Jennifer Kollmer1, Markus Weiler2, Jan Purrucker2, Sabine Heiland2, Stefan O Schönland2, Ernst Hund2, Christoph Kimmich2, John M Hayes2, Tim Hilgenfeld2, Mirko Pham2, Martin Bendszus2, Ute Hegenbart2. 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. jennifer.kollmer@med.uni-heidelberg.de. 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.
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.
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.
Authors: Adriana M Pietsch; Andrea Viehöver; Ricarda Diem; Markus Weiler; Mirjam Korporal-Kuhnke; Brigitte Wildemann; Georges Sam; John M Hayes; Olivia Fösleitner; Johann M E Jende; Sabine Heiland; Martin Bendszus; Jennifer C Hayes Journal: Clin Neuroradiol Date: 2022-10-20 Impact factor: 3.156
Authors: Tim W Rattay; Jennifer Just; Benjamin Röben; Holger Hengel; Rebecca Schüle; Matthis Synofzik; Anne S Söhn; Natalie Winter; Nele Dammeier; Ludger Schöls; Alexander Grimm Journal: Orphanet J Rare Dis Date: 2018-11-03 Impact factor: 4.123
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Authors: 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 Journal: Ann Clin Transl Neurol Date: 2020-04-25 Impact factor: 4.511