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Abstract #1545

Progression of Miyoshi muscular dystrophy monitored by quantitative MRI

Ivica Just1,2, Petra Hnilicova3, Radka Klepochova2,4, Siegfried Trattnig5,6,7,8, Monika Turcanova Koprusakova9, Martin Kolisek3, and Martin Krššák4
1Internal Medicine III, Medical University of Vienna, Vienna, Austria, 2High Field MR Center, Department of Biomedical Imaging and Image-Guided Therapy, Medical University of Vienna, Vienna, Austria, 3Biomedical Centre Martin, Comenius University in Bratislava, Jessenius Faculty of Medicine in Martin, Martin, Slovakia, 4internal Medicine III, Medical University of Vienna, Vienna, Austria, 5High Field MR Center, Department of Biomedical Imaging and Image-Guided Therapy, Medical University of Vienna, Vienna, Austria, 6CD Laboratory for MR Imaging Biomarkers (BIOMAK), Vienna, Austria, 7Austrian Cluster for Tissue Regeneration, Ludwig Boltymann Institute for Experimental and Clinical Traumatology, Vienna, Austria, 8Institute for Clinical Molecular MRI in the Musculoskeletal System, Karl Landsteiner Society, Vienna, Austria, 9Department of Neurology, Comenius University in Bratislava, Jessenius Faculty of Medicine in Martin, Martin, Slovakia

Synopsis

Keywords: Muscle, Rare disease, dystrophy, dysferlinopathy

Motivation: To characterize the skeletal muscle and assess progression of the Miyoshi dystrophy by quantitative MRI.

Goal(s): Compare the changes in fat fractions in individual calf muscles 10 months apart in time.

Approach: Lower extremity of 4 dystrophic patients were measured in 3T scanner by multiecho-Dixon sequence in two time points. Three asymptomatic DYSF gene carriers served as reference.

Results: Fat fraction increased in all measured muscles in dystrophic patients while staying stable in controls. The most significant increments were detected in the muscles most preserved (TP, FDL, EXT, FHL). Variation can be observed even within the patients with the same genotype.

Impact: Using quantitative analysis based on automatically generated fat fraction maps for assessment of Miyoshi dystrophy in muscles allow clinically feasible monitoring of the disease progression and description of the pattern of the disease.

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