Disease Severity Scoring

Background: There are several ways of measuring disease severity but they can be broadly divided into physician/evaluator determined severity scales and patient self-reported scales. The former are usually dependent on measurements of motor function either by direct strength measurement or determination of functional abilities. Severity scales, in addition to being useful clinical tools for patient managment, are important in describing the natural history of the disease which, in turn, is critical in the design of future therapeutic trials. In addition, clinical severity scoring allows us to look at genotype-phenotype associations. There are no fully validated clinical severity scoring systems specific for FSHD. However, the scale used below has been used in several published studies.

Clinical Severity Scoring (Download PDF file)

 

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