MD: Many Forms, Many Differences

Pediatric neurologist Dr. Daniel Katz of Cotton-O'Neil Clinic says a piece of muscle is an amazingly intricate thing. But when a person has muscular dystrophy, the muscles don't work well.

Dr. Katz says it can be for a variety of reasons. He says sometimes it's a problem with the muscle itself, sometimes it's the connection between the nerve and the muscle, sometimes it's the actual nerve, sometimes it's the signal from the spine, and the list goes on.

Muscular dystrophy refers to a group of more than forty diseases. Different diseases strike at different ages; some are more common in boys than girls. Because the forms can affect people so differently, diagnosis can take time.

Dr. Katz says muscular dystrophy could show up as a delay in development with muscle coordination as a child, or it may show up later as acquiring weakness.

While there is no cure for MD, Dr. Katz says doctors know what complications each form is likely to bring, and they watch for and treat those. For example, weakness may mean braces or wheelchairs, and some patients may be susceptible to breathing problems and pneumonia.

Though much is still unknown about the muscular dystrophies, Dr. Katz says much has been discovered. He says research into MD has brought a lot of understanding in how the whole motor system of the body works, how the system from the brain to the spinal cord to the nerves to the muscles works.

With that understanding comes hope for better treatments, and cures, for MD and more.

You can join the fight against muscular dystrophy by watching the annual Jerry Lewis MDA Labor Day Telethon. It begins Sunday, Sept. 5, following 13 News at 10 and runs through 6 p.m. Labor Day.

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Muscular Dystrophy

  • Muscular dystrophy (MD) refers to a group of genetic diseases characterized by progressive weakness and degeneration of the skeletal or voluntary muscles that control movement.

  • The muscles of the heart and some other involuntary muscles are also affected in some forms of MD, and a few forms involve other organs as well.

  • The major forms of MD include myotonic, Duchenne, Becker, limb-girdle, facioscapulohumeral, congenital, oculopharyngeal, distal and Emery-Dreifuss.

  • Duchenne is the most common form of MD affecting children, and myotonic MD is the most common form that affects adults.

Treatment

  • There is no specific treatment for any of the forms of MD.

  • Physical therapy to prevent contractures (a condition in which shortened muscles around joints cause abnormal and sometimes painful positioning of the joints), orthoses (orthopedic appliances used for support) and corrective orthopedic surgery may be needed to improve the quality of life in some cases.

  • The cardiac problems that occur with Emery-Dreifuss MD and myotonic MD may require a pacemaker.

  • The myotonia (delayed relaxation of a muscle after a strong contraction) occurring in myotonic MD may be treated with medications such as phenytoin or quinine.

Prognosis

  • The prognosis of MD varies according to the type of MD and the progression of the disorder.

  • Some cases may be mild and very slowly progressive, with normal lifespan, while other cases may have more marked progression of muscle weakness, functional disability and loss of ambulation.

  • Life expectancy may depend on the degree of progression and late respiratory deficit. In Duchenne MD, death usually occurs in the late teens to early 20s.

Source: www.ninde.nih.gov (National Institute of Neurological Disorders Web site) contributed to this report.


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