Muscular Dystrophy

Muscular Dystrophy

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muscular dystrophy
muscular dystrophy

What is Muscular Dystrophy?

This disease will cause the muscle fibers in your body to weaken and begin to break down making your muscles susceptible to damage. Muscular Dystrophy is not just one disease but an umbrella term for a list of more than thirty muscle related diseases. This disease affects your skeletal or voluntary muscles. These are the muscles that control the movements of your arms, legs, and trunk. It can also affect involuntary muscles such as the ones in your stomach and even your heart muscles. It is a chronic disease that affects your mobility. This disease can start in infancy or childhood or it may wait to appear when a person reaches middle age. Muscular Dystrophy is a disease that is more prominent in boys than girls and is a progressive disease. There are more than fifty thousand people in the United States that are suffering from some type of Muscular Dystrophy.

Types of Muscular Dystrophy

Muscular Dystrophy has nine types that are more commonly known. They are based on the age that the symptoms first appear and where the location is of the muscles that are involved. The type is also based on how fast or slow that the symptoms are progressing and how the bad gene was passed on to that person. These nine types include:

Duchenne Muscular Dystrophy

This is the one that is most common in children between the ages of two and six years old and will usually affect boys. Girls may have the gene but not experience any of the symptoms. This is the most severe form of this disease.

Becker’s Muscular Dystrophy

This is also another commonly known type and affects the age group of two to sixteen years old. The progression of this type is very slowly.

Congenital Muscular Dystrophy

This is the type that is present at birth.

Emery-Dreifuss Muscular Dystrophy

This type affects people from childhood to early teenage years.

Distal Muscular Dystrophy

This type is usually found in people in late adulthood in the age group of twenty to sixty years of age.

Myotonic Muscular Dystrophy

This type is the most common for of adult Muscular Dystrophy with the age group being between the ages of twenty and sixty but there have been some people under the age of twenty that have had this type. It is also known as Steinert’s Disease.

Facioscapulohumeral Muscular Dystrophy

This type is also referred to as Landouzy-Dejerine Dystrophy. It can affect age groups from teenagers to early adulthood.

Oculopharyngeal Muscular Dystrophy

This type will usually effect the ages between forty and seventy.

Limb-Girdle Muscular Dystrophy

This type can affect people from late childhood to middle age.

Symptoms

Many of the different forms of Muscular Dystrophy have their own set of symptoms but there are some common symptoms that include:

  • Weakness of your muscles
  • An apparent lack of coordination
  • Obesity
  • Progress muscle wasting
  • Lost of function of your muscles
  • Joint contractures
  • Frontal baldness
  • Drooping eyelids
  • Cataracts
  • Gonadal atrophy
  • Mental impairment
  • Difficult in walking
  • Calf pain
  • Having a gait that makes you look like you are waddling
  • Falling down frequently

Becker MD

The symptoms of this type may start later and are less severe. The onset of symptoms usually starts around the age of eleven. The symptoms are similar to those that suffer from Duchenne’s Muscular Dystrophy but also include:

  • Muscle breakdown
  • Weakness
  • Problems that is related to the heart, joints, bones, and breathing.

Duchenne MD

  • Weakening of the pelvic muscles
  • Arms and legs become weak, especially in the lower leg muscles
  • Having calf muscles that are larger than they are suppose to be
  • Having a gait that looks as if you are waddling
  • Falling down frequently

Emery-Dreifuss MD

The symptoms usually start in the muscles of your upper arms, shins, and shoulders.

  • Joint deformities
  • Cardiac problems
  • Stiffness of the spine
  • Muscle contractures

Facioscapulohumeral MD

Weakness of the muscles of your face, abdomen, shoulders, lower and upper arms, pelvic area and feet.

Myotonic MD

  • Muscles which control your legs and arms become weak. They are referred to as voluntary muscles
  • Your neck, head, and face muscles start to become weak
  • Muscles involved in breathing and swallowing become weak
  • Muscles of your internal organs that are hollow become weak
  • Fainting or dizziness
  • Difficulty with sleeping during the night
  • Sleepiness during the daytime
  • Frontal balding, predominately in men
  • Cataracts

Congenital MD

  • Muscle weakness that is general
  • Joint deformities
  • Possible brain malfunctions

Causes

In order for a person to have muscles that are healthy and function properly they need a constant supply of proteins. With Muscular Dystrophy there is a lack of that particular muscle protein. As a result your muscles are not receiving the nutrients that are required to function properly. Over time your muscles will start to weaken and you will loose muscle function. The gene that is defective and responsible for this disease comes from your parents and is there when you are born but you may not show any symptoms until later in life. Many of the different types of Muscular Dystrophy have their own cause.

Becker and Duchenne MD

The cause of this type is that it is a sex-linked recessive disease which means that there is a bad gene present in the X-chromosome. This is passed from mother to son. The carrier is the mother of this type but she may or may not have any symptoms. The one that has this type is unable to synthesize the dystrophin which is a protein that is found in the muscle fiber membrane.

Myotonic MD

This type is a type of mutation that happens in the gene which codes for the protein kinase. In a person that is healthy the sequence that is present is triple repeat but in a person who has this type the sequence is repeated differently, usually many times.

Limb-Girdle MD

This type is passed on from one of the parents or both of them. It comes from an autosomal inheritance dominant pattern mutated gene. There are thirty types of this type.

Facioscapulohumeral MD

It can be inherited from just one parent and is an autosomal inheritance dominate pattern mutated gene.

Oculopharyngeal MD

In this type there are both patterns of autosomal inheritance dominant and recessive. The gene that is abnormal is gotten from one parent in the dominant inherited case and in the recessive pattern the defective gene is passed from both parents.

Congenital MD

With this type both autosomal inheritance patterns are seen.

Treatment

Unfortunately there is no cure for Muscular Dystrophy or any particular treatment that can stop the growth or development. The treatment that is used is to help those with Muscular Dystrophy curb the chances of developing severe muscle contractions in their calf muscles or joints. Some of these treatments include:

  • Physical therapy such as certain deep breathing exercise to help with the joint pain and to help slow down the process of spine concaveness.
  • Deep breathing exercises to help reduce the risk of respiratory failure.
  • Helping them learn to navigate in a wheelchair to help them become more independent.
  • Taking certain medications to help curb and relieve muscle weakness and wastage such as quinine, corticosteroids, and phenytoin.

Life Expectancy

The life expectancy of someone with Muscular Dystrophy depends on the type that they have because it is different for each type.

Duchenne MD

They rarely live past twenty to twenty-five years of age

Becker MD

People with this type usually live a long life but how long depends on how severe it is and how it progresses. They may or may not be dependent on a wheelchair.

Congenital MD

Because this type is present at birth the infant may have a short lifespan.

Emery-Dreifuss MD

Because this type causes severe cardiac complications and can lead to sudden death the life span is questionable.

Myotonic MD

The rate of progression of this type of Muscular Dystrophy is fairly slow and since it affects adults, this person can have a lifespan up to sixty years.

Facioscapulohumeral MD

A person with this type can expect to live many decades after it begins to affect them.

Oculopharyngeal MD

The lifespan for a person who has this type varies because with this type the weakness of their throat muscles can lead to further complications such as the inability to swallow which can lead to severe nutritional deficiency and shorten their life.

Limb-Girdle MD

This type is progresses slowly so the lifespan varies. Most with this type die because of cardiopulmonary complications.

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