title: Validity of the 6 minute walk test in facioscapulohumeral muscular dystrophy.
abstract: 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).
  
  
  
  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.
  
  
  
  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.
  
  
  
  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.
authors.affiliation: Department of Neurology, University of Rochester Medical Center, Box 673, 601 Elmwood Avenue, Rochester, New York, 14642.
authors.forename: Katy
authors.initials: K
authors.lastname: Eichinger
authors.affiliation: Department of Neurology, University of Rochester Medical Center, Box 673, 601 Elmwood Avenue, Rochester, New York, 14642.
authors.forename: Chad
authors.initials: C
authors.lastname: Heatwole
authors.affiliation: Department of Neurology, University of Rochester Medical Center, Box 673, 601 Elmwood Avenue, Rochester, New York, 14642.
authors.forename: Susanne
authors.initials: S
authors.lastname: Heininger
authors.affiliation: Center for Genetic Muscle Disorders, Kennedy Krieger Institute, Baltimore, Maryland, USA.
authors.forename: Nikia
authors.initials: N
authors.lastname: Stinson
authors.affiliation: Center for Genetic Muscle Disorders, Kennedy Krieger Institute, Baltimore, Maryland, USA.
authors.forename: Carly
authors.initials: C
authors.lastname: Matichak Stock
authors.affiliation: Center for Genetic Muscle Disorders, Kennedy Krieger Institute, Baltimore, Maryland, USA.|Rady Children's Hospital and Department of Neurosciences, UC San Diego School of Medicine, San Diego, California, USA.
authors.forename: Carla
authors.initials: C
authors.lastname: Grosmann
authors.affiliation: Center for Genetic Muscle Disorders, Kennedy Krieger Institute, Baltimore, Maryland, USA.|Departments of Neurology and Neuroscience, The Johns Hopkins School of Medicine, Baltimore, Maryland, USA.
authors.forename: Kathryn R
authors.initials: KR
authors.lastname: Wagner
authors.affiliation: Department of Neurology, University of Rochester Medical Center, Box 673, 601 Elmwood Avenue, Rochester, New York, 14642.
authors.forename: Rabi
authors.initials: R
authors.lastname: Tawil
authors.affiliation: Department of Neurology, University of Rochester Medical Center, Box 673, 601 Elmwood Avenue, Rochester, New York, 14642.|Department of Neurology, University of Kansas Medical Center, Kansas City, Kansas, USA.
authors.forename: Jeffrey M
authors.initials: JM
authors.lastname: Statland
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D053580:Muscle Strength; D020391:Muscular Dystrophy, Facioscapulohumeral / Q000175:diagnosis* / Q000503:physiopathology*; D011446:Prospective Studies; D015203:Reproducibility of Results; D012189:Retrospective Studies; D012720:Severity of Illness Index; D000070857:Walk Test; D016138:Walking / Q000502:physiology*; D055815:Young Adult
keywords: 6 Minute Walk Test; clinical trials; facioscapulohumeral muscular dystrophy; neuromuscular disease; outcome measures