MR neurography biomarkers to characterize peripheral neuropathy in AL amyloidosis

Jennifer Kollmer; Markus Weiler; Jan Purrucker; Sabine Heiland; Stefan O Schönland; Ernst Hund; Christoph Kimmich; John M Hayes; Tim Hilgenfeld; Mirko Pham; Martin Bendszus; Ute Hegenbart

doi:10.1212/WNL.0000000000006002

MR neurography biomarkers to characterize peripheral neuropathy in AL amyloidosis

Neurology. 2018 Aug 14;91(7):e625-e634. doi: 10.1212/WNL.0000000000006002. Epub 2018 Jul 20.

Affiliations

¹ From the Department of Neuroradiology (J.K., S.H., T.H., M.P., M.B.), Amyloidosis Center Heidelberg (J.K., M.W., J.P., S.O.S., E.H., C.K., U.H.), Department of Neurology (M.W., J.P., E.H.), Division of Experimental Radiology, Department of Neuroradiology (S.H.), and Medical Department V (S.O.S., C.K., U.H.), Heidelberg University Hospital, Germany; Department of Neurology (J.M.H.), University of Michigan, Ann Arbor; and Department of Neuroradiology (M.P.), Würzburg University Hospital, Germany. [email protected].
² From the Department of Neuroradiology (J.K., S.H., T.H., M.P., M.B.), Amyloidosis Center Heidelberg (J.K., M.W., J.P., S.O.S., E.H., C.K., U.H.), Department of Neurology (M.W., J.P., E.H.), Division of Experimental Radiology, Department of Neuroradiology (S.H.), and Medical Department V (S.O.S., C.K., U.H.), Heidelberg University Hospital, Germany; Department of Neurology (J.M.H.), University of Michigan, Ann Arbor; and Department of Neuroradiology (M.P.), Würzburg University Hospital, Germany.

PMID: 30030328
DOI: 10.1212/WNL.0000000000006002

Abstract

Objective: To detect, localize, and quantify peripheral nerve lesions in amyloid light chain (AL) amyloidosis by magnetic resonance neurography (MRN) in correlation with clinical and electrophysiologic findings.

Methods: We prospectively examined 20 patients with AL-polyneuropathy (PNP) and 25 age- and sex-matched healthy volunteers. After detailed neurologic and electrophysiologic testing, the patient group was subdivided into mild and moderate PNP. MRN in a 3.0 tesla scanner with anatomical coverage from the lumbosacral plexus and proximal thigh down to the tibiotalar joint was performed by using T2-weighted and dual-echo 2-dimensional sequences with spectral fat saturation and a 3-dimensional, T2-weighted inversion recovery sequence. Besides evaluation of nerve T2-weighted signal, detailed quantification of nerve injury by morphometric (nerve caliber) and microstructural MRN markers (proton spin density, T2 relaxation time) was conducted.

Results: Nerve T2-weighted signal increase correlated with disease severity: moderate (420.2 ± 60.1) vs mild AL-PNP (307.2 ± 17.9; p = 0.0003) vs controls (207.0 ± 6.4; p < 0.0001). Proton spin density was also higher in moderate (tibial: 525.5 ± 53.0; peroneal: 553.6 ± 64.5; sural: 492.0 ± 56.6) and mild AL-PNP (tibial: 431.6 ± 22.0; peroneal: 457.6 ± 21.7; sural: 404.8 ± 25.2) vs controls (tibial: 310.5 ± 14.1; peroneal: 313.6 ± 11.6; sural: 261.7 ± 11.0; p < 0.0001 for all nerves). T2 relaxation time was elevated in moderate AL-PNP only (tibial: p = 0.0106; peroneal: p = 0.0070; sural: p = 0.0190). Tibial nerve caliber was higher in moderate (58.0 ± 8.8 mm³) vs mild AL-PNP (46.5 ± 2.5 mm³; p = 0.008) vs controls (39.1 ± 1.2 mm³; p < 0.0001).

Conclusions: MRN detects and quantifies peripheral nerve injury in AL-PNP in vivo with high sensitivity and in close correlation with the clinical stage. Quantitative parameters are feasible new imaging biomarkers for the detection of early AL-PNP and might help to monitor microstructural nerve tissue changes under treatment.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Adult
Aged
Analysis of Variance
Case-Control Studies
Evoked Potentials, Motor
Female
Ganglia, Spinal / pathology
Humans
Imaging, Three-Dimensional
Immunoglobulin Light-chain Amyloidosis / complications*
Magnetic Resonance Imaging*
Male
Middle Aged
Polyneuropathies / diagnostic imaging*
Polyneuropathies / etiology*
Protons
Severity of Illness Index

Substances

Protons