1. Neurophysiological indices in amyotrophic lateral sclerosis correlate with functional outcome measures, staging and disease progression.
- Author
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Barp A, Lizio A, Gerardi F, Tarlarini C, Mauro L, Sansone VA, and Lunetta C
- Subjects
- Aged, Cohort Studies, Cross-Sectional Studies, Female, Humans, Male, Middle Aged, Vital Capacity physiology, Amyotrophic Lateral Sclerosis diagnosis, Amyotrophic Lateral Sclerosis physiopathology, Disease Progression, Electromyography methods, Neural Conduction physiology, Outcome Assessment, Health Care methods
- Abstract
Objective: This study examined neurophysiological (NI), split-hand (SI) and split-leg (SLI) index in patients with amyotrophic lateral sclerosis (ALS), and their correlation with functional status, disease duration, staging and survival., Methods: Eighty-two patients underwent nerve conduction study to analyze NI, SI and SLI. Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R), disease progression rate (ΔFS), Milano-Torino (MiToS) and King's staging systems, Forced Vital Capacity (FVC), and survival data were collected., Results: Both NI and SI indices were significantly associated with ALSFRS-R, MiToS, King's and FVC. Slow progressor patients (ΔFS < 0.5) reported a significantly higher NI and SI values compared to both normal (0.5 ≤ ΔFS < 1.00) and fast progressors (ΔFS ≥ 1.0). After dichotomizing patients in slow progressors (ΔFS < 0.5) and not-slow progressors (ΔFS ≥ 0.5), a combination of SI index and disease duration revealed to be the best prediction model to discriminate patients in accordance with their disease progression (c-index: 0.92), leading to a new prognostic index: the 'Split-Hand prognostic index' (SHpi)., Conclusion: SI and NI are correlated with functional status and FVC. SHpi index could represent an useful tool to discriminate patients in accordance with their disease progression., Significance: These data provide novel evidence of neurophysiological indices as promising biomarkers in ALS., Competing Interests: Declaration of Competing Interest CL received compensation for occasional scientific consulting from Neuraltus, Cytokinetics, Mitsubishi Tanabe Pharma Europe and Italfarmaco and has received funds from ARISLA and Italian Ministry of Health. VAS participates in Advisory Boards or Teaching activities for Biogen, Roche, Avexis, PTC, Santhera, Sarepta, Dyne. The remaining authors have nothing to declare., (Copyright © 2021 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved.)
- Published
- 2021
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