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April 07, 2020; 94 (14) Article

Fat fraction distribution in lower limb muscles of patients with CMT1A

A quantitative MRI study

Joachim Bas, Augustin C. Ogier, Arnaud Le Troter, Emilien Delmont, Benjamin Leporq, Lauriane Pini, Maxime Guye, Amandine Parlanti, Marie-Noëlle Lefebvre, David Bendahan, Shahram Attarian
First published January 24, 2020, DOI: https://doi.org/10.1212/WNL.0000000000009013
Joachim Bas
From the Reference Center for Neuromuscular Diseases and ALS (J.B., E.D., A.P., S.A.) and CIC-CPCET (M.-N.L.), La Timone University Hospital, Aix-Marseille University; Aix-Marseille University (A.C.O., A.L.T., L.P., M.G., D.B.), CNRS, Center for Magnetic Resonance in Biology and Medicine; Aix-Marseille University (E.D.), UMR 7286, Medicine Faculty; Aix-Marseille University (S.A.), Inserm, GMGF; Aix Marseille University (A.C.O.), Université de Toulon, CNRS, LIS, Marseille; Université de Lyon (B.L.); and CREATIS CNRS UMR 5220 (B.L.), Inserm U1206, INSA-Lyon, UCBL Lyon 1, France.
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Augustin C. Ogier
From the Reference Center for Neuromuscular Diseases and ALS (J.B., E.D., A.P., S.A.) and CIC-CPCET (M.-N.L.), La Timone University Hospital, Aix-Marseille University; Aix-Marseille University (A.C.O., A.L.T., L.P., M.G., D.B.), CNRS, Center for Magnetic Resonance in Biology and Medicine; Aix-Marseille University (E.D.), UMR 7286, Medicine Faculty; Aix-Marseille University (S.A.), Inserm, GMGF; Aix Marseille University (A.C.O.), Université de Toulon, CNRS, LIS, Marseille; Université de Lyon (B.L.); and CREATIS CNRS UMR 5220 (B.L.), Inserm U1206, INSA-Lyon, UCBL Lyon 1, France.
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Arnaud Le Troter
From the Reference Center for Neuromuscular Diseases and ALS (J.B., E.D., A.P., S.A.) and CIC-CPCET (M.-N.L.), La Timone University Hospital, Aix-Marseille University; Aix-Marseille University (A.C.O., A.L.T., L.P., M.G., D.B.), CNRS, Center for Magnetic Resonance in Biology and Medicine; Aix-Marseille University (E.D.), UMR 7286, Medicine Faculty; Aix-Marseille University (S.A.), Inserm, GMGF; Aix Marseille University (A.C.O.), Université de Toulon, CNRS, LIS, Marseille; Université de Lyon (B.L.); and CREATIS CNRS UMR 5220 (B.L.), Inserm U1206, INSA-Lyon, UCBL Lyon 1, France.
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Emilien Delmont
From the Reference Center for Neuromuscular Diseases and ALS (J.B., E.D., A.P., S.A.) and CIC-CPCET (M.-N.L.), La Timone University Hospital, Aix-Marseille University; Aix-Marseille University (A.C.O., A.L.T., L.P., M.G., D.B.), CNRS, Center for Magnetic Resonance in Biology and Medicine; Aix-Marseille University (E.D.), UMR 7286, Medicine Faculty; Aix-Marseille University (S.A.), Inserm, GMGF; Aix Marseille University (A.C.O.), Université de Toulon, CNRS, LIS, Marseille; Université de Lyon (B.L.); and CREATIS CNRS UMR 5220 (B.L.), Inserm U1206, INSA-Lyon, UCBL Lyon 1, France.
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Benjamin Leporq
From the Reference Center for Neuromuscular Diseases and ALS (J.B., E.D., A.P., S.A.) and CIC-CPCET (M.-N.L.), La Timone University Hospital, Aix-Marseille University; Aix-Marseille University (A.C.O., A.L.T., L.P., M.G., D.B.), CNRS, Center for Magnetic Resonance in Biology and Medicine; Aix-Marseille University (E.D.), UMR 7286, Medicine Faculty; Aix-Marseille University (S.A.), Inserm, GMGF; Aix Marseille University (A.C.O.), Université de Toulon, CNRS, LIS, Marseille; Université de Lyon (B.L.); and CREATIS CNRS UMR 5220 (B.L.), Inserm U1206, INSA-Lyon, UCBL Lyon 1, France.
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Lauriane Pini
