Validation of MRC Centre MRI calf muscle fat fraction protocol as an outcome measure in CMT1A

Jasper M Morrow; Matthew R B Evans; Tiffany Grider; Christopher D J Sinclair; Daniel Thedens; Sachit Shah; Tarek A Yousry; Michael G Hanna; Peggy Nopoulos; John S Thornton; Michael E Shy; Mary M Reilly

doi:10.1212/WNL.0000000000006214

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$Validation of MRC Centre MRI calf muscle fat fraction protocol as an outcome measure in CMT1A$

Journal article

Open access

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Validation of MRC Centre MRI calf muscle fat fraction protocol as an outcome measure in CMT1A

Jasper M Morrow, Matthew R B Evans, Tiffany Grider, Christopher D J Sinclair, Daniel Thedens, Sachit Shah, Tarek A Yousry, Michael G Hanna, Peggy Nopoulos, John S Thornton, …

Neurology, Vol.91(12), pp.e1125-e1129

09/18/2018

DOI: 10.1212/WNL.0000000000006214

PMCID: PMC6161551

PMID: 30120135

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Abstract

To translate the quantitative MRC Centre MRI protocol in Charcot-Marie-Tooth disease type 1A (CMT1A) to a second site; validate its responsiveness in an independent cohort; and test the benefit of participant stratification to increase outcome measure responsiveness. Three healthy volunteers were scanned for intersite standardization. For the longitudinal patient study, 11 patients with CMT1A were recruited with 10 patients rescanned at a 12-month interval. Three-point Dixon MRI of leg muscles was performed to generate fat fraction (FF) maps, transferred to a central site for quality control and analysis. Clinical data collected included CMT Neuropathy Score. Test-retest reliability of FF within individual healthy calf muscles at the remote site was excellent: intraclass correlation coefficient 0.79, limits of agreement -0.67 to +0.85 %FF. In patients, mean calf muscle FF was 21.0% and correlated strongly with disease severity and age. Calf muscle FF significantly increased over 12 months (+1.8 ± 1.7 %FF, = 0.009). Patients with baseline FF >10% showed a 12-month FF increase of 2.9% ± 1.3% (standardized response mean = 2.19). We have validated calf muscle FF as an outcome measure in an independent cohort of patients with CMT1A. Responsiveness is significantly improved by enrolling a stratified patient cohort with baseline calf FF >10%.

