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Quantitative sonographic assessment of relaxed and contracted muscle thickness predicts survival in ALS
David Kravitz1, Tahani Sheikh Saker1, Noga Odess2
1Neuromuscular Diseases Unit, Department of Neurology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel.
Objectives:
To explore the ability of quantitative sonographic assessment of muscle thickness to predict mortality in amyotrophic lateral sclerosis (ALS) patients compared with manual muscle testing (MMT) and the ALS functional rating scale-revised (ALSFRS-R).
Methods:
86 prospectively recruited ALS patients underwent clinical assessment and quantitative sonographic assessment of muscle thickness in 8 relaxed and 4 contracted limb muscles. Average monthly decline rates of MMT and ALSFRS-R scores and measured relaxed and contracted muscle thickness were calculated at study entry. The area under the curve (AUC), optimal cutoff points, and hazard ratio (HR) for 1 to 3-year mortality were calculated and adjusted for covariates.
Results:
Significant increased 1-year mortality was associated only with lower contracted muscle thickness (HR-8.1), while increased 3-year mortality was in greater correlation with a reduced contracted muscle thickness (HR-4.85) than with a decline in MMT (HR-3.31) and ALSFRS-R (HR-2.12) or a reduced relaxed muscle thickness (HR-2.65).
Conclusion:
Reduced limb muscle thickness, especially in a contracted state, is associated with significantly increased mortality in ALS.
Significance:
Sonographic muscle measurement is a novel estimator of ALS mortality and has the potential to serve as an additional biomarker in future multi-variable prognostication models, clinical decision making and research.

