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Muscle Nerve. 2017 Mar;55(3):333-337. doi: 10.1002/mus.25251. Epub 2016 Dec 5.

Validity of the 6 minute walk test in facioscapulohumeral muscular dystrophy.

Author information

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.

KEYWORDS:

6 Minute Walk Test; clinical trials; facioscapulohumeral muscular dystrophy; neuromuscular disease; outcome measures

PMID:
27421252
PMCID:
PMC5793210
DOI:
10.1002/mus.25251
[Indexed for MEDLINE]
Free PMC Article

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