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Abstract Details

Novel Measurement of Labial[LAR] and Tongue[TAR]-Speech Articulation Rates[Words-Per-Minute-Per-Breath(WpMpB)] and 3-Ounce Liquid Swallowing Rate[LSR][mL-Per-Swallow-Per-Minute-Per-Breath (mLpSwpMpB)] in Patients with Amyotrophic Lateral Sclerosis (ALS) and Other Neuromuscular Diseases (NMD) - Test-Re-Test, Inter-Rater Reliability, Correlations with ALS Functional Rating Scale- Revised (ALSFRS-R), Total(-T), Bulbar(-B) Item Sub-Scores[Speech(-Sp)/Salivation(-Sa)/Swallow(-Sw)], Respiratory Function and Longitudinal Change over Time with Disease Activity
Anterior Horn
P07 - (-)
068
BACKGROUND: LAR/TAR changes over time significantly prior to intelligibility changes. LSR changes prior to video-fluoroscopic changes across causes of neurogenic dysphagia. Quantitative changes in LAR/TAR/LSR allow assessment of disease progression in bulbar function.
DESIGN/METHODS: LAR[measured by repeating "pepper"]/TAR[measured by repeating "ticker"]10 times with recording the start/completion time in seconds and the number of breaths. LSR[measured by swallowing 90 mL(3oz)water] with recording time from the initiation of liquid leaving the cup at the lips to the completion of the final swallow of liquid in seconds and the number of breaths. WpMpB and mLpSwpMpB were calculated and statistical analysis was performed with MedcCalc software(version-12. 2.1)[www.medcalc.org/].
RESULTS: At diagnosis in 118 ALS patients[60-M/58-F;age-58.8卤7.5yr;ALSFRS-Rt-32.2卤7.9] and 136 NMD patients[72-M/64-F;age-44.4卤20.8yr], LAR/TAR/LSR were measurable in 104 ALS and 128 NMD patients. ALS LAR/TAR [110卤70.5(SD)WpMpB/112.9卤71.8]were significantly(p<0.001)decreased compared with NMD LAR/TAR [163.9卤46.7/173.7卤46.2]. ALS LSR[24.3卤37.4(SD)mLpSwpMpB]was significantly(p<0.001)decreased from NMD LSR[107.8卤115.5]. All were different from normal controls. Test-re-test in 33 patients and inter-rater reliability in 75 patients correlated significantly[r=0.8901/0.9121;both p<0.001). LAR/TAR/LSR correlated significantly, but poorly with ALSFRS-Rt[r=0.3482(p=0.0064)/0.3672(p=0.0039)/0.2993(p=0.0237)]but significantly and better with ALSFRS-R-B [r=0.6752(p=0.001)/0.6675(p=0.0001)/0.3211(p=0.0149)]. LAR/TAR/LSR change with disease in ALS and NMD patients but LAR/TAR may change differently than LSR in different patients. Over 12 months, ALS patients LAR/TAR change significantly in 34% of patients while LSR changes significantly in 48% of patients.
CONCLUSIONS: LAR/TAR/LSR are robust quantitative bulbar functional measures that provide specific outcome measurements providing earlier change than bulbar functional milestone scales(-B/-Sp/-Sa/-Sw) in the ALSFRS-R. LSR specifically provides more robust information concerning swallowing than ALSFRS-R bulbar function milestone scales earlier in the course of ALS.
Authors/Disclosures

PRESENTER 		No disclosure on file
Elena Bravver, MD No disclosure on file
No disclosure on file
No disclosure on file
No disclosure on file
No disclosure on file
Benjamin R. Brooks, MD, FAAN (Clinical Trials Planning LLC) Dr. Brooks has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Mitsubishi Tanabe Pharma America. Dr. Brooks has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Medicinova. Dr. Brooks has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Dr. Brooks has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for AB Science. Dr. Brooks has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Ionis. Dr. Brooks has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Mitsubishi Tanabe Pharma America. The institution of Dr. Brooks has received research support from Mitsubishi TanabePharma America. Dr. Brooks has received personal compensation in the range of $0-$499 for serving as a Member Annual Surveillance Committee CDC National ALS Registry with Center for Disease Control Agency Toxic Substances Disease Registry. Dr. Brooks has a non-compensated relationship as a Member ALS Quality Measures Subcommittee with 好色先生 that is relevant to AAN interests or activities.