Myotonic dystrophy, also called Curschmann and Steiner disease is a condition in which muscle atrophy associated with myopathy Thomsen type myotonia and hereditary endocrine disorders. Myotonic dystrophy is a hereditary and family, are more common in men than in women, and the observation of a rare degenerative. Started between 20 and 30 years. The patients had a family history of properties such as high infant mortality, many abortion, sterility, cataracts the beginning of each successive generation.
In myotonic dystrophy, the affected muscles showed a gradual loss of longitudinal and transverse striation and infiltration of the muscle fibers and fat drops of nuclear proliferation. While in the nervous system have been checked degenerative lesions in the diencephalon, and the vegetative nucleus splanchnic nerve, lateral and anterior horn of the spinal cord and posterior. Muscle atrophy that occurs in this disease occurs in the neck, face and distal limbs, myopathy kind uncontracted fibrils.
The condition occurs in myotonic hand, sternocleidomastoid muscle and eyelid. Endocrine and dystrophic signs of premature baldness comprise, eye cataracts, testicular atrophy, mumps, kyphosis or lordosis, with or without hypothyroidism, hypogonadism.
The prognosis is not good, as the disease progresses slowly and patients end up as myopathy.
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