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FSHD1 constitutes 95% of situations and is associated with truncation associated with D4Z4 macrosatellite at 4q35. In most cases the condition initially provides with facial and proximal weakness associated with upper limbs, but over the course of the disease involves reduced limb and truncal muscles. Weakness is progressive and frequently asymmetric, that will be a hallmark of this illness. Right here we performed an analysis of 643 FSHD1 customers in the united kingdom