Measuring Fatigue and Fatigability in Spinal Muscular Atrophy (SMA): Challenges and Opportunities

Author:

Rodriguez-Torres Rafael S.12ORCID,Uher David23ORCID,Gay Emma L.4,Coratti Giorgia56ORCID,Dunaway Young Sally7ORCID,Rohwer Annemarie8,Muni Lofra Robert9,De Vivo Darryl C.1,Hirano Michio1,Glynn Nancy W.4ORCID,Montes Jacqueline2ORCID

Affiliation:

1. Department of Neurology, Columbia University Irving Medical Center, New York, NY 10032, USA

2. Department of Rehabilitation and Regenerative Medicine, Columbia University Irving Medical Center, New York, NY 10032, USA

3. Department of Biobehavioral Sciences, Teachers College, Columbia University, New York, NY 10115, USA

4. Department of Epidemiology, School of Public Health, University of Pittsburgh, Pittsburgh, PA 15213, USA

5. Pediatric Neurology Unit, Catholic University, 00135 Rome, Italy

6. Centro Clinico Nemo, U.O.C. Neuropsichiatria Infantile Fondazione Policlinico Universitario Agostino Gemelli IRCCS, 00168 Rome, Italy

7. Department of Neurology and Clinical Neurosciences, Stanford University School of Medicine, Palo Alto, CA 94305, USA

8. Dubowitz Neuromuscular Centre, UCL Great Ormond Street Institute of Child Health, London WC1N 1EH, UK

9. The John Walton Muscular Dystrophy Research Centre, Translational and Clinical Research Institute, Newcastle University and Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne NE1 7RU, UK

Abstract

Fatigue, a common symptom, together with the characteristic of performance fatigability, are well-documented features of SMA that impact quality of life and function. Importantly, establishing associations between multidimensional self-reported fatigue scales and patient performance has proven difficult. This review was conducted to evaluate the various patient-reported fatigue scales applied in SMA, with the objective of considering the limitations and advantages of each measure. Variable use of fatigue-related nomenclature, including conflicting terminology interpretation, has affected assessment of physical fatigue attributes, specifically perceived fatigability. This review encourages the development of original patient-reported scales to enable perceived fatigability assessment, providing a potential complementary method of evaluating treatment response.

Funder

Cure Spinal Muscular Atrophy

Italian Health Ministry

Publisher

MDPI AG

Subject

General Medicine

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