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

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...

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Main Authors: Poncelet, Anysia (Author) , Hegenbart, Ute (Author) , Schönland, Stefan (Author) , Sam, Georges (Author) , Purrucker, Jan (Author) , Hund, Ernst (Author) , Siepen, Fabian aus dem (Author) , Göldner, Kira (Author) , Hayes, John M. (Author) , Heiland, Sabine (Author) , Bendszus, Martin (Author) , Weiler, Markus (Author) , Hayes, Jennifer (Author)
Format: Article (Journal)
Language:English
Published: 2024
In: Amyloid
Year: 2024, Pages: 1-9
ISSN:1744-2818
DOI:10.1080/13506129.2024.2398453
Online Access:Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1080/13506129.2024.2398453
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Author Notes: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
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Summary: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.
Item Description:Gesehen am 23.09.2024
Physical Description:Online Resource
ISSN:1744-2818
DOI:10.1080/13506129.2024.2398453

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