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Abstract #3177

IDEAL-CPMG muscle T2-water and Dixon Fat-Fraction Maps in People with Inclusion Body Myositis and Charcot-Marie-Tooth disease Type 1A

Amy R. McDowell1, Stephen J. Wastling1,2, Lara Cristiano3, Jasper M. Morrow1, Matthew R.B. Evans4, Christopher D.J. Sinclair1, Pedro M. Machado1, Michael Hanna1, Mary M. Reilly1, Tarek Yousry1, and John S. Thornton1
1MRC Centre for Neuromuscular Diseases, UCL Queen Square Institute of Neurology, London, United Kingdom, 2Lysholm Department of Neuroradiology, National Hospital for Neurology and Neurosurgery, London, United Kingdom, 3Department of Pediatric Neurology and Radiology, Fondazione Policlinico Universitario, Rome, Italy, 4St Thomas Hospital, London, United Kingdom

Muscle T2 and fat-fraction are two promising metrics for sensitive measures of early disease-related changes in muscle for use in clinical trials. However, fat has a longer T2 than water in muscle and masks underlying increases in water T2 due to muscle oedema. The IDEAL-CPMG sequence with appropriate image-data processing produces a T2-water map uncontaminated by the fat signal. This sequence was acquired in healthy volunteers and two different muscular dystrophy disease types. It was found that elevated T2-water may be a predictor of later progression to fatty-atrophy in muscle, supporting the value of future longitudinal studies to test this.

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