Skeletal muscle MRI differentiates SBMA and ALS and correlates with disease severity.

Autor:	Klickovic U; From the Neuroradiological Academic Unit (C.D.J.S., S.J.W., A.E., S.S., T.A.Y., J.S.T.), and MRC Centre for Neuromuscular Diseases (U.K., L.Z., K.T., R.S.H., N.S., K.S., M.G.H., L.G., J.M.M., P.F.), UCL Queen Square Institute of Neurology, University College London; Blizard Institute (A.M.), Queen Mary University of London, UK; and Department of Radiology (U.K.), University Hospital Tulln, Karl Landsteiner University of Health Sciences, Tulln, Austria., Zampedri L; From the Neuroradiological Academic Unit (C.D.J.S., S.J.W., A.E., S.S., T.A.Y., J.S.T.), and MRC Centre for Neuromuscular Diseases (U.K., L.Z., K.T., R.S.H., N.S., K.S., M.G.H., L.G., J.M.M., P.F.), UCL Queen Square Institute of Neurology, University College London; Blizard Institute (A.M.), Queen Mary University of London, UK; and Department of Radiology (U.K.), University Hospital Tulln, Karl Landsteiner University of Health Sciences, Tulln, Austria., Sinclair CDJ; From the Neuroradiological Academic Unit (C.D.J.S., S.J.W., A.E., S.S., T.A.Y., J.S.T.), and MRC Centre for Neuromuscular Diseases (U.K., L.Z., K.T., R.S.H., N.S., K.S., M.G.H., L.G., J.M.M., P.F.), UCL Queen Square Institute of Neurology, University College London; Blizard Institute (A.M.), Queen Mary University of London, UK; and Department of Radiology (U.K.), University Hospital Tulln, Karl Landsteiner University of Health Sciences, Tulln, Austria., Wastling SJ; From the Neuroradiological Academic Unit (C.D.J.S., S.J.W., A.E., S.S., T.A.Y., J.S.T.), and MRC Centre for Neuromuscular Diseases (U.K., L.Z., K.T., R.S.H., N.S., K.S., M.G.H., L.G., J.M.M., P.F.), UCL Queen Square Institute of Neurology, University College London; Blizard Institute (A.M.), Queen Mary University of London, UK; and Department of Radiology (U.K.), University Hospital Tulln, Karl Landsteiner University of Health Sciences, Tulln, Austria., Trimmel K; From the Neuroradiological Academic Unit (C.D.J.S., S.J.W., A.E., S.S., T.A.Y., J.S.T.), and MRC Centre for Neuromuscular Diseases (U.K., L.Z., K.T., R.S.H., N.S., K.S., M.G.H., L.G., J.M.M., P.F.), UCL Queen Square Institute of Neurology, University College London; Blizard Institute (A.M.), Queen Mary University of London, UK; and Department of Radiology (U.K.), University Hospital Tulln, Karl Landsteiner University of Health Sciences, Tulln, Austria., Howard RS; From the Neuroradiological Academic Unit (C.D.J.S., S.J.W., A.E., S.S., T.A.Y., J.S.T.), and MRC Centre for Neuromuscular Diseases (U.K., L.Z., K.T., R.S.H., N.S., K.S., M.G.H., L.G., J.M.M., P.F.), UCL Queen Square Institute of Neurology, University College London; Blizard Institute (A.M.), Queen Mary University of London, UK; and Department of Radiology (U.K.), University Hospital Tulln, Karl Landsteiner University of Health Sciences, Tulln, Austria., Malaspina A; From the Neuroradiological Academic Unit (C.D.J.S., S.J.W., A.E., S.S., T.A.Y., J.S.T.), and MRC Centre for Neuromuscular Diseases (U.K., L.Z., K.T., R.S.H., N.S., K.S., M.G.H., L.G., J.M.M., P.F.), UCL Queen Square Institute of Neurology, University College London; Blizard Institute (A.M.), Queen Mary University of London, UK; and Department of Radiology (U.K.), University Hospital Tulln, Karl Landsteiner University of Health