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Updated: Jul 11, 2025
Measurements of Motor Function and Other Clinical Outcome Parameters in Ambulant Children with Duchenne Muscular Dystrophy
Published on: January 12, 2019
Longitudinal Course of Long Finger Flexor Shortening in Males with Duchenne Muscular Dystrophy: A Retrospective
Saskia L S Houwen-van Opstal1, Menno van der Holst2, Michel A A P Willemsen3
1Department of Rehabilitation, Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, The Netherlands ORCID: 0000-0002-9221-5679.
Background:
Shortening of the long finger flexors (Flexor Digitorum Profundus, FDPs) in Duchenne Muscular Dystrophy (DMD) causes reduced hand function. Until now, longitudinal studies on the natural course of the shortening of the FDPs are lacking, which impedes recommendations on timing and evaluation of preventive measures.
Objective:
To investigate the longitudinal course of the FDP length during different disease stages focusing on symmetry, timing, and decline of the FDP length.
Methods:
A retrospective, longitudinal multicenter study was conducted in the Radboud university medical center and the Leiden university medical center. The FDP outcome was measured using goniometry and gross motor function was assessed using the Brooke score. Longitudinal mixed model analyses were used to describe the course of the FDP outcome, and to investigate symmetry in both hands.
Results:
Data on 534 visits of 197 males (age ranged 4-48 years) showed that in the ambulatory stages the FDP outcome was within a normal range. The mean decline in FDP outcome is 3.5 degrees per year, the biggest decline was seen in Brooke 5 (>15 degrees per year). In Brooke 4, 41% of the FDP outcome was < 40 degrees. No significant differences were found between right and left.
Conclusions:
This study supports the consideration of preventive measures to delay shortening of the FDPs in DMD patients transitioning to a Brooke scale of 4 or higher. Besides, natural history of FDP outcome has been established, which provides a base to evaluate (preventive) interventions.
Insights
Shortening of finger flexors in Duchenne Muscular Dystrophy (DMD) accelerates after Brooke 4, impacting hand function. This study establishes the natural history of Flexor Digitorum Profundus (FDP) decline, aiding intervention timing.
Area of Science:
- Neurology
- Orthopedics
- Rehabilitation Medicine
Background:
- Shortening of the long finger flexors (Flexor Digitorum Profundus, FDPs) in Duchenne Muscular Dystrophy (DMD) leads to reduced hand function.
- Longitudinal data on FDP shortening in DMD is lacking, hindering the development of preventive strategies.
- Understanding the natural course of FDP length is crucial for managing hand function in DMD patients.
Purpose of the Study:
- To analyze the longitudinal changes in FDP length across different Duchenne Muscular Dystrophy disease stages.
- To investigate the symmetry of FDP length decline between the right and left hands.
- To establish the timing and rate of FDP shortening in relation to disease progression.
Main Methods:
- Retrospective, longitudinal multicenter study involving 197 males with DMD.
- FDP length measured by goniometry; gross motor function assessed using the Brooke score.
- Longitudinal mixed-effects models used to analyze FDP outcome trends and hand symmetry.
Main Results:
- FDP length remained within normal ranges during ambulatory stages of DMD.
- A mean decline of 3.5 degrees per year in FDP outcome was observed, with accelerated decline (>15 degrees/year) in Brooke 5.
- In Brooke 4, 41% of patients showed FDP outcome less than 40 degrees; no significant inter-hand differences were noted.
Conclusions:
- Preventive measures for FDP shortening should be considered for DMD patients reaching Brooke scale 4 or higher.
- This study provides a natural history benchmark for evaluating the efficacy of interventions aimed at preserving hand function in DMD.
- The findings support proactive management strategies to mitigate the impact of FDP contractures in Duchenne Muscular Dystrophy.
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