CMT AND NEUROGENIC DISEASE

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Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder that results in progressive facial, bulbar, respiratory and limb muscle weakness. The ALSFRS-R is a reliable patient reported outcome measure used in ALS, however, we lack objective scales that can quantitate deterioration in bulbar functions. We are conducting a prospective study to test the feasibility of using, and validate the use of the modified oculobulbar facial respiratory score (mOBFRS) in ALS. We will evaluate if the changes in the mOBFRS in ALS): 1) can be reliably measured on a serial basis; 2) correlate with scores of the existing subjective ALS functional rating scale (ALSFRS-R) and objective bulbar rating scale (BRS); and 3) can be used as a potential outcome measure in clinical trials.

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