Abstract | INTRODUCTION/AIMS: 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.
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Authors | Andrew Hannaford, Karen Byth, Nathan Pavey, Robert D Henderson, Susan Mathers, Merrilee Needham, David Schultz, Parvathi Menon, Matthew C Kiernan, Steve Vucic |
Journal | Muscle & nerve
(Muscle Nerve)
Vol. 67
Issue 1
Pg. 17-24
(01 2023)
ISSN: 1097-4598 [Electronic] United States |
PMID | 36214183
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Copyright | © 2022 Wiley Periodicals LLC. |
Chemical References |
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Topics |
- Humans
- Amyotrophic Lateral Sclerosis
- Disease Progression
- Prognosis
- Biomarkers
- Body Mass Index
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