Muscle MR Imaging in Tubular Aggregate Myopathy |
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| Authors: | Valeria Beltrame Paolo Ortolan Alessandro Coran Riccardo Zanato Matteo Gazzola Annachiara Frigo Luca Bello Elena Pegoraro Roberto Stramare |
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| Institution: | 1. Department of Medicine, Section of Radiology, University of Padova, Padova, Italy.; 2. Department of Cardiac, Thoracic and Vascular Sciences, University of Padova, Padova, Italy.; 3. Department of Neurosciences, University of Padova, Padova. Italy.; Earl and Christy Powell University, United States of America, |
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| Abstract: | PurposeTo evaluate with Magnetic Resonance (MR) the degree of fatty replacement and edematous involvement in skeletal muscles in patients with Tubular Aggregate Myopathy (TAM). To asses the inter-observer agreement in evaluating muscle involvement and the symmetry index of fatty replacement.Materials and Methods13 patients were evaluated by MR to ascertain the degree of fatty replacement (T1W sequences) according to Mercuri''s scale, and edema score (STIR sequences) according to extent and site.ResultsFatty replacement mainly affects the posterior superficial compartment of the leg; the anterior compartment is generally spared. Edema was generally poor and almost only in the superficial compartment of the leg. The inter-observer agreement is very good with a Krippendorff''s coefficient >0.9. Data show a total symmetry in the muscular replacement (McNemar-Bowker test with p = 1).ConclusionsMR reveals characteristic muscular involvement, and is a reproducible technique for evaluation of TAM. There may also be a characteristic involvement of the long and short heads of the biceps femoris. It is useful for aimed biopsies, diagnostic hypotheses and evaluation of disease progression. |
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