Muscular dystrophy and scoliosis

While we specialise in the study and treatment of curvatures of the spine, we also possess an excellent working knowledge of other health problems that are linked to scoliosis. There are many disorders that may lead to a curvature of the spine, and one of the more severe disorders that can causes scoliosis is muscular dystrophy.

What causes muscular dystrophy?

Muscular dystrophy (MD) is a group of diseases that affect the skeletal muscles and cause them to weaken and break down. MD is a progressive condition, meaning it gets worse over time, and it often begins by affecting a particular group of muscles before then affecting the rest of your muscles more widely. 

MD is generally hereditary, and is caused by mutations of genes on the X chromosome. Each disorder is formed due to a different set of mutations, but each type of MD prevents the body from producing dystrophin - a protein that is essential for the building and repairing of muscles. Dystrophin is part of a very complex group of proteins that ensure your muscles work correctly. The protein links various components of the muscle cells together and anchors them to the sarcolemma (outer membrane). If the protein is absent or deformed, this process will not work correctly, and disruptions will occur in the outer membrane. This will weaken the muscle and damage the muscle cells themselves. 

MD can also be caused by a spontaneous mutation. This can happen when there are errors in the replication of DNA and spontaneous lesions occur due to natural damage to the DNA.

Types of muscular dystrophy 

There are many different types of muscular dystrophy, and each disorder differs in which muscles it affects, the degree of muscle weakness caused, how quickly things worsen, and when the symptoms begin.

Here are some common types of muscular dystrophy:

  • Duchenne Muscular Dystrophy - This is the most common form of MD. It generally affects boys in early childhood and is first noticeable when the child starts walking. By age 10, the child may be required to wear a brace for walking; by age 12, most sufferers are unable to walk at all. Sufferers generally do not live beyond their 20s or 30s.

  • Myotonic Dystrophy - This type of MD causes delayed relaxation of muscles, as well as muscle wasting and weakness. Myotonic MD varies in severity and affects many body systems in addition to the skeletal muscles. It can develop at any age, and life expectancy isn't always affected; however, sufferers may live shortened lives.

  • Facioscapulohumeral Muscular Dystrophy (FSHD) - FSHD initially affects the muscles of the face, shoulders and upper arms, then progresses slowly. It usually develops during early adulthood and isn't life-threatening, but individuals who suffer from FSHD will become severely disabled.

  • Becker Muscular Dystrophy - Becker MD is a less severe form of Duchenne MD (see above) that is caused by the production of a shortened but still partially functional form of dystrophin. It affects boys in late childhood, and individuals with this type of MD can live to reach old age.

  • Limb-Girdle Muscular Dystrophy (LGMD) - LGMD affects the upper arms and legs of both boys and girls. A person suffering from LGMD will normally live a normal life with some assistance; however, in some extreme cases, it can cause death due to complications. 

  • Oculopharyngeal Muscular Dystrophy - This type of MD doesn't usually develop until a person is 50-60 years old. It affects the muscles of the eyelids, face, and throat, later followed by pelvic and shoulder weakness. It doesn't tend to affect the patient's life expectancy.

  • Emery-Dreifuss Muscular Dystrophy (EDMD) - EDMD usually develops in childhood or early adulthood. It starts in the hands and feet, then progresses to the arms and legs later on. Most patients will also suffer from cardiac conduction defects and arrhythmias. Most people with this condition live until at least middle age.

Signs and symptoms

The signs and symptoms of muscular dystrophy are:
  • Progressive muscular wasting
  • Poor balance
  • Inability to walk
  • Breathing problems
  • Cardiac problems (due to the muscles of the heart being weakened)
  • Muscle spasms 
  • Scoliosis

The connection between MD and scoliosis

As mentioned above, one common symptom of MD is scoliosis (a sideways curvature of the spine). This happens because the muscles are no longer strong enough to support the spine, causing it to curve over time. Sadly, there is no cure for MD, but there are a range of treatments that can help with the physical disabilities and problems that develop as a result of the disorder. Medicines such as steroids are used to improve muscle strength, while ACE inhibitors and beta blockers can be used to treat heart problems. 

In addition to these medications, physical therapy can used to manage the physical disabilities that are caused by muscular dystrophy. If you suffer from scoliosis due to MD, the Scoliosis SOS Clinic can help - our ScolioGold programme is an exercise-based treatment regime that we use to help scoliosis sufferers combat their symptoms and achieve a higher quality of life.

If you would like to learn more about ScolioGold treatment and how it can help you to manage the symptoms of your condition, please get in touch with us today!