MR neurography biomarkers to characterize peripheral neuropathy in AL amyloidosis

• Published in: Neurology Vol. 91; no. 7; pp. e625 - e634
• Main Authors: Kollmer, Jennifer, Weiler, Markus, Purrucker, Jan, Heiland, Sabine, Schönland, Stefan O, Hund, Ernst, Kimmich, Christoph, Hayes, John M, Hilgenfeld, Tim, Pham, Mirko, Bendszus, Martin, Hegenbart, Ute
• Format: Journal Article
• Language: English
• Published: United States American Academy of Neurology 14-08-2018
• Subjects: 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
• ISSN: 0028-3878; 1526-632X
• DOI: 10.1212/WNL.0000000000006002

OBJECTIVETo 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.
METHODSWe 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.
RESULTSNerve T2-weighted signal increase correlated with disease severitymoderate (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 (tibial525.5 ± 53.0; peroneal553.6 ± 64.5; sural492.0 ± 56.6) and mild AL-PNP (tibial431.6 ± 22.0; peroneal457.6 ± 21.7; sural404.8 ± 25.2) vs controls (tibial310.5 ± 14.1; peroneal313.6 ± 11.6; sural261.7 ± 11.0; p < 0.0001 for all nerves). T2 relaxation time was elevated in moderate AL-PNP only (tibialp = 0.0106; peronealp = 0.0070; suralp = 0.0190). Tibial nerve caliber was higher in moderate (58.0 ± 8.8 mm) vs mild AL-PNP (46.5 ± 2.5 mm; p = 0.008) vs controls (39.1 ± 1.2 mm; p < 0.0001).
CONCLUSIONSMRN 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.