Abstract Details

Title Validation of a New Functional Dyspnea Score in Amyotrophic Lateral Sclerosis: ALS Functional Dyspnea Score

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) S42 - Neuromuscular Disorders (4:03 PM-4:14 PM)

Poster/Presentation
Number 004

Background Noninvasive ventilation (NIV) is initiated in ALS when Forced Vital Capacity (FVC) falls below 50% or the patient is dyspneic. While several respiratory scales measure symptoms of dyspnea in patients with pulmonary diseases, it is unclear if these scales are applicable to ALS patients and correlate with FVC

Objective To examine whether the responses to standard dyspnea questionnaires and a new functional dyspnea scale modified for use in ALS correlate with measures of pulmonary function and can be administered by phone

Design/Methods

153 ALS subjects in a pilot study of Nutrition (80) and NIV 73) in ALS completed three dyspnea scales: the Medical Research Council Dyspnea Scale (MDS,0 to 5), the Borg Dyspnea score (0 to 10) and the ALSFDS score(sum of 12 questions, each 1-10) at baseline and follow-up (16,32, and 48 weeks). All participants had sitting FVC(sFVC), and NIV subjects had supine FVC (lFVC) and nasal inspiratory pressure(SNIP). Relationship between measurements was evaluated using Pearson/Spearman Correlation Coefficients. NIV subjects had telephone administration of scales before week 16 and 48 visits.

Results

153 subjects completed baseline visits. Fewer than 1% had a total score of zero on the ALSFDS scale, while 76.9% and 34.0% scored zero (indicating no symptoms) on the Borg and MDC scales respectively whereas the ALSFDS was more sensitive. The ALSFDS was significantly correlated with laying and sitting FVC and SNIP at baseline, 32 and 48 weeks and with the ALSFRS-R. ALSFDS phone scores correlated with office scores at 16(ρ=0.73; p<0.001) and 48(ρ=0.67; p<0.001) weeks

Conclusions The ALSFDS scale is a more sensitive measure of dyspnea in ALS than MDS or Borg scales; correlates with the ALSFRS-R and pulmonary impairment, and is validated for telephone administration. This scale may be important for determining who is at risk for pulmonary compromise and in assessing efficacy of respiratory interventions in ALS trials

Authors/Disclosures
Terry D. Heiman-Patterson, MD (Temple University Lewis Katz School of Medicine) PRESENTER	Dr. Heiman-Patterson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for MTPA. Dr. Heiman-Patterson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Amylyx. Dr. Heiman-Patterson has received personal compensation in the range of $0-$499 for serving as a Consultant for novartis. Dr. Heiman-Patterson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for biogen. The institution of Dr. Heiman-Patterson has received research support from MTPA. The institution of Dr. Heiman-Patterson has received research support from State of Pennsylvania . The institution of Dr. Heiman-Patterson has received research support from ALS Association. The institution of Dr. Heiman-Patterson has received research support from ALS United. The institution of Dr. Heiman-Patterson has received research support from ALS Hope Foundation.
Carlayne E. Jackson, MD, FAAN (University of Texas Health Science Center at San Antonio)	Dr. Jackson has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Mitsubishi Tanabe Pharma America. Dr. Jackson has received personal compensation in the range of $100,000-$499,999 for serving as an officer or member of the Board of Directors for AAN. The institution of Dr. Jackson has received research support from NIH. The institution of Dr. Jackson has received research support from Massachussetts General Hospital.
	No disclosure on file
Jeremy M. Shefner, MD, PhD, FAAN (Barrow Neurological Institute)	Dr. Shefner has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Amylyx. Dr. Shefner has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Uniqure. Dr. Shefner has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Clene. Dr. Shefner has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Cytokinetics. Dr. Shefner has received personal compensation in the range of $0-$499 for serving as a Consultant for Mitsubishi Tanabe Pharma America. Dr. Shefner has received personal compensation in the range of $500-$4,999 for serving as a Consultant for annexon. Dr. Shefner has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Novartis. Dr. Shefner has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sanofi. Dr. Shefner has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Neurosense. Dr. Shefner has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Neuvivo. Dr. Shefner has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Nura BIo. Dr. Shefner has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Prilenia. Dr. Shefner has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for swanbio. Dr. Shefner has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for vertex. Dr. Shefner has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Argenx. Dr. Shefner has received personal compensation in the range of $50,000-$99,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for UpToDate. The institution of Dr. Shefner has received research support from NIH. The institution of Dr. Shefner has received research support from Mitsubishi Tanabe Pharma America. The institution of Dr. Shefner has received research support from Amylyx. The institution of Dr. Shefner has received research support from BIogen. The institution of Dr. Shefner has received research support from Cytokinetics. The institution of Dr. Shefner has received research support from IONIS. The institution of Dr. Shefner has received research support from Healey Center for ALS. Dr. Shefner has received personal compensation in the range of $500-$4,999 for serving as a grant review chair with DOD- General Dynamics.
Daniel S. Newman, MD (Henry Ford Hospital)	Dr. Newman has nothing to disclose.
Stephen N. Scelsa, MD (Mount Sinai BETH ISRAEL, Mount Sinai Downtown)	No disclosure on file
Ashok Verma, MD, FAAN (University of Miami, Clinical Research Bldg)	Dr. Verma has nothing to disclose.
Yvonne Rollins, MD (Heart of the Rockies Regional Medical Center)	No disclosure on file
Edward J. Kasarskis, MD, PhD, FAAN (UK Healthcare)	The institution of Dr. Kasarskis has received research support from Healey Platform Trial Group. The institution of Dr. Kasarskis has received research support from Amylyx . The institution of Dr. Kasarskis has received research support from Alexion. The institution of Dr. Kasarskis has received research support from Woolsey . Dr. Kasarskis has a non-compensated relationship as a Director of ALSA-supported clinic at the University of Kentucky with ALS Association that is relevant to AAN interests or activities.
Edward J. Kasarskis, MD, PhD, FAAN (UK Healthcare)	The institution of Dr. Kasarskis has received research support from Healey Platform Trial Group. The institution of Dr. Kasarskis has received research support from Amylyx . The institution of Dr. Kasarskis has received research support from Alexion. The institution of Dr. Kasarskis has received research support from Woolsey . Dr. Kasarskis has a non-compensated relationship as a Director of ALSA-supported clinic at the University of Kentucky with ALS Association that is relevant to AAN interests or activities.

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