Journal article
Clinical and neurophysiological biomarkers of disease progression in amyotrophic lateral sclerosis
Muscle & Nerve, Vol.67(1), pp.17-24
2023
Abstract
Introduction/Aims
Rate of disease progression (ΔFS), measured as change in the revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) and body mass index (BMI), are predictors of survival in amyotrophic lateral sclerosis (ALS). Our aim in this study was to assess the utility of these clinical biomarkers along with neurophysiological measures, such as the split hand index (SI), in monitoring disease progression.
Methods
Clinical trial data were collected from 107 patients recruited into the Tecfidera in ALS trial. The prognostic utility of clinical and neurophysiological measures, including ΔFS, BMI, SI, and neurophysiological index (NPI), were assessed cross-sectionally and longitudinally (40 weeks). The outcome measures of disease severity and progression included: (i) ALSFRS-R score; (ii) Medical Research Council (MRC) score; and (iii) forced vital capacity and sniff nasal inspiratory pressure.
Results
Fast-progressor ALS patients (ΔFS ≥1.1) exhibited significantly lower ALSFRS-R and total MRC scores at baseline. A baseline ΔFS score ≥1.1 was associated with a greater reduction in ALSFRS-R (P = .002) and MRC (P = .002) scores over 40 weeks. Baseline BMI <25 was also associated with faster reduction of ALSFRS-R and MRC scores. SI and NPI were associated with disease severity at baseline, but not with subsequent rate of disease progression.
Discussion
Implementation of the assessed clinical and neurophysiological biomarkers may assist in patient management and stratification into clinical trials.
Details
- Title
- Clinical and neurophysiological biomarkers of disease progression in amyotrophic lateral sclerosis
- Authors/Creators
- A. Hannaford (Author/Creator) - The University of SydneyK. Byth (Author/Creator) - Westmead HospitalN. Pavey (Author/Creator) - The University of SydneyR.D. Henderson (Author/Creator) - Department of Neurology Royal Brisbane & Women’s Hospital BrisbaneS. Mathers (Author/Creator) - Calvary Health Care BethlehemM. Needham (Author/Creator) - Murdoch UniversityD. Schultz (Author/Creator) - Flinders Medical CentreP. Menon (Author/Creator) - The University of SydneyM.C. Kiernan (Author/Creator) - Royal Prince Alfred HospitalS. Vucic (Author/Creator) - The University of Sydney
- Publication Details
- Muscle & Nerve, Vol.67(1), pp.17-24
- Publisher
- John Wiley and Sons Inc.
- Identifiers
- 991005541539007891
- Copyright
- © 2022 Wiley Periodicals LLC.
- Murdoch Affiliation
- Centre for Molecular Medicine and Innovative Therapeutics
- Language
- English
- Resource Type
- Journal article
UN Sustainable Development Goals (SDGs)
This output has contributed to the advancement of the following goals:
Source: InCites
Metrics
75 Record Views
InCites Highlights
These are selected metrics from InCites Benchmarking & Analytics tool, related to this output
- Collaboration types
- Domestic collaboration
- Citation topics
- 1 Clinical & Life Sciences
- 1.52 Neurodegenerative Diseases
- 1.52.765 ALS Mechanisms
- Web Of Science research areas
- Clinical Neurology
- Neurosciences
- ESI research areas
- Neuroscience & Behavior