Neck flexor weakness at diagnosis predicts respiratory impairment in amyotrophic lateral sclerosis.
amyotrophic lateral sclerosis
cohort studies
epidemiology
survival analysis
Journal
European journal of neurology
ISSN: 1468-1331
Titre abrégé: Eur J Neurol
Pays: England
ID NLM: 9506311
Informations de publication
Date de publication:
04 2021
04 2021
Historique:
received:
21
10
2020
accepted:
03
12
2020
pubmed:
15
12
2020
medline:
13
8
2021
entrez:
14
12
2020
Statut:
ppublish
Résumé
The purpose was to assess the prognostic role of neck muscle weakness at diagnosis in amyotrophic lateral sclerosis (ALS) patients with respect to survival and respiratory impairment. A retrospective cohort study was conducted. All ALS patients seen in the Turin ALS Centre from 2007 to 2014 were included. Muscle strength at diagnosis was evaluated using the Medical Research Council (MRC) scale. Survival was considered as the time from diagnosis to death or tracheostomy; time to respiratory impairment was considered as the interval from diagnosis to the first event amongst an ALS Functional Rating Scale revised item 10 <4, forced vital capacity <70%, start of non-invasive ventilation or tracheostomy. Time from diagnosis to dysarthria, dysphagia and walking impairment were considered as secondary outcomes. Cox proportional hazard regression models adjusted for sex, age at diagnosis, diagnostic delay, onset site, genetics status and the MRC scores of other muscle groups were used to assess the prognostic role of neck muscles. A total of 370 patients were included in the study. Fifty-nine (15.9%) patients showed neck flexor weakness at diagnosis; MRC values were mostly in agreement for neck extensors. Neck flexors were the only muscles able to predict survival (hazard ratio 0.49, 95% confidence interval 0.28-0.86; p = 0.01). Furthermore, neck flexor normal strength decreased the risk of respiratory impairment (hazard ratio 0.46, 95% confidence interval 0.22-0.96; p = 0.04) but did not influence any secondary outcomes. Neck flexor weakness at diagnosis predicts survival and respiratory impairment in ALS. This result could be valuable for both planning of patients' interventions and clinical trials' design.
Sections du résumé
BACKGROUND AND PURPOSE
The purpose was to assess the prognostic role of neck muscle weakness at diagnosis in amyotrophic lateral sclerosis (ALS) patients with respect to survival and respiratory impairment.
METHODS
A retrospective cohort study was conducted. All ALS patients seen in the Turin ALS Centre from 2007 to 2014 were included. Muscle strength at diagnosis was evaluated using the Medical Research Council (MRC) scale. Survival was considered as the time from diagnosis to death or tracheostomy; time to respiratory impairment was considered as the interval from diagnosis to the first event amongst an ALS Functional Rating Scale revised item 10 <4, forced vital capacity <70%, start of non-invasive ventilation or tracheostomy. Time from diagnosis to dysarthria, dysphagia and walking impairment were considered as secondary outcomes. Cox proportional hazard regression models adjusted for sex, age at diagnosis, diagnostic delay, onset site, genetics status and the MRC scores of other muscle groups were used to assess the prognostic role of neck muscles.
RESULTS
A total of 370 patients were included in the study. Fifty-nine (15.9%) patients showed neck flexor weakness at diagnosis; MRC values were mostly in agreement for neck extensors. Neck flexors were the only muscles able to predict survival (hazard ratio 0.49, 95% confidence interval 0.28-0.86; p = 0.01). Furthermore, neck flexor normal strength decreased the risk of respiratory impairment (hazard ratio 0.46, 95% confidence interval 0.22-0.96; p = 0.04) but did not influence any secondary outcomes.
DISCUSSION
Neck flexor weakness at diagnosis predicts survival and respiratory impairment in ALS. This result could be valuable for both planning of patients' interventions and clinical trials' design.
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
1181-1187Informations de copyright
© 2020 European Academy of Neurology.
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