Sciences, Tulln, Austria., Sharma N; From the Neuroradiological Academic Unit (C.D.J.S., S.J.W., A.E., S.S., T.A.Y., J.S.T.), and MRC Centre for Neuromuscular Diseases (U.K., L.Z., K.T., R.S.H., N.S., K.S., M.G.H., L.G., J.M.M., P.F.), UCL Queen Square Institute of Neurology, University College London; Blizard Institute (A.M.), Queen Mary University of London, UK; and Department of Radiology (U.K.), University Hospital Tulln, Karl Landsteiner University of Health Sciences, Tulln, Austria., Sidle K; From the Neuroradiological Academic Unit (C.D.J.S., S.J.W., A.E., S.S., T.A.Y., J.S.T.), and MRC Centre for Neuromuscular Diseases (U.K., L.Z., K.T., R.S.H., N.S., K.S., M.G.H., L.G., J.M.M., P.F.), UCL Queen Square Institute of Neurology, University College London; Blizard Institute (A.M.), Queen Mary University of London, UK; and Department of Radiology (U.K.), University Hospital Tulln, Karl Landsteiner University of Health Sciences, Tulln, Austria., Emira A; From the Neuroradiological Academic Unit (C.D.J.S., S.J.W., A.E., S.S., T.A.Y., J.S.T.), and MRC Centre for Neuromuscular Diseases (U.K., L.Z., K.T., R.S.H., N.S., K.S., M.G.H., L.G., J.M.M., P.F.), UCL Queen Square Institute of Neurology, University College London; Blizard Institute (A.M.), Queen Mary University of London, UK; and Department of Radiology (U.K.), University Hospital Tulln, Karl Landsteiner University of Health Sciences, Tulln, Austria., Shah S; From the Neuroradiological Academic Unit (C.D.J.S., S.J.W., A.E., S.S., T.A.Y., J.S.T.), and MRC Centre for Neuromuscular Diseases (U.K., L.Z., K.T., R.S.H., N.S., K.S., M.G.H., L.G., J.M.M., P.F.), UCL Queen Square Institute of Neurology, University College London; Blizard Institute (A.M.), Queen Mary University of London, UK; and Department of Radiology (U.K.), University Hospital Tulln, Karl Landsteiner University of Health Sciences, Tulln, Austria., Yousry TA; From the Neuroradiological Academic Unit (C.D.J.S., S.J.W., A.E., S.S., T.A.Y., J.S.T.), and MRC Centre for Neuromuscular Diseases (U.K., L.Z., K.T., R.S.H., N.S., K.S., M.G.H., L.G., J.M.M., P.F.), UCL Queen Square Institute of Neurology, University College London; Blizard Institute (A.M.), Queen Mary University of London, UK; and Department of Radiology (U.K.), University Hospital Tulln, Karl Landsteiner University of Health Sciences, Tulln, Austria., Hanna MG; From the Neuroradiological Academic Unit (C.D.J.S., S.J.W., A.E., S.S., T.A.Y., J.S.T.), and MRC Centre for Neuromuscular Diseases (U.K., L.Z., K.T., R.S.H., N.S., K.S., M.G.H., L.G., J.M.M., P.F.), UCL Queen Square Institute of Neurology, University College London; Blizard Institute (A.M.), Queen Mary University of London, UK; and Department of Radiology (U.K.), University Hospital Tulln, Karl Landsteiner University of Health Sciences, Tulln, Austria., Greensmith L; From the Neuroradiological Academic Unit (C.D.J.S., S.J.W., A.E., S.S., T.A.Y., J.S.T.), and MRC Centre for Neuromuscular Diseases (U.K., L.Z., K.T., R.S.H., N.S., K.S., M.G.H., L.G., J.M.M., P.F.), UCL Queen Square Institute of Neurology, University College London; Blizard Institute (A.M.), Queen Mary University of London, UK; and Department of Radiology (U.K.), University Hospital Tulln, Karl Landsteiner University of Health Sciences, Tulln, Austria., Morrow JM; From the Neuroradiological Academic Unit (C.D.J.S., S.J.W., A.E., S.S., T.A.Y., J.S.T.), and MRC Centre for Neuromuscular Diseases (U.K., L.Z., K.T., R.S.H., N.S., K.S., M.G.H., L.G., J.M.M., P.F.), UCL Queen Square Institute of Neurology, University College London; Blizard Institute (A.M.), Queen Mary University of London, UK; and Department of Radiology (U.K.), University Hospital Tulln, Karl Landsteiner University of Health Sciences, Tulln, Austria., Thornton JS; From the Neuroradiological Academic Unit (C.D.J.S., S.J.W., A.E., S.S., T.A.Y., J.S.T.), and MRC Centre for Neuromuscular Diseases (U.K., L.Z., K.T., R.S.H., N.S., K.S., M.G.H., L.G., J.M.M., P.F.), UCL Queen Square Institute of Neurology, University College London; Blizard Institute (A.M.), Queen Mary University of London, UK; and Department of Radiology (U.K.), University Hospital Tulln, Karl Landsteiner University of Health Sciences, Tulln, Austria. p.fratta@ucl.ac.uk john.thornton@ucl.ac.uk., Fratta P; From the Neuroradiological Academic Unit (C.D.J.S., S.J.W., A.E., S.S., T.A.Y., J.S.T.), and MRC Centre for Neuromuscular Diseases (U.K., L.Z., K.T., R.S.H., N.S., K.S., M.G.H., L.G., J.M.M., P.F.), UCL Queen Square Institute of Neurology, University College London; Blizard Institute (A.M.), Queen Mary University of London, UK; and Department of Radiology (U.K.), University Hospital Tulln, Karl Landsteiner University of Health Sciences, Tulln, Austria. p.fratta@ucl.ac.uk john.thornton@ucl.ac.uk.
Jazyk:	angličtina
Zdroj:	Neurology [Neurology] 2019 Aug 27; Vol. 93 (9), pp. e895-e907. Date of Electronic Publication: 2019 Aug 07.
DOI:	10.1212/WNL.0000000000008009
Abstrakt:	Objective: To investigate the use of muscle MRI for the differential diagnosis and as a disease progression biomarker for 2 major forms of motor neuron disorders: spinal bulbar muscular atrophy (SBMA) and amyotrophic lateral sclerosis (ALS). Methods: We applied quantitative 3-point Dixon and semiquantitative T1-weighted and short tau inversion recovery (STIR) imaging to bulbar and lower limb muscles and performed clinical and functional assessments in ALS (n = 21) and SBMA (n = 21), alongside healthy controls (n = 16). Acquired images were analyzed for the presence of fat infiltration or edema as well as specific patterns of muscle involvement. Quantitative MRI measurements were correlated with clinical measures of disease severity in ALS and SBMA. Results: Quantitative imaging revealed significant fat infiltration in bulbar ( p < 0.001) and limb muscles in SBMA compared to controls (thigh: p < 0.001; calf: p = 0.001), identifying a characteristic pattern of muscle involvement. In ALS, semiquantitative STIR imaging detected marked hyperintensities in lower limb muscles, distinguishing ALS from SBMA and controls. Finally, MRI measurements correlated significantly with clinical scales of disease severity in both ALS and SBMA. Conclusions: Our findings show that muscle MRI differentiates between SBMA and ALS and correlates with disease severity, supporting its use as a diagnostic tool and biomarker for disease progression. This highlights the clinical utility of muscle MRI in motor neuron disorders and contributes to establish objective outcome measures, which is crucial for the development of new drugs. (Copyright © 2019 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.)
Databáze:	MEDLINE
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