From the Reference Center for Neuromuscular Diseases and ALS (J.B., E.D., A.P., S.A.) and CIC-CPCET (M.-N.L.), La Timone University Hospital, Aix-Marseille University; Aix-Marseille University (A.C.O., A.L.T., L.P., M.G., D.B.), CNRS, Center for Magnetic Resonance in Biology and Medicine; Aix-Marseille University (E.D.), UMR 7286, Medicine Faculty; Aix-Marseille University (S.A.), Inserm, GMGF; Aix Marseille University (A.C.O.), Université de Toulon, CNRS, LIS, Marseille; Université de Lyon (B.L.); and CREATIS CNRS UMR 5220 (B.L.), Inserm U1206, INSA-Lyon, UCBL Lyon 1, France.
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Maxime Guye
From the Reference Center for Neuromuscular Diseases and ALS (J.B., E.D., A.P., S.A.) and CIC-CPCET (M.-N.L.), La Timone University Hospital, Aix-Marseille University; Aix-Marseille University (A.C.O., A.L.T., L.P., M.G., D.B.), CNRS, Center for Magnetic Resonance in Biology and Medicine; Aix-Marseille University (E.D.), UMR 7286, Medicine Faculty; Aix-Marseille University (S.A.), Inserm, GMGF; Aix Marseille University (A.C.O.), Université de Toulon, CNRS, LIS, Marseille; Université de Lyon (B.L.); and CREATIS CNRS UMR 5220 (B.L.), Inserm U1206, INSA-Lyon, UCBL Lyon 1, France.
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Amandine Parlanti
From the Reference Center for Neuromuscular Diseases and ALS (J.B., E.D., A.P., S.A.) and CIC-CPCET (M.-N.L.), La Timone University Hospital, Aix-Marseille University; Aix-Marseille University (A.C.O., A.L.T., L.P., M.G., D.B.), CNRS, Center for Magnetic Resonance in Biology and Medicine; Aix-Marseille University (E.D.), UMR 7286, Medicine Faculty; Aix-Marseille University (S.A.), Inserm, GMGF; Aix Marseille University (A.C.O.), Université de Toulon, CNRS, LIS, Marseille; Université de Lyon (B.L.); and CREATIS CNRS UMR 5220 (B.L.), Inserm U1206, INSA-Lyon, UCBL Lyon 1, France.
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Marie-Noëlle Lefebvre
From the Reference Center for Neuromuscular Diseases and ALS (J.B., E.D., A.P., S.A.) and CIC-CPCET (M.-N.L.), La Timone University Hospital, Aix-Marseille University; Aix-Marseille University (A.C.O., A.L.T., L.P., M.G., D.B.), CNRS, Center for Magnetic Resonance in Biology and Medicine; Aix-Marseille University (E.D.), UMR 7286, Medicine Faculty; Aix-Marseille University (S.A.), Inserm, GMGF; Aix Marseille University (A.C.O.), Université de Toulon, CNRS, LIS, Marseille; Université de Lyon (B.L.); and CREATIS CNRS UMR 5220 (B.L.), Inserm U1206, INSA-Lyon, UCBL Lyon 1, France.
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David Bendahan
From the Reference Center for Neuromuscular Diseases and ALS (J.B., E.D., A.P., S.A.) and CIC-CPCET (M.-N.L.), La Timone University Hospital, Aix-Marseille University; Aix-Marseille University (A.C.O., A.L.T., L.P., M.G., D.B.), CNRS, Center for Magnetic Resonance in Biology and Medicine; Aix-Marseille University (E.D.), UMR 7286, Medicine Faculty; Aix-Marseille University (S.A.), Inserm, GMGF; Aix Marseille University (A.C.O.), Université de Toulon, CNRS, LIS, Marseille; Université de Lyon (B.L.); and CREATIS CNRS UMR 5220 (B.L.), Inserm U1206, INSA-Lyon, UCBL Lyon 1, France.
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Shahram Attarian
From the Reference Center for Neuromuscular Diseases and ALS (J.B., E.D., A.P., S.A.) and CIC-CPCET (M.-N.L.), La Timone University Hospital, Aix-Marseille University; Aix-Marseille University (A.C.O., A.L.T., L.P., M.G., D.B.), CNRS, Center for Magnetic Resonance in Biology and Medicine; Aix-Marseille University (E.D.), UMR 7286, Medicine Faculty; Aix-Marseille University (S.A.), Inserm, GMGF; Aix Marseille University (A.C.O.), Université de Toulon, CNRS, LIS, Marseille; Université de Lyon (B.L.); and CREATIS CNRS UMR 5220 (B.L.), Inserm U1206, INSA-Lyon, UCBL Lyon 1, France.
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Citation
Fat fraction distribution in lower limb muscles of patients with CMT1A
A quantitative MRI study
Joachim Bas, Augustin C. Ogier, Arnaud Le Troter, Emilien Delmont, Benjamin Leporq, Lauriane Pini, Maxime Guye, Amandine Parlanti, Marie-Noëlle Lefebvre, David Bendahan, Shahram Attarian
Neurology Apr 2020, 94 (14) e1480-e1487; DOI: 10.1212/WNL.0000000000009013

