To evaluate if changes in the modified Oculobulbar Facial Respiratory score (mOBFRS) in Amyotrophic lateral sclerosis (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.

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, facial and respiratory functions.  The mOBFRS is validated in immune myasthenia gravis.

This is an ongoing prospective study.  Subjects are tested at baseline, month 3 and month 6.  Pearson’s correlation was calculated between mOBFRS, BRS, and ALSFRS-R at each visit.  A linear mixed model assessed mOBFRS as the dependent variable and time as the independent variable to capture how the mOBFRS score changed over time.

Since study initiation, 150 ALS subjects were enrolled in 18 months. To date, 66 subjects have completed the study.  The mOBFRS score negatively correlated with the BRS total score, ALSFRS-R total score, and ALSFRS-R bulbar sub-score at all three visits (p<0.0001). Subject’s mOBFRS score changed by 2.53 points from baseline to month 6 (p<0.0001), consistent with the decrease in the BRS score from 1.0 point from baseline to month 6 (p<0.0221).  The ALSFRS-R swallow ability question was compared to the mOBFRS timed swallow test which showed moderate correlation.  Interestingly, patients tended to underscore their dysphagia in the ALSFRS-R when compared to the objective score measured by the mOBFRS.

Preliminary results show that the mOBFRS is a sensitive measure and has the potential to be a useful outcome measure in evaluating dysphagia in ALS.