Becker Muscular Dystrophy

Characteristics

Becker muscular dystrophy is a hereditary muscle disorder that primarily affects boys and men. The disease causes muscles to gradually break down and weaken, but the progression is highly variable. Some boys may experience difficulty walking early and require a wheelchair before the age of 20, while others may only suffer from muscle cramps during exertion, even into old age.

Heart muscle involvement is also common but varies widely in severity. Muscle weakness in the limbs does not always correlate with the degree of heart muscle weakness, leading to significant differences between patients.

Cause

Becker muscular dystrophy is considered a milder form of Duchenne muscular dystrophy, as both diseases involve the same protein: dystrophin. In Duchenne muscular dystrophy, dystrophin is completely absent, while in Becker muscular dystrophy, the body produces a shortened and reduced form of the protein.

Dystrophin plays a crucial role in protecting muscle fibers from damage during movement. The defect in dystrophin production is caused by a mutation on the X chromosome, which explains why the condition primarily affects boys and men, who have only one X chromosome. Girls and women, with two X chromosomes, are less likely to be affected.

Treatment

While there is no cure for Becker muscular dystrophy, good monitoring and treatment can significantly improve outcomes:

• Heart Care: Medications are prescribed if heart function declines, and in some cases, an implantable cardioverter defibrillator (ICD) may be needed to manage dangerous heart rhythm disturbances.
• Respiratory Support: Younger patients with severe muscle weakness may require assistance with breathing.
• Orthopedic Care: Foot deformities can be corrected by an orthopedic surgeon.
• Physiotherapy: Regular physical therapy helps maximize muscle function and prevent joint and muscle contractures.

A multidisciplinary approach is essential to address the many aspects of the disease. Collaboration among specialists in pediatric and adult care ensures optimal management of symptoms and progression.