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Measurements of Motor Function and Other Clinical Outcome Parameters in Ambulant Children with Duchenne Muscular Dystrophy
Published on: January 12, 2019
The 6-minute walk test in Duchenne/Becker muscular dystrophy: longitudinal observations
Craig M McDonald1, Erik K Henricson, Jay J Han
1Department of Physical Medicine and Rehabilitation, University of California Davis School of Medicine, Sacramento, California 95817, USA. cmmcdonald@ucdavis.edu
Insights
The 6-minute walk distance (6MWD) declines in Duchenne/Becker muscular dystrophy (DBMD) boys, with many losing ambulation. Preserving walking ability is crucial for DBMD treatment goals.
Area of Science:
- Neurology
- Pediatrics
- Rehabilitation Medicine
Background:
- Duchenne/Becker muscular dystrophy (DBMD) is a progressive genetic disorder affecting muscle strength.
- Characterizing ambulation changes over time is essential for assessing disease progression and treatment efficacy in DBMD.
- The 6-minute walk distance (6MWD) is a common functional measure used in DBMD studies.
Purpose of the Study:
- To evaluate the changes in 6-minute walk distance (6MWD) over time in boys with Duchenne/Becker muscular dystrophy (DBMD).
- To compare ambulation changes in DBMD patients with healthy age-matched controls.
- To identify factors influencing ambulation changes in DBMD.
Main Methods:
- Assessed 6-minute walk distance (6MWD) in 18 DBMD boys and 22 healthy boys (ages 4-12 years) over approximately one year.
- Monitored height and weight changes in both groups.
- Analyzed the relationship between 6MWD changes, stride length, and age.
Main Results:
- 6MWD decreased significantly in DBMD subjects (average -57 meters, -15.9%) over 52 weeks.
- In contrast, 6MWD increased slightly in healthy subjects (average +13 meters, +2.1%).
- Two DBMD subjects lost ambulation entirely; changes were age-dependent, with younger DBMD patients showing decline and older patients worsening, while healthy subjects remained stable.
Conclusions:
- The 6-minute walk distance (6MWD) is a valid endpoint for tracking ambulation decline in DBMD.
- Significant ambulation loss occurs in DBMD boys within one year.
- Maintaining ambulation should be a primary therapeutic objective for DBMD.
Abstract:
In this study we used the 6-minute walk distance (6MWD) to characterize ambulation over time in Duchenne/Becker muscular dystrophy (DBMD). The 6MWD was assessed in 18 boys with DBMD and 22 healthy boys, ages 4-12 years, over mean [range] intervals of 58 [39-87] and 69 [52-113] weeks, respectively. Height and weight increased similarly in both groups. At 52 weeks, 6MWD decreased in 12 of 18 (67%) DBMD subjects (overall mean [range]: 357 [125-481] to 300 [0-510] meters; Δ -57 meters, -15.9%), but increased in 14 of 22 (64%) healthy subjects (overall mean [range]: 623 [479-754] to 636 [547-717] meters; Δ +13 meters, +2.1%). Two DBMD subjects lost ambulation. Changes in 6MWD depended on stride length and age; improvements usually occurred by 7-8 years of age; older DBMD subjects worsened, whereas older healthy subjects were stable. The 6MWD changes at 1 year confirm the validity of this endpoint and emphasize that preserving ambulation must remain a major goal of DBMD therapy.

