Muscular dystrophy

Muscular-dystrophy
Muscular dystrophy is a disease of the musculoskeletal system in which, the voluntary muscles get gradually weakened and degraded. It is an inherited disorder which is caused by a defect or mutation in one gene of the gene pool. This defective gene is a part of those genes which are responsible for generating proteins that are required to protect muscle mass from damage.

It is broadly classified into 9 types and for each type, one particular gene is defective. There are total nine types of muscular dystrophies found. All the nine types have different defective genes and different clinical features. Some of them are discussed below:

  • Myotonic or Steinert’s disease: it is the most widespread form in adults, affecting both the sexes, and usually appears in early childhood. The name is derived from the word myotonia, which means stiffness or spasm in muscle after use. The disease also affects brain, heart, eyes and gastrointestinal tract.
  • Duchenne: most common form among children. It affects only males and occurs from 2 to 6 years of age. Life expectancy in not more than early twenties.
  • Becker: similar to Duchenne form, but the symptoms appear late and the destruction is milder. It also affects only males.
  • Limb-girdle: this affects both males and females and appearance is seen in early adulthood. Affects the hips, shoulder legs and arms. Life expectancy is till late adulthood.
  • Facioscapulohumeral: affects the face, shoulder and upper arm portions. Due to involvement of the face, chewing, speaking and swallowing is difficult. Life expectancy is normal.
  • Congenital: it affects both males and females and is present at birth. This type progresses gradually, causing muscle contractures in first few months of life.
  • Oculopharyngeal: affecting eye and throat, causes weakening of the eye and can cause recurrent pneumonia.
  • Distal: affects the distal parts like hands and feet. It is less severe and progresses slowly.
  • Emery-Dreifuss: it affects only males and appears from late childhood to early teenage. It is less severe than other forms of muscular dystrophies.

Any of the following types does not have any specific treatment, but the discomfort can be reduced and the life expectancy can be increased by various methods like:

Physical therapy, speech therapy, occupational therapy, various orthodontic instruments like wheelchairs and standing frames can be given. Prednisone can be given to avoid muscle contractures. Dietary intake should be restricted with a high protein diet and low on carbohydrate diet. Alcohol and caffeine consumption should be decreased.

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