Ann Rehabil Med.  2012 Feb;36(1):22-32. 10.5535/arm.2012.36.1.22.

The Correlation Analysis of Functional Factors and Age with Duchenne Muscular Dystrophy

• Jung IY
• Chae JH
• Park SK
• Kim JH
• Kim JY
• Kim SJ
• Bang MS

• KMID: 2266777
• DOI: http://doi.org/10.5535/arm.2012.36.1.22

Abstract

OBJECTIVE
To correlate existing evaluation tools with clinical information on Duchenne muscular dystrophy (DMD) patients following age and to investigate genetic mutation and its relationship with clinical function. METHOD: The medical records of 121 children with DMD who had visited the pediatric rehabilitation clinic from 2006 to 2009 were reviewed. The mean patient age was 9.9+/-3.4 years and all subjects were male. Collected data included Brooke scale, Vignos scale, bilateral shoulder abductor and knee extensor muscles power, passive range of motion (PROM) of ankle dorsi-flexion, angle of scoliosis, peak cough flow (PCF), fractional shortening (FS), genetic abnormalities, and use of steroid.
RESULTS
The Brooke and Vignos scales were linearly increased with age (Brooke (y1), Vignos (y2), age (x), y1=0.345x-1.221, RBrooke2=0.435, y2=0.813x-3.079, RVignos2=0.558, p<0.001). In relation to the PROM of ankle dorsi-flexion, there was a linear decrease in both ankles (right and left R2=0.364, 0.372, p<0.001). Muscle power, Cobb angle, PCF, and FS showed diversity in their degrees, irrespective of age. The genetic test for dystrophin identified exon deletions in 58.0% (69/119), duplications in 9.2% (11/119), and no deletions or duplications in 32.8% (39/119). Statistically, the genetic abnormalities and use of steroid were not definitely associated with functional scale.
CONCLUSION
The Brooke scale, Vignos scale and PROM of ankle dorsi-flexion were partially available to assess DMD patients. However, this study demonstrates the limitations of preexisting scales and clinical parameters incomprehensively reflecting functional changes of DMD patients.

Keyword

Duchenne muscular dystrophy; Dystrophin; Gene deletion; Function; Age

MeSH Terms

Animals
Ankle
Child
Cough
Dystrophin
Exons
Gene Deletion
Humans
Knee
Male
Medical Records
Muscles
Muscular Dystrophy, Duchenne
Range of Motion, Articular
Scoliosis
Shoulder
Weights and Measures
Dystrophin

Figure

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  Fig. 1 The Brooke scale (A), and Vignos scale (B) of Duchenne muscular dystrophy patients with age. A score of 10 on the Vignos scale means dead (*p<0.001).

• 

  Fig. 2 Correlation between age and right ankle dorsi-flexion PROM (A), and left ankle dorsi-flexion PROM (B), PROM: Passive range of motion (*p<0.001).

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  Fig. 3 Bilateral muscle power (N) with age, right (A) and left (B) shoulder abductor, right (C) and left (D) knee extensor.

• 

  Fig. 4 Relationship between age and Cobb angle for scoliosis (A), peak cough flow (B), and fractional shortening (C).

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