Highlights
- •Acceleration timing of respiratory decline in ALS was introduced as a novel metric.
- •Logistic curve fitted well with the time-serial decline in %VC, estimating acceleration.
- •The acceleration timing strongly correlated with respiratory/nutritional support timing.
- •The acceleration time-point was at which %VC is becoming 0.789 times of maximum %VC.
- •This new metric may be of help in the management for earlier stage ALS.
Abstract
Objective
We aimed to derive and assess a novel metric for respiratory decline: the timing of
acceleration of respiratory functional decline during the course of the disease in
patients with amyotrophic lateral sclerosis (ALS).
Methods
In this single-center retrospective study, we reviewed consecutive definite/probable
ALS patients, diagnosed and followed up at our hospital. We recorded serial slow vital
capacity (percentage of predicted slow vital capacity; %VC) since diagnosis for all
patients. These serial %VC data were fitted with logistic function of the time since
diagnosis, and ‘acceleration point’ was calculated as the week in which the second
derivative of the fitted logistic function had the minimum value.
Results
We included 62 patients with ALS, whose serial %VC data had been recorded for a median
of 8 times over a median of 94.3 weeks. The calculated acceleration time-point was
the time-point at which the %VC is becoming 0.789 times of maximum %VC, and had a
strong association with the period since diagnosis to the administration of nutritional/respiratory
support (p < 0.001). Bulbar-type ALS or lower Body Mass Index at diagnosis, both are well-known
ALS prognostic factors, were also associated with more rapid arrival of the acceleration
time-point.
Conclusions
We introduced the time-point of acceleration in the vital capacity decline during
disease progression as a novel metric for ALS respiratory decline. Although we could
not build a practically-available clinical model that directly predicts acceleration
time-point due to the limited sample size, our metric may be used as one of the helpful
indicators in the management during earlier disease course of ALS, such as to be careful
for the potentially approaching acceleration time-point when the %VC is decreasing
to approximately 0.789 times of initial %VC.
Keywords
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Article info
Publication history
Published online: May 30, 2019
Accepted:
May 27,
2019
Received in revised form:
April 19,
2019
Received:
January 23,
2019
Identification
Copyright
© 2019 Elsevier B.V. All rights reserved.
