Distrofia muscular de Duchenne versus distrofia muscular de Becker: revisión sistemática

Abstract

Context: Muscular dystrophies are a group of hereditary, degenerative, and progressive disorders of the skeletal striated muscle, whose main clinical manifestation is the weakness of the proximal skeletal striated musculature. Objective: To explain the main differences between Duchenne and Becker Muscular Dystrophy. Methodology: Systematic review of studies published in Scopus and PubMed in the last 5 years in Spanish and English, using tools for risk of bias assessment: JBI Critical Appraisal Tools. Results: A total of 17 articles were included, comprising case-control studies and observational studies, describing categories such as clinical presentations, ages of symptom onset for each dystrophy, and therapeutic approaches. Discussion: Duchenne Muscular Dystrophy (DMD) is a severe, progressive, X-linked neuromuscular disorder that primarily affects males, while Becker Muscular Dystrophy (BMD) is usually characterized by a milder and more varied phenotype, in which dystrophin can be produced, but in smaller amounts or with reduced function due to a characteristic mutation known as “in frame.” Conclusions: Duchenne Muscular Dystrophy presents greater severity than Becker Muscular Dystrophy, with onset ages of 1-3 years and 12-15 years, respectively. Although both present similar symptoms, DMD progresses rapidly, while BMD progresses more slowly. Identification is based on symptomatology, genetic testing, and biopsy showing disorganization of muscle fibers. There is no cure, and multidisciplinary palliative treatment aims to extend life expectancy, which is lower in DMD.