Katy Eichinger1, Chad Heatwole1, Susanne Heininger1, Nikia Stinson2, Carly Matichak Stock2, Carla Grosmann2,3, Kathryn R Wagner2,4, Rabi Tawil1, Jeffrey M Statland1,5. 1. Department of Neurology, University of Rochester Medical Center, Box 673, 601 Elmwood Avenue, Rochester, New York, 14642. 2. Center for Genetic Muscle Disorders, Kennedy Krieger Institute, Baltimore, Maryland, USA. 3. Rady Children's Hospital and Department of Neurosciences, UC San Diego School of Medicine, San Diego, California, USA. 4. Departments of Neurology and Neuroscience, The Johns Hopkins School of Medicine, Baltimore, Maryland, USA. 5. Department of Neurology, University of Kansas Medical Center, Kansas City, Kansas, USA.
Abstract
INTRODUCTION: In preparation for future clinical trials, we determined the reliability, relationship to measures of disease severity, and consistency across sites of the 6 Minute Walk Test (6MWT) in patients with facioscapulohumeral muscular dystrophy (FSHD). METHODS: Genetically defined and clinically affected FSHD participants at 2 sites performed the 6MWT, the Timed Up and Go, and the 30 foot Go/Timed 10 meter test as measures of mobility using standard procedures. RESULTS: Eight-six participants representing the full range of severity performed the 6MWT. The mean 6MWT distance was 404.3 meters (SD 123.9), with no difference between sites. The 6MWT was reliable (n = 25; intraclass correlation coefficient = 0.99) and demonstrated moderate to strong correlations with lower extremity strength, functional outcomes, and FSHD Clinical Score. CONCLUSIONS: The 6MWT is reliable and is associated with other measures of FSHD disease severity. Future directions include assessing its sensitivity to disease progression. Muscle Nerve 55: 333-337, 2017.
INTRODUCTION: In preparation for future clinical trials, we determined the reliability, relationship to measures of disease severity, and consistency across sites of the 6 Minute Walk Test (6MWT) in patients with facioscapulohumeral muscular dystrophy (FSHD). METHODS: Genetically defined and clinically affected FSHDparticipants at 2 sites performed the 6MWT, the Timed Up and Go, and the 30 foot Go/Timed 10 meter test as measures of mobility using standard procedures. RESULTS: Eight-six participants representing the full range of severity performed the 6MWT. The mean 6MWT distance was 404.3 meters (SD 123.9), with no difference between sites. The 6MWT was reliable (n = 25; intraclass correlation coefficient = 0.99) and demonstrated moderate to strong correlations with lower extremity strength, functional outcomes, and FSHD Clinical Score. CONCLUSIONS: The 6MWT is reliable and is associated with other measures of FSHD disease severity. Future directions include assessing its sensitivity to disease progression. Muscle Nerve 55: 333-337, 2017.
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