Magnetization transfer ratio quantifies polyneuropathy in hereditary transthyretin amyloidosis

• Verfasst von: Hayes, Jennifer [VerfasserIn]
• Verfasst von: Hegenbart, Ute [VerfasserIn]
• Verfasst von: Kimmich, Christoph [VerfasserIn]
• Verfasst von: Hund, Ernst [VerfasserIn]
• Verfasst von: Purrucker, Jan [VerfasserIn]
• Verfasst von: Hayes, John M. [VerfasserIn]
• Verfasst von: Lentz, Stephen I. [VerfasserIn]
• Verfasst von: Sam, Georges [VerfasserIn]
• Verfasst von: Jende, Johann [VerfasserIn]
• Verfasst von: Schönland, Stefan [VerfasserIn]
• Verfasst von: Bendszus, Martin [VerfasserIn]
• Verfasst von: Heiland, Sabine [VerfasserIn]
• Verfasst von: Weiler, Markus [VerfasserIn]
• Titel: Magnetization transfer ratio quantifies polyneuropathy in hereditary transthyretin amyloidosis
• Verf.angabe: Jennifer Kollmer, Ute Hegenbart, Christoph Kimmich, Ernst Hund, Jan C. Purrucker, John M. Hayes, Stephen I. Lentz, Georges Sam, Johann M. E. Jende, Stefan O. Schönland, Martin Bendszus, Sabine Heiland & Markus Weiler
• Jahr: 2020
• Umfang: 9 S.
• Fussnoten: First published: 25 April 2020 ; Gesehen am 14.09.2021
• Titel Quelle: Enthalten in: Annals of Clinical and Translational Neurology
• Ort Quelle: Chichester [u.a.] : Wiley, 2013
• Jahr Quelle: 2020
• Band/Heft Quelle: 7(2020), 5, Seite 799-807
• ISSN Quelle: 2328-9503
• DOI: doi:10.1002/acn3.51049
• Datenträger: Online-Ressource
• Sprache: eng
• K10plus-PPN: 177011145X
• K10plus-PPN:
  Universitätsbibliothek
• Lokale URL UB: Zum Volltext

Abstract

Objective To quantify peripheral nerve lesions in symptomatic and asymptomatic hereditary transthyretin amyloidosis with polyneuropathy (ATTRv-PNP) by analyzing the magnetization transfer ratio (MTR) of the sciatic nerve, and to test its potential as a novel biomarker for macromolecular changes. Methods Twenty-five patients with symptomatic ATTRv-PNP, 30 asymptomatic carriers of the mutant transthyretin gene (mutTTR), and 20 age-/sex-matched healthy controls prospectively underwent magnetization transfer contrast imaging at 3 Tesla. Two axial three-dimensional gradient echo sequences with and without an off-resonance saturation rapid frequency pulse were conducted at the right distal thigh. Sciatic nerve regions of interest were manually drawn on 10 consecutive axial slices in the images without off-resonance saturation, and then transferred to the corresponding slices that were generated by the sequence with the off-resonance saturation pulse. Subsequently, the MTR and cross-sectional area (CSA) of the sciatic nerve were evaluated. Detailed neurologic and electrophysiologic examinations were conducted in all ATTRv-PNP patients and mutTTR-carriers. Results Sciatic nerve MTR and CSA reliably differentiated between ATTRv-PNP, mutTTR-carriers, and controls. MTR was lower in ATTRv-PNP (26.4 ± 0.7; P < 0.0001) and in mutTTR-carriers (32.6 ± 0.8; P = 0.0005) versus controls (39.4 ± 2.1), and was also lower in ATTRv-PNP versus mutTTR-carriers (P = 0.0009). MTR correlated negatively with the NIS-LL and positively with CMAPs and SNAPs. CSA was higher in ATTRv-PNP (34.3 ± 1.7 mm3) versus mutTTR-carriers (26.0 ± 1.1 mm3; P = 0.0005) and versus controls (20.4 ± 1.2 mm3; P < 0.0001). CSA was also higher in mutTTR-carriers versus controls. Interpretation MTR is a novel imaging marker that can quantify macromolecular changes in ATTRv-PNP and differentiate between symptomatic ATTRv-PNP and asymptomatic mutTTR-carriers and correlates with electrophysiology.