myotonic dystrophy

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Related to Steinert's disease: Proximal myotonic myopathy

myotonic dystrophy

[¦mī·ə¦tän·ik ′dis·trə·fē]
(medicine)
A hereditary disease, transmitted as an autosomal dominant, characterized by lack of normal relaxation of muscles after contraction, slowly progressive