Magnetization transfer ratio quantifies polyneuropathy in hereditary transthyretin amyloidosis

Autor: 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
Jazyk: angličtina
Rok vydání: 2020
Předmět:
Zdroj: Annals of Clinical and Translational Neurology, Vol 7, Iss 5, Pp 799-807 (2020)
Druh dokumentu: article
ISSN: 2328-9503
DOI: 10.1002/acn3.51049
Popis: 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
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