Exploration of New Contrasts, Targets, and MR Imaging and Spectroscopy Techniques for Neuromuscular Disease – A Workshop Report of Working Group 3 of the Biomedicine and Molecular Biosciences COST Action BM1304 MYO-MRI

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Abstract

Neuromuscular diseases are characterized by progressive muscle degeneration and muscle weakness resulting in functional disabilities. While each of these diseases is individually rare, they are common as a group, and a large majority lacks effective treatment with fully market approved drugs. Magnetic resonance imaging and spectroscopy techniques (MRI and MRS) are showing increasing promise as an outcome measure in clinical trials for these diseases. In 2013, the European Union funded the COST (co-operation in science and technology) action BM1304 called MYO-MRI (www.myo-mri.eu), with the overall aim to advance novel MRI and MRS techniques for both diagnosis and quantitative monitoring of neuromuscular diseases through sharing of expertise and data, joint development of protocols, opportunities for young researchers and creation of an online atlas of muscle MRI and MRS . In this report, the topics that were discussed in the framework of working group 3, which had the objective to: Explore new contrasts, new targets and new imaging techniques for NMD are described. The report is written by the scientists who attended the meetings and presented their data. An overview is given on the different contrasts that MRI can generate and their application, clinical needs and desired readouts, and emerging methods.

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Histological parameters for the quantitative assessment of muscular dystrophy in the mdx-mouse.

Isabelle Courdier Fruh, Alexandre Briguet, Thomas Meier … (2004)

Duchenne muscular dystrophy is a severe X-linked hereditary disease caused by the absence of functional dystrophin. The dystrophin-deficient mdx-mouse strain is a widely used animal model for dystrophin-deficiency. Several therapeutic approaches for muscular dystrophy have been proposed by different laboratories. In order to compare the efficacy of these therapies in the mdx-mouse, it is essential to implement standardized protocols for the assessment of functional and histological parameters in this mouse model. Here, we determine that the minimal 'Feret's diameter' is a geometrical parameter that allows for reliable measure of muscle fiber cross-sectional size. Using this geometrical parameter we calculate variance coefficients of the muscle fiber size and provide reference values for the quantitative assessment of dystrophic symptoms in frequently investigated muscles of wild-type and mdx-mouse. In addition, we compare the variance coefficients of the muscle fiber size with the percentage of muscle fibers with centralized nuclei; another histological hallmark of muscular dystrophy.

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Magnetic resonance elastography by direct visualization of propagating acoustic strain waves.

A Manduca, Richard L. Ehman, William J Greenleaf … (1995)

A nuclear magnetic resonance imaging (MRI) method is presented for quantitatively mapping the physical response of a material to harmonic mechanical excitation. The resulting images allow calculation of regional mechanical properties. Measurements of shear modulus obtained with the MRI technique in gel materials correlate with independent measurements of static shear modulus. The results indicate that displacement patterns corresponding to cyclic displacements smaller than 200 nanometers can be measured. The findings suggest the feasibility of a medical imaging technique for delineating elasticity and other mechanical properties of tissue.

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MRI biomarker assessment of neuromuscular disease progression: a prospective observational cohort study

Jasper M. Morrow, Christopher D. J. Sinclair, Arne Fischmann … (2016)

Summary Background A substantial impediment to progress in trials of new therapies in neuromuscular disorders is the absence of responsive outcome measures that correlate with patient functional deficits and are sensitive to early disease processes. Irrespective of the primary molecular defect, neuromuscular disorder pathological processes include disturbance of intramuscular water distribution followed by intramuscular fat accumulation, both quantifiable by MRI. In pathologically distinct neuromuscular disorders, we aimed to determine the comparative responsiveness of MRI outcome measures over 1 year, the validity of MRI outcome measures by cross-sectional correlation against functionally relevant clinical measures, and the sensitivity of specific MRI indices to early muscle water changes before intramuscular fat accumulation beyond the healthy control range. Methods We did a prospective observational cohort study of patients with either Charcot-Marie-Tooth disease 1A or inclusion body myositis who were attending the inherited neuropathy or muscle clinics at the Medical Research Council (MRC) Centre for Neuromuscular Diseases, National Hospital for Neurology and Neurosurgery, London, UK. Genetic confirmation of the chromosome 17p11·2 duplication was required for Charcot-Marie-Tooth disease 1A, and classification as pathologically or clinically definite by MRC criteria was required for inclusion body myositis. Exclusion criteria were concomitant diseases and safety-related MRI contraindications. Healthy age-matched and sex-matched controls were also recruited. Assessments were done at baseline and 1 year. The MRI outcomes—fat fraction, transverse relaxation time (T2), and magnetisation transfer ratio (MTR)—were analysed during the 12-month follow-up, by measuring correlation with functionally relevant clinical measures, and for T2 and MTR, sensitivity in muscles with fat fraction less than the 95th percentile of the control group. Findings Between Jan 19, 2010, and July 7, 2011, we recruited 20 patients with Charcot-Marie-Tooth disease 1A, 20 patients with inclusion body myositis, and 29 healthy controls (allocated to one or both of the 20-participant matched-control subgroups). Whole muscle fat fraction increased significantly during the 12-month follow-up at calf level (mean absolute change 1·2%, 95% CI 0·5–1·9, p=0·002) but not thigh level (0·2%, −0·2 to 0·6, p=0·38) in patients with Charcot-Marie-Tooth disease 1A, and at calf level (2·6%, 1·3–4·0, p=0·002) and thigh level (3·3%, 1·8–4·9, p=0·0007) in patients with inclusion body myositis. Fat fraction correlated with the lower limb components of the inclusion body myositis functional rating score (ρ=–0·64, p=0·002) and the Charcot-Marie-Tooth examination score (ρ=0·63, p=0·003). Longitudinal T2 and MTR changed consistently with fat fraction but more variably. In muscles with a fat fraction lower than the control group 95th percentile, T2 was increased in patients compared with controls (regression coefficients: inclusion body myositis thigh 4·0 ms [SE 0·5], calf 3·5 ms [0·6]; Charcot-Marie-Tooth 1A thigh 1·0 ms [0·3], calf 2·0 ms [0·3]) and MTR reduced compared with controls (inclusion body myositis thigh −1·5 percentage units [pu; 0·2], calf −1·1 pu [0·2]; Charcot-Marie-Tooth 1A thigh −0·3 pu [0·1], calf −0·7 pu [0·1]). Interpretation MRI outcome measures can monitor intramuscular fat accumulation with high responsiveness, show validity by correlation with conventional functional measures, and detect muscle water changes preceding marked intramuscular fat accumulation. Confirmation of our results in further cohorts with these and other muscle-wasting disorders would suggest that MRI biomarkers might prove valuable in experimental trials. Funding Medical Research Council UK.

