Weakness and Fatigue in Diverse Neuromuscular Diseases

Author:

Montes Jacqueline1,Blumenschine Michelle1,Dunaway Sally1,Alter Aliza S.1,Engelstad Kristin1,Rao Ashwini K.2,Chiriboga Claudia A.1,Sproule Douglas M.1,Vivo Darryl C. De1

Affiliation:

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

2. Department of Rehabilitation Medicine (Program in Physical Therapy), and G.H. Sergievsky Center, Columbia University Medical Center, New York, NY, USA

Abstract

Weakness and fatigue are captured by the 6-minute walk test, but the relationship between these symptoms is uncertain. Comparison across neuromuscular diseases has not been examined. A cohort study of 114 patients with spinal muscular atrophy, Duchenne/Becker muscular dystrophy, myasthenia gravis, and energy failure syndromes were included. Percent-predicted distance on the 6-minute walk test was computed from normative values to determine weakness. Fatigue was determined by the decrement in distance from the first to sixth minute. Weakness was seen across all groups (61.9%) but significant fatigue was seen only in spinal muscular atrophy (21.0%). Other groups showed little fatigue. Correlation between weakness and fatigue was significant only in spinal muscular atrophy ( R = –0.71; P < .001). Longitudinally, distance walked declined only in Duchenne/Becker muscular dystrophy. In spinal muscular atrophy, weakness did not change, but fatigue increased significantly. These findings suggest independent mechanisms underlying weakness and fatigue in diverse neuromuscular conditions.

Publisher

SAGE Publications

Subject

Clinical Neurology,Pediatrics, Perinatology, and Child Health

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