Becker Muscular Dystrophy in Pediatric Neurology at Sheikh Shakhbout Medical City
What is Becker Muscular Dystrophy?
Becker Muscular Dystrophy (BMD) is a genetic disorder characterized by progressive muscle weakness and degeneration. It primarily affects boys due to its X-linked inheritance pattern, where the mutated gene is passed from carrier mothers to their sons. Unlike Duchenne Muscular Dystrophy, which is more severe, BMD progresses more slowly and presents with milder symptoms, often becoming noticeable in late childhood or early adolescence
Symptoms and Diagnosis
Symptoms of Becker Muscular Dystrophy typically manifest between the ages of
5 and 15 years, although they can appear later. Early signs may include:
- Delayed walking: Children may start walking later than their peers.
- Muscle weakness: Initial weakness often affects the hips, pelvis, and thighs, leading to difficulties in activities such as running and climbing stairs.
- Gait changes: Affected individuals may develop a waddling gait or walk on their toes due to muscle weakness.
As the condition progresses, it can lead to more significant challenges, including difficulty lifting objects and potential use of mobility aids like wheelchairs in later life
Diagnostic Process
Diagnosing Becker Muscular Dystrophy involves a comprehensive evaluation by healthcare professionals at Sheikh Shakhbout Medical City. The diagnostic process includes:
- Family history assessment: Understanding genetic background is crucial.
- Physical examination: A thorough examination helps identify patterns of muscle weakness.
- Blood tests: Elevated levels of creatine kinase (CK) can indicate muscle damage.
- Genetic testing: Identifies mutations in the dystrophin gene responsible for BMD.
- Muscle biopsy and electromyography (EMG): These tests help confirm muscle involvement and differentiate BMD from other neuromuscular disorders
Treatment Options
Currently, there is no cure for Becker Muscular Dystrophy; however, management strategies focus on maintaining quality of life and maximizing mobility. Treatment options may include:
- Physical therapy: Customized exercise programs to enhance strength and flexibility.
- Occupational therapy: Assistance with daily living activities to promote independence.
- Medications: Corticosteroids may be prescribed to prolong ambulation capabilities.
- Multidisciplinary care: A team approach involving pediatric neurologists, cardiologists, physiotherapists, and occupational therapists ensures comprehensive management of symptoms
Prognosis and Living with Becker Muscular Dystrophy
The progression of Becker Muscular Dystrophy varies significantly among individuals. While some may require a wheelchair by their twenties or thirties, others can maintain mobility into later life with appropriate interventions. Most individuals with BMD can lead fulfilling lives, often reaching adulthood without severe complications. However, regular monitoring for potential heart issues is essential, as cardiac involvement can occur due to muscle degeneration affecting the heart
At Sheikh Shakhbout Medical City, pediatric neurology specialists are dedicated to providing comprehensive care for children with Becker Muscular Dystrophy. Through early diagnosis and tailored treatment plans, they aim to enhance the quality of life for affected children and support their families in navigating this challenging condition