Magnetic Resonance Imaging

Predictive Value of Tests

Reproducibility of Results

Age Factors

Humans

Middle Aged

Lower Extremity - diagnostic imaging

Male

Adipose Tissue - diagnostic imaging

Disease Progression

Young Adult

Time Factors

Charcot-Marie-Tooth Disease - diagnostic imaging

Muscle, Skeletal - diagnostic imaging

Adult

Female

Charcot-Marie-Tooth Disease - diagnosis

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Title: Subtitle: Validation of MRC Centre MRI calf muscle fat fraction protocol as an outcome measure in CMT1A
Creators: Jasper M Morrow - From the MRC Centre for Neuromuscular Diseases (J.M.M., M.R.B.E., C.D.J.S., T.A.Y., M.G.H., J.S.T., M.M.R.) and Neuroradiological Academic Unit (S.S.), UCL Institute of Neurology, London, UK; Carver College of Medicine (T.G., P.N., M.E.S.) and Department of Radiology (D.T.), University of Iowa, Iowa City
Matthew R B Evans - From the MRC Centre for Neuromuscular Diseases (J.M.M., M.R.B.E., C.D.J.S., T.A.Y., M.G.H., J.S.T., M.M.R.) and Neuroradiological Academic Unit (S.S.), UCL Institute of Neurology, London, UK; Carver College of Medicine (T.G., P.N., M.E.S.) and Department of Radiology (D.T.), University of Iowa, Iowa City
Tiffany Grider - From the MRC Centre for Neuromuscular Diseases (J.M.M., M.R.B.E., C.D.J.S., T.A.Y., M.G.H., J.S.T., M.M.R.) and Neuroradiological Academic Unit (S.S.), UCL Institute of Neurology, London, UK; Carver College of Medicine (T.G., P.N., M.E.S.) and Department of Radiology (D.T.), University of Iowa, Iowa City
Christopher D J Sinclair - From the MRC Centre for Neuromuscular Diseases (J.M.M., M.R.B.E., C.D.J.S., T.A.Y., M.G.H., J.S.T., M.M.R.) and Neuroradiological Academic Unit (S.S.), UCL Institute of Neurology, London, UK; Carver College of Medicine (T.G., P.N., M.E.S.) and Department of Radiology (D.T.), University of Iowa, Iowa City
Daniel Thedens - From the MRC Centre for Neuromuscular Diseases (J.M.M., M.R.B.E., C.D.J.S., T.A.Y., M.G.H., J.S.T., M.M.R.) and Neuroradiological Academic Unit (S.S.), UCL Institute of Neurology, London, UK; Carver College of Medicine (T.G., P.N., M.E.S.) and Department of Radiology (D.T.), University of Iowa, Iowa City
Sachit Shah - From the MRC Centre for Neuromuscular Diseases (J.M.M., M.R.B.E., C.D.J.S., T.A.Y., M.G.H., J.S.T., M.M.R.) and Neuroradiological Academic Unit (S.S.), UCL Institute of Neurology, London, UK; Carver College of Medicine (T.G., P.N., M.E.S.) and Department of Radiology (D.T.), University of Iowa, Iowa City
Tarek A Yousry - From the MRC Centre for Neuromuscular Diseases (J.M.M., M.R.B.E., C.D.J.S., T.A.Y., M.G.H., J.S.T., M.M.R.) and Neuroradiological Academic Unit (S.S.), UCL Institute of Neurology, London, UK; Carver College of Medicine (T.G., P.N., M.E.S.) and Department of Radiology (D.T.), University of Iowa, Iowa City
Michael G Hanna - From the MRC Centre for Neuromuscular Diseases (J.M.M., M.R.B.E., C.D.J.S., T.A.Y., M.G.H., J.S.T., M.M.R.) and Neuroradiological Academic Unit (S.S.), UCL Institute of Neurology, London, UK; Carver College of Medicine (T.G., P.N., M.E.S.) and Department of Radiology (D.T.), University of Iowa, Iowa City
Peggy Nopoulos - From the MRC Centre for Neuromuscular Diseases (J.M.M., M.R.B.E., C.D.J.S., T.A.Y., M.G.H., J.S.T., M.M.R.) and Neuroradiological Academic Unit (S.S.), UCL Institute of Neurology, London, UK; Carver College of Medicine (T.G., P.N., M.E.S.) and Department of Radiology (D.T.), University of Iowa, Iowa City
John S Thornton - From the MRC Centre for Neuromuscular Diseases (J.M.M., M.R.B.E., C.D.J.S., T.A.Y., M.G.H., J.S.T., M.M.R.) and Neuroradiological Academic Unit (S.S.), UCL Institute of Neurology, London, UK; Carver College of Medicine (T.G., P.N., M.E.S.) and Department of Radiology (D.T.), University of Iowa, Iowa City
Michael E Shy - From the MRC Centre for Neuromuscular Diseases (J.M.M., M.R.B.E., C.D.J.S., T.A.Y., M.G.H., J.S.T., M.M.R.) and Neuroradiological Academic Unit (S.S.), UCL Institute of Neurology, London, UK; Carver College of Medicine (T.G., P.N., M.E.S.) and Department of Radiology (D.T.), University of Iowa, Iowa City
Mary M Reilly - From the MRC Centre for Neuromuscular Diseases (J.M.M., M.R.B.E., C.D.J.S., T.A.Y., M.G.H., J.S.T., M.M.R.) and Neuroradiological Academic Unit (S.S.), UCL Institute of Neurology, London, UK; Carver College of Medicine (T.G., P.N., M.E.S.) and Department of Radiology (D.T.), University of Iowa, Iowa City. m.reilly@ucl.ac.uk
Resource Type: Journal article
Publication Details: Neurology, Vol.91(12), pp.e1125-e1129
DOI: 10.1212/WNL.0000000000006214
PMID: 30120135
PMCID: PMC6161551
NLM abbreviation: Neurology
ISSN: 0028-3878
eISSN: 1526-632X
Publisher: United States
Grant note: G0601943 / Medical Research Council MR/K000608/1 / Medical Research Council MR/M009106/1 / Medical Research Council
Language: English
Date published: 09/18/2018
Academic Unit: Roy J. Carver Department of Biomedical Engineering; Neurology; Radiology; Electrical and Computer Engineering; Molecular Physiology and Biophysics; Psychiatry; Stead Family Department of Pediatrics; Iowa Neuroscience Institute
Record Identifier: 9984070277802771

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