verfasst von: Weiler, Markus [VerfasserIn]
• Verfasst von: Hayes, Jennifer [VerfasserIn]
• Titel: T2-relaxometry in a large cohort of hereditary transthyretin amyloidosis with polyneuropathy
• Verf.angabe: Anysia Poncelet, Ute Hegenbart, Stefan O. Schönland, Georges Sam, Jan C. Purrucker, Ernst Hund, Fabian aus dem Siepen, Kira Göldner, John M. Hayes, Sabine Heiland, Martin Bendszus, Markus Weiler, Jennifer C. Hayes
• Jahr: 2024
• Umfang: 9 S.
• Fussnoten: Gesehen am 23.09.2024
• Titel Quelle: Enthalten in: Amyloid
• Ort Quelle: Abingdon : Taylor & Francis Group, 1994
• Jahr Quelle: 2024
• Band/Heft Quelle: (2024), Seite 1-9
• ISSN Quelle: 1744-2818
• DOI: doi:10.1080/13506129.2024.2398453
• Datenträger: Online-Ressource
• Sprache: eng
• Sach-SW: Apparent T2-relaxation time
• Sach-SW: hereditary transthyretin amyloidosis
• Sach-SW: MR neurography
• Sach-SW: proton spin density
• Sach-SW: T2-relaxometry
• K10plus-PPN: 1903199018

Abstract

Previously, T2-relaxation time (T2app) and proton spin density (ρ) detected nerve injury in a small group of ATTRv amyloidosis. Here, we aim to quantify peripheral nerve impairment in a large cohort of symptomatic and asymptomatic ATTRv amyloidosis and correlate T2-relaxometry markers with clinical parameters and nerve conduction studies (NCS). Eighty participants with pathologic variants of the transthyretin gene (TTRv) and 40 controls prospectively underwent magnetic resonance neurography. T2-relaxometry was performed, allowing to calculate tibial ρ, T2app and cross-sectional-area (CSA). Detailed clinical examinations and NCS of tibial and peroneal nerves were performed. Forty participants were classified as asymptomatic TTRv-carriers, 40 as symptomatic patients with polyneuropathy. ρ, T2app and CSA were significantly higher in symptomatic ATTRv amyloidosis (484.2 ± 14.8 a.u.; 70.6 ± 1.8 ms; 25.7 ± 0.9 mm2) versus TTRv-carriers (413.1 ± 9.4 a.u., p < 0.0001; 62.3 ± 1.3 ms, p = 0.0002; 19.0 ± 0.8 mm2, p < 0.0001) and versus controls (362.6 ± 7.5 a.u., p < 0.0001; 59.5 ± 1.0 ms, p < 0.0001; 15.4 ± 0.5 mm2, p < 0.0001). Only ρ and CSA differentiated TTRv-carriers from controls. ρ and CSA correlated with NCS in TTRv-carriers, while T2app correlated with NCS in symptomatic ATTRv amyloidosis. Both ρ and T2app correlated with clinical score. ρ and CSA can detect early nerve injury and correlate with electrophysiology in asymptomatic TTRv-carriers. T2app increases only in symptomatic ATTRv amyloidosis in whom it correlates with clinical scores and electrophysiology. Our results suggest that T2-relaxometry can provide biomarkers for disease- and therapy-monitoring in the future.