Muscular Dystrophy

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Muscular Dystrophy (MD) is a group of muscle diseases that weaken the musculoskeletal system and hamper locomotion. Muscular dystrophies are characterized by progressive skeletal muscle weakness, defects in muscle proteins, and the death of muscle cells and tissue. These damaged muscles become progressively weaker. Most people who have muscular dystrophy will eventually need to use a wheelchair.

Causes

These conditions are generally inherited, and the different muscular dystrophies follow various inheritance patterns. However, mutations of the dystrophin gene and nutritional defects (with no genetics history) at the prenatal stage are also possible in about 33% of people affected by DMD.The main cause of the Duchenne and Becker types of muscular dystrophy is the muscle tissue’s cytoskeletal impairment to properly create the functional protein dystrophin and  dystrophin-associated protein complex. Dystrophin protein is found in muscle fibre membrane, its helical nature allows it to act like a spring or shock absorber. In addition to mechanical stabilization, dystrophin also regulates calcium levels

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Typesmuscular-dystrophy-types

  1. Becker’s muscular dystrophy
  2. Congenital muscular dystrophy
  3. Duchenne muscular dystrophy
  4. Distal muscular dystrophy
  5. Emery-Dreifuss muscular dystrophy
  6. Facioscapulohumeral muscular dystrophy
  7. Limb-girdle muscular dystrophy
  8. Myotonic muscular dystrophy
  9. Oculopharyngeal muscular dystrophy

Symptoms include

  • Intellectual disability (only present in some types of the condition)
  • Muscle weakness that slowly gets worse
    • Delayed development of muscle motor skills
    • Difficulty using one or more muscle groups
    • Drooling
    • Eyelid drooping (ptosis)
    • Frequent falls
    • Loss of strength in a muscle or group of muscles as an adult
    • Loss in muscle size
    • Problems walking (delayed walking)

Treatment

The goal of treatment is to control symptoms.

  1. Physical therapy may help patients maintain muscle strength and function.
    1. Stabilize your breathing and heart functions. Exercise can lead to an increase in blood pressure and shortness of breath, so make sure you undergo tests on your cardiovascular and respiratory systems before you start doing physical therapy.
      • Doctors may prescribe an oxygen machine, sleep apnea device or ventilators for muscular dystrophy patients that have problems with breathing.
      • In severe cases, a pacemaker may be inserted into the body to regulate the beating of the heart.
    2. Begin physical therapy treatment with supervised exercise. It may take several weeks or months before you are able to develop an at-home exercise routine. Undergo an initial consultation.
    3. Begin low-impact cardiovascular exercise. Under the guidance of your physical therapist, begin regular swimming, walking on flat surfaces and/or bike riding. Schedule exercise that is energizing, rather than tiring.
      • For example, schedule a 10 to 20 minute walk or swim every day rather than an hour of activity every few days. Exercise that encourages cramping the next day will not have the positive impact that light to moderate exercise will.
      • The aim of regular exercise is to keep muscles in shape. It can also reduce weight, leaving a lower burden on joints, tendons and muscles.
    4. Develop a range-of-motion exercise routine. These prescribed exercises are tailored to your body to promote joint flexibility. Doing these exercises every day is likely to increase mobility and lessen your risk of contractures.
      • For example, a type of range-of-motion exercise for the shoulders involves raising your arms over your head. You would first grasp your weaker arm with your stronger arm at the wrist, then raise it above your head.
      • These exercises should be simple enough to begin at home and do regularly. Stop the exercise immediately if it causes a severe increase in pain. You should not try to push your joints past the point where they move.
      • Sometimes, doing exercises after warming up with cardiovascular activity will increase your mobility further.
    5. Return to your physical therapist to adjust your exercises. Muscular dystrophy patients must adjust their exercise routine as the condition progresses. Return every few months to assess any changes that need to be made in your plan.
  2. Mobility aids. If you are suffering from muscle weakness, a cane, wheelchair or walker is needed to reduce the risk of falling. But you should try a full physical therapy regime first before using an aid, or you can use an aid when performing the exercises.
  3. Orthopedic appliances such as braces and wheelchairs can improve mobility and self-care abilities.
  4. In some cases, surgery on the spine or legs may help improve function.
  5. Other than that, corticosteroids taken by mouth are sometimes prescribed to children to keep them walking for as long as possible.

The person should be as active as possible. Complete inactivity (such as bed rest) can make the disease worse.

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