Clinical scales for the evaluation of neuromuscular patients

The slow progressive nature of the many muscle disorders and the wide variability ofpattern of involvment in muscular dystrophies and myopathies make it difficult toestablish the prognosis, to predict the clinical evolution of the disease and to define theimpact on natural history of new therapies that are becoming available, such as EnzymeReplacement Therapy. Quantitative and qualitative scales appropriated to myopathicpatients are, therefore, needed in order to describe accurately the clinical status anddisease evolution along the years.Many tests and scales have been constructed to evaluate motor function, includingmanual muscle testing for muscular strength, the Walton and Medwin-Gardner scale forthe motor disability, and timed test such as the 6-Minute Walk Test, the GSGC score andthe Jebsen Hand Function Test. To describe more accurately the evolution and theclinical status of specific diseases, appropriate scales have been created, including theFSHD evaluation scale for the Facio-Scapulo-Humeral Dystrophy and the North StarAmbulatory Assessment for boys affected by Duchenne muscular dystrophy.The evaluation of Quality of Life is mandatory in progressive disorders such asmuscular pathologies. It is usually measured with different tests; the most important forneuromuscular disorders are the SF-36, the Fatigue Severity Scale, the RotterdamHandicap Scale and the InQoL.