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Author and article information

Journal

Journal ID (nlm-ta): J Neuromuscul Dis

Journal ID (iso-abbrev): J Neuromuscul Dis

Journal ID (publisher-id): JND

Title: Journal of Neuromuscular Diseases

Publisher: IOS Press (Nieuwe Hemweg 6B, 1013 BG Amsterdam, The Netherlands )

ISSN (Print): 2214-3599

ISSN (Electronic): 2214-3602

Publication date (Electronic, pub): 30 January 2019

Publication date (Electronic, collection): 2019

Volume: 6

Issue: 1

Pages: 1-30

Affiliations

[a ]Amsterdam UMC, University of Amsterdam , Amsterdam, The Netherlands

[b ]NMR Laboratory, Neuromuscular Investigation Center, Institute of Myology & NMR Laboratory , CEA/DRF/IBFJ/MIRCen, Paris, France

[c ]Aix Marseille University , Marseille, France

[d ]Institute of Cellular Medicine, Newcastle University , Newcastle-upon-Tyne, UK

[e ]Department of Radiology, Leiden University Medical Center , Leiden, the Netherlands

[f ] Vanderbilt University Medical Center , Nashville, USA

[g ]Department of Radiology, Division of Radiological Physics, University Hospital Basel, Basel, Switzerland & Department of Biomedical Engineering, University of Basel , Basel, Switzerland

[h ]University Medical Center Utrecht , Utrecht, the Netherlands

[i ]Radboud University Medical Center , Nijmegen, the Netherlands

[j ] Technical University of Munich , Munich, Germany

[k ] BioEimmision Technology Solutions , Athens, Greece

[l ] New York University School of Medicine , New York, USA

[m ]Institute of Radiology, University Hospital Erlangen , Friedrich-Alexander-Universität Erlangen-Nürnberg (FAU), Erlangen, Germany & Division of Medical Physics in Radiology, German Cancer Research Center (DKFZ), Heidelberg, Germany

[n ]Department of Neurology, Inselspital, Bern University Hospital, University of Bern , Switzerland

[o ]University of Tübingen , Section on Experimental Radiology, Tübingen, Germany

[p ]UCL Institute of Neurology , London, UK

[q ] King’s College London , London, UK

[r ]Université de Strasbourg, Strasbourg, France

[s ] University of Florida , Gainesville, USA

Author notes

[* ]Correspondence to: Gustav J. Strijkers, Amsterdam UMC, University of Amsterdam, Amsterdam, the Netherlands. E-mail: g.j.strijkers@ 123456amc.uva.nl .

Article

Publisher ID: JND180333

DOI: 10.3233/JND-180333

PMC ID: 6398566

PubMed ID: 30714967

SO-VID: c3287f47-3aec-40f6-9409-0cab8b27b058

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This is an open access article distributed under the terms of the Creative Commons Attribution Non-Commercial (CC BY-NC 4.0) License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Exploration of New Contrasts, Targets, and MR Imaging and Spectroscopy Techniques for Neuromuscular Disease – A Workshop Report of Working Group 3 of the Biomedicine and Molecular Biosciences COST Action BM1304 MYO-MRI

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Most cited references 252

Histological parameters for the quantitative assessment of muscular dystrophy in the mdx-mouse.

Magnetic resonance elastography by direct visualization of propagating acoustic strain waves.

MRI biomarker assessment of neuromuscular disease progression: a prospective observational cohort study

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