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Abstract

Objective To quantitatively describe the MRI fat infiltration pattern of muscle degeneration in Charcot-Marie-Tooth (CMT) type 1A (CMT1A) disease and to look for correlations with clinical variables.

Methods MRI fat fraction was assessed in lower-limb musculature of patients with CMT1A and healthy controls. More particularly, 14 muscle compartments were selected at leg and thigh levels and for proximal, distal, and medial slices. Muscle fat infiltration profile was determined quantitatively in each muscle compartment and along the entire volume of acquisition to determine a length-dependent gradient of fat infiltration. Clinical impairment was evaluated with muscle strength measurements and CMT Examination Scores (CMTESs).

Results A total of 16 patients with CMT1A were enrolled and compared to 11 healthy controls. Patients with CMT1A showed a larger muscle fat fraction at leg and thigh levels with a proximal-to-distal gradient. At the leg level, the largest fat infiltration was quantified in the anterior and lateral compartments. CMTES was correlated with fat fraction, especially in the anterior compartment of leg muscles. Strength of plantar flexion was also correlated with fat fraction of the posterior compartments of leg muscles.

Conclusion On the basis of quantitative MRI measurements combined with a dedicated segmentation method, muscle fat infiltration quantified in patients with CMT1A disclosed a length-dependent peroneal-type pattern of fat infiltration and was correlated to main clinical variables. Quantification of fat fraction at different levels of the leg anterior compartment might be of interest in future clinical trials.

Glossary

CMT=
Charcot-Marie-Tooth;
CMTES=
CMT Examination Score;
CMTNSv2=
CMT Neuropathy Score Version 2;
CMT1A=
CMT type 1;
ONLS=
Overall Neuropathy Limitations Scale;
PDFF=
proton-density fat fraction;
PMP22=
peripheral myelin protein 22;
T1W=
T1-weighted

Footnotes

  • Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.

  • Received July 28, 2019.
  • Accepted in final form October 10, 2019.
  • © 2020 American Academy of Neurology
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