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Abstract Details

Longitudinal bulbar and respiratory declines in Amyotrophic Lateral Sclerosis (ALS)
General Neurology
P13 - Poster Session 13 (5:30 PM-6:30 PM)
6-006
The primary objective of this study was to estimate the effect of progressive respiratory decline on progressive bulbar decline.  A secondary goal was to determine which other factors may predict advancement of bulbar disease.
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder that results in progressive loss of motor function. Respiratory muscle weakness occurs in all patients, resulting in eventual respiratory failure and death.  Bulbar (speech and swallowing) muscles are also affected in up to 85% of patients.  Bulbar and respiratory impairments are related on the physiological level through the loss of motor neurons in the hypoglossal nucleus and functionally, as both speech and swallowing are tightly coupled to respiration. Because of this tight coupling, it is often difficult to delineate the extent to which bulbar disease progression is conflated with the rapid decline in respiratory function.
471 patients were followed longitudinally (total number of session=1022).  At each session patients completed ALS – Functional Rating Scale – Revised, functional vital capacity test (%FVC), as well as a detailed bulbar assessment protocol, which included a Sentence Intelligibility Test and passage reading. An ordered logistic random effects model with fixed effects and random intercept was used. The stage 0 of bulbar disease progression was operationalized as SIT speaking rate > 155 words per minute (WPM), and stage 1 (active) as SIT speaking rate <155 WPM. The stage 0 of respiratory disease was operationalized as %FVC 

The preliminary results indicated that %FVC was highly predictive of advancing bulbar disease to its active stage;  the odds of a patient moving to stage 1 of bulbar disease given that they were at stage 1 of their FVC decline were 3.9 fold.  Ongoing work is focused on incorporating the fixed effects (e.g., patient demographics) into the model.

NA
Authors/Disclosures
Nnenna Asidianya
PRESENTER 		No disclosure on file
Lawrence Korngut, MD Dr. Korngut has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen. Dr. Korngut has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Mitsubishi Tanabe. Dr. Korngut has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Roche.
Angela L. Genge, MD (Mcgill University) Dr. Genge has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for AL-S Pharma. Dr. Genge has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Amylyx. Dr. Genge has received personal compensation in the range of $100,000-$499,999 for serving as a Consultant for Quralis. Dr. Genge has received personal compensation in the range of $500-$4,999 for serving as a Consultant for MTPA. Dr. Genge has received personal compensation in the range of $0-$499 for serving as a Consultant for WAVE. Dr. Genge has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for eikonizo. Dr. Genge has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for rapa.
Agessandro Abrahao, Jr., MD (Sunnybrook Health Sciences Centre - University of Toronto) Dr. Abrahao has nothing to disclose.
Sanjay Kalra, MD (University of Alberta) The institution of Dr. Kalra has received research support from Brain Canada Foundation. The institution of Dr. Kalra has received research support from Biogen.
Lorne H. Zinman, MD Dr. Zinman has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen, MTP, AB Science, Cytokinetics, Amylyx. Dr. Zinman has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amylyx.
No disclosure on file