Muscular Dystrophy

Introduction

Muscles are made up of elastic tissue. In the human body, more than six hundred muscles are present. Thousands of muscle fibers constitute elastic tissues. Muscle strength depends on the presence of fibers. These muscles work together with the nerve fibers to do all voluntary and involuntary functions of the body. There are three types of muscles: skeletal muscles, striated muscles, and smooth muscles. Muscular dystrophy is the damage and weakening of the muscles. In this tutorial, we will learn about muscular dystrophy in depth.

Dystrophy Meaning

Dystrophy generally occurs due to malnutrition or diseases. Muscular dystrophy is the damage and weakness of the muscles. It is a genetically inherited disease and more than thirty muscular dystrophy diseases are identified till date. It mainly affects the skeleton muscles, over a period of time the muscles become weak and hamper the ability to walk. In case of severity, the affected person will not able to brush his teeth properly. Muscular dystrophy can appear at any age like childhood or adulthood based on the type. It cannot be cured completely but its effect can be subsided.

The main cause of muscle weakness and damage is due to shortfall or absence of protein dystrophin. This dystrophin is essential in the different muscle functions. As a result of this, individuals suffer problems in waking, swallowing, muscle coordination, etc. Muscular dystrophy is a rare condition and most runs in families. A child, who has muscular dystrophy may inherit muted genes that cause muscular dystrophy from parents. Sometimes the carrier adults can pass the muted genes of muscular dystrophy to their offspring.

Types of Muscular Dystrophy

Different types of muscular dystrophy are described below −

• Duchenne Muscular Dystrophy (DMD) − Duchenne muscular dystrophy is one of the most common muscular dystrophies. It generally affects boys in comparison to girls. The affected age group is 2 years. The affected toddler finds it very difficult to walk, run and jump. Further, it may affect the lungs and heart as the disease progress. In Europe and North America, six out of 100000 children are attacked by Duchenne muscular dystrophy.

• Becker Muscular Dystrophy (BMD) − After Duchenne muscular dystrophy, Becker muscular dystrophy is the most common type of muscular dystrophy. The BMD is most common in teenagers but it can appear in the age group between 5 and 60 years. In comparison to women, men are more likely to be affected by Becker muscular dystrophy. This disease mainly occurs in the thigh, shoulder, and hip muscles and as the disease progress, it affects the heart. In the US approximately 18000 to 30000 boys developed Becker muscular dystrophy.

• Facioscapulohumeral Muscular Dystrophy (FSHD) − This type of muscular dystrophy is seen in an individual before the age of twenty. Facioscapulohumeral muscular dystrophy mainly affects the muscles of the upper arm, shoulder blade, and also in facial muscles.

• Congenital Muscular Dystrophy (CMD) − This muscular dystrophy is present at the time of birth. In this, the infected baby's muscles become too weak, joints appear either stiff or loose, and curved spines. Along with these symptoms, they exhibit problems with eyesight, seizures as well as learning disabilities.

• Emery Dreiffus Muscular Dystrophy (EDMD)− Emery Dreiffus muscular dystrophy mainly affects children. Symptoms like weak shoulder, upper arm and calf muscles and appears within the age of ten. Further, this disease affects the heart.

• Limb-girdle Muscular Dystrophy (LGMD) − Limb-girdle muscular dystrophy affects all age groups of people. The hip and shoulder muscles are affected by this disease.

• Myotonic Dystrophy − The individuals suffering from myotonic dystrophy find it hard to relax their muscles. As the diseases progress, it affects the heart and lungs. This disease occurs in adults of European descent.

• Oculopharyngeal Muscular Dystrophy (OPMD) − Oculopharyngeal muscular dystrophy is an unusual form of muscular dystrophy. It usually affects the muscles of the throat and eyes. As a result, an individual suffers from dysphagia (swallowing difficulties) and ptosis (drooping of eyelids).

Muscular Dystrophy Symptoms

The symptoms of muscular dystrophy are listed below −

• Weakness in the muscles.

• Calf muscles become enlarged.

• Not able to walk properly.

• Difficulty in swallowing.

• Heart-related problems like arrhythmia and cardiomyopathy.

• Learning impairment.

• Joints are either stiff or loose.

• Pain in the muscles.

• Scoliosis.

• Trouble in breathing.

Muscular Dystrophy Treatment

As muscular dystrophy is a genetic disorder it cannot be completely cured but symptoms can subside with treatment. The treatment of muscular dystrophy is listed below −

• The strength of the muscles can be increased with the help of occupation and physical therapy. These therapies help muscular movement.

• Speech therapy is given to individuals who have a problem with swallowing and speech delay.

• Surgery is done for scoliosis.

• Pacemakers are implanted to control the rheumatic movement of the heart also in case of heart failure.

• Wheelchairs and walkers are given to the affected individuals to move from one place to another. These devices prevent falls and improve mobility.

• Respiratory therapy or aids are given in case of lung infections.

• The different types of injections like eteplirsen, golodirsen, viltolarsen, and casimersen are given on a weekly basis. Deflazacort is given orally in the form of tablets to treat age groups above five with Duchenne muscular dystrophy.

Conclusion

Muscles are made up of elastic tissue. In the human body, more than six hundred muscles are present. There are three types of muscles: skeletal muscles, striated muscles, and smooth muscles. Muscular dystrophy is the damage and weakening of the muscles. The main cause of muscle weakness and damage is due to shortfall or absence of protein dystrophin. As a result of this, the individuals suffer problems in waking, swallowing, muscle coordination, etc. Muscular dystrophy is a rear condition and mostly runs in families. A child with muscular dropsy inherits muted genes that cause muscular dystrophy from parents. Sometimes the carrier adults can pass the muted genes of muscular dropsy to their offspring.

FAQs

Q1. Write down the complications of muscular dystrophy?

Ans. Muscular dystrophy affects muscles, the heart, and the lungs. In the advantage stage of muscular dystrophy arrhythmias, failure of the heart, pneumonia, choking, and breathing problems can occur.

Q2. What do you understand by arrhythmia?

Ans. After a heart attack sometimes the heart generates irregular heartbeats which is known as arrhythmia. This is due to the fact that heart muscles are damaged by heart attack and derange the electrical signals. The damaged signals make the heart beat too slow or fast.

Q3. How muscular dystrophy can be diagnosed?

Ans. Muscular dystrophy can be diagnosed with the help of enzyme protein blood tests, electromyography, muscular biopsy, and genetic test. In electromyography nerve and muscle electrical activity is diagnosed.

Q4. When do heart failure occur?

Ans. Heart failure occurs when the heart muscles are weak or damaged. They are unable to pump the amount of blood needed by the body.