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Abstract #1703

One-year longitudinal assessment of CMT1A patients using Quantitative MRI

Etienne FORTANIER1, Marc-Adrien HOSTIN2, Constance Michel3, Emilien DELMONT4, Marc-Emmanuel BELLEMARE5, maxime Guye3, David bendahan3, and Shahram ATTARIAN6
1Neurology, APHM, MARSEILLE, France, 2CRMBM, marseille, France, 3CRMBM, MARSEILLE, France, 4APHM, MARSEILLE, France, 5LIS, MARSEILLE, France, 6APHM, Marseille, France

Synopsis

Keywords: Muscle, Quantitative Imaging, Neuropathy, Follow-up

Motivation: Intramuscular fat fraction (FF) quantified using MRI has emerged as one of the few responsive outcome measures in neuropathic patients.

Goal(s): In the present one-year follow-up study we tracked changes in multiple qMRI biomarkers for CMT1A patients.

Approach: We assessed correlations between MRI and clinical parameters and compared 2D vs 3D segmentation analysis.

Results: As expected, we quantified a significant FF rise in both thigh and leg muscles and a length-dependent gradient in leg muscles. Given the varied FF distribution, the existence of a length-dependent gradient, and the differential fatty involution across muscles, 3D volume analysis appeared more faithful than single slice analysis.

Impact: Our longitudinal study further supports FF as a outcome of interest in neuropathic patients. The complexity of fat infiltration in terms of distribution among muscles and heterogeneity along the proximo-distal axis can be identified using a 3D volume analysis.

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