Becker Muscular Dystrophy



Becker Muscular Dystrophy

Becker Muscular Dystrophy refers to an allelic disorder caused by non-truncating mutations in the dystrophin gene. It is considered to be a milder form of dystrophinopathy which occurs commonly in males.

Some of the characteristics of this disorder are often fully observed at a later stage in life such as progressive weakness of the muscles, calf hypertrophy, myalgia and cognitive dysfunction – ambulation is usually present beyond the age of 16/20 years.

In an effort to establish the prevalence and genetic profile of Duchenne Muscular Dystrophy (DMD) and Becker Muscular Dystrophy (BMD) in Puerto Rico, Ramos et al. collected data from medical records in all Muscular Dystrophy Association (MDA) clinics in Puerto Rico.

The authors determined that patients with DMD were diagnosed at an earlier age compared to those with BMD because of symptoms related to the latter becoming prominent only later in life.

Molecular genetic testing of dystrophin is considered to be a more reliable measure in the diagnosis of either DMD or BMD; deletion/duplication dystrophin gene analysis needs to be performed first followed by dystrophin gene sequencing analysis, if the initial analysis is deemed non-informative.

While discussing muscle hypertrophy in patients with DMD and BMD, Kornegay et al. mention hypertrophy as being commonly observed in the calves, sartorius, gracilis, adductor longus, semitendinosus and rectus femoris; the ankle was reported to be the most frequently affected in patients with BMD.

The authors highly recommend treatment modalities to be formed after careful consideration of the detrimental consequences associated with muscle hypertrophy namely, exaggerated postural instability and joint contractures.

For this purpose, rehabilitative strategies need to focus on improving muscular strength and addressing postural disparities in order to prevent limitations in mobility and to enhance the individual’s quality of life.

MASS4D® foot orthotics can be used to augment treatment efforts by providing the correct balance of a resistant force to allow optimal muscle function throughout the functional range of motion while coaxing weaker muscles into strengthening and re-establishing joint alignment.

Optimal arthrokinematics is achieved through the optimal alignment of bones and ligaments, further promoting muscle strength and tendon function in the process.

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References:

  1. Ramos, E., Conde, J. G., Berrios, R. A., Pardo, S., Gómez, O., Rodríguez, M. F. M. (2016) Prevalence and Genetic Profile of Duchenne and Becker Muscular Dystrophy in Puerto Rico. Journal of Neuromuscular Diseases: May 2016, pp. 261-266. DOI 10.3233/JND-160147
  2. Mah, J. K. (2015) Duchenne and Becker Muscular Dystrophies: Underlying Genetic and Molecular Mechanisms. Chapter 4. Muscular Dystrophy: A Concise Guide. Springer.
  3. Kornegay, J. N., Childers, M. K., Bogan, D. J., Bogan, J. R., Nghiem, P., Wang, J., Fan, Z., Howard Jr., J. F., Schatzberg, S. J., Dow, J. L., Grange, R. W., Styner, M. A., Hoffman, E. P., Wagner, K. R. (2012) The Paradox of Muscle Hypertrophy in Muscular Dystrophy. Physical Medicine and Rehabilitation Clinics of North America: February 2012, Vol. 23, No. 1, pp. 150-172. DOI: 10.1016/j.pmr.2011.11.014

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