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Abstract #1164

Muscular fat infiltration in FSHD starts with a “fat burst” near the distal tendon and advances towards the proximal tendon

Linda Heskamp1, Augustin C. Ogier2,3, David Bendahan3, and Arend Heerschap1
1Radiology and Nuclear Medicine, Radboud university medical center, Nijmegen, Netherlands, 2Aix Marseille Univ, Université de Toulon, CNRS, LIS, Marseille, France, 3Aix Marseille Univ, CNRS, CRMBM, Marseille, France

In patients with facioscapulohumeral muscular dystrophy (FSHD) it is known what the genetic origin of the disease is, but unknown how it is initiated and propagates along muscles. In a cross-sectional and longitudinal study we analyzed fat infiltration in lower leg muscles, tendon-to-tendon, with a 3D Dixon method. This revealed that fat infiltration starts with a distal “fat burst” in the first years of its initiation after which fat replacement further proceeds in a slower pace towards the proximal tendons. This indicates that the disease is triggered by an event typical for the distal parts of lower extremity muscles.

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