Myotonic dystrophy (Myotonia atrophica or Steinert’s disease )

 chronic, slowly progressive neuromuscular disease

Etiology:

  • genetic autosomal dominant disorder
  • severe form of muscular dystrophy
  • symptom severity increases and onset age decrease with increased mutuated copies (the number of repeats) of the gene carried by the individual

Manifestations:

  • weakness and muscular wasting that affects the face and feet and hands and neck
  • dysphasia
  • typically difficulty with the hands progresses inferiorly affecting arms and shoulders and legs and hips.

Onset and progression:

  • highly variable multisystemic disorder.
  • slow progression

Management and treatment

the disease is progressive

 management and treatment of symptoms and complications as they arise.

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