P125 Quantitative MRI in upper limb muscles of patients with dysferlinopathy: 6-months and 12-months longitudinal data from the natural history Jain COS 2 project

In dysferlinopathy patients, the upper limb muscles are affected in the later stages of the disease, and muscle fat replacement of arm and forearm muscles follows thigh and leg involvement. Additionally, increased disease activity (such as inflammation) in lower limb muscles has been reported to be a feature in patients with dysferlinopathy. Adding to the COS1 results, a longitudinal quantitative MRI study in upper limbs was set up in four clinical centers (UK, France, Italy, USA), investigating both muscle fat fraction (FF; reflecting the extent of muscle fat replacement) and water T2 (reflecting the extent of muscle inflammation).

Source: Neuromuscular Disorders - October 1, 2023 Category: Neurology Authors: I. Wilson, H. Reyngoudt, E Caldas de Almeida Araujo, P. Baudin, B. Marty, C. Bolano-Diaz, J. Diaz-Manera, L. Rufibach, H. Hilsden, G. Querin, E. Pegoraro, J. Mendell, T. Stojkovic, V. Straub, A. Blamire, P. Carlier Source Type: research