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Abstract Details

Relationships Between Forced Vital Capacity and Respiratory Sub-domain Scale on the ALSFRS-R
General Neurology
P14 - Poster Session 14 (8:00 AM-9:00 AM)
6-004

 Evaluate the relationship between respiratory function subscore of ALSFRS-R (ALSFRS-R R-sum) with Forced vital capacity (FVC).

 

 

 
Respiratory failure is associated with morbidity and mortalityof amyotrophic lateral sclerosis (ALS). Both FVC and ALSFRS-R are widely used clinically. The respiratory subscore of the ALSFRS (R-sum) is used as a marked for disease, but there is controversy whether there is correlation between ALSFRS-R R-sum and percentage predict FVC (%FVC).
1644 patients from PRO-ACT database were included. The Pearson correlation was used to evaluate the correlation. Generalized estimating equations (GEE) model was used to analyze the correlation between ALSFRS-R R-sum and the %FVC cut-off result. The Receiver operator characteristic (ROC) curve was used to assess the ability of diagnostic test to distinguish between two discreet states. Linear regression and segmented regression were used to explore the decline of FVC.

ALSFRS-R R-sum correlated with %FVC moderately (r =0.47; p<0.001) but weaker between individual items and %FVC. Sensitivity was 60.9% and specificity was 71.5% for the ALSFRS-R R-sum when we set %FVC 80% as a cut-off value, below which implied respiratory restriction. Sensitivity was 69.8% and specificity was 75.9% for the ALSFRS-R R-sum when we set %FVC 50% as a cut-off value, below which implied non-invasive positive pressure ventilation (NIPPV) is recommended. Decline of %FVC separated sub-groups with different respiratory function progression rate more obviously and more sensitively than that of ALSFRS-R R-sum. Older age, shorter diagnostic delay, shorter time from diagnosis to first follow-up, fewer follow-up times, lower baseline FVC were significantly associated with faster decline of respiratory function.
 Objective respiratory function measurement FVC is essential in ALS. The ALSFRS R-sum moderately correlated with FVC. Our findings suggest the importance of FVC over ALSFRS R-sum to predict the respiratory progression rate and timely decision about respiratory interventions.
Authors/Disclosures

PRESENTER 		No disclosure on file
No disclosure on file
James P. Wymer, MD, PhD, FÂ鶹´«Ã½Ó³»­ (Department of Neurology, University of Florida) No disclosure on file