1. T2-relaxometry in a large cohort of hereditary transthyretin amyloidosis with polyneuropathy.
- Author
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Poncelet, Anysia, Hegenbart, Ute, Schönland, Stefan O., Sam, Georges, Purrucker, Jan C., Hund, Ernst, aus dem Siepen, Fabian, Göldner, Kira, Hayes, John M., Heiland, Sabine, Bendszus, Martin, Weiler, Markus, and Hayes, Jennifer C.
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MAGNETIC resonance neurography , *NERVE conduction studies , *PERONEAL nerve , *PERIPHERAL nervous system , *TIBIAL nerve - Abstract
AbstractBackgroundMethodsResultsConclusionPreviously, 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 asymptomaticTTRv -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) versusTTRv- 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 differentiatedTTRv- carriers from controls. ρ and CSA correlated with NCS inTTRv -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 asymptomaticTTRv -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. [ABSTRACT FROM AUTHOR]- Published
- 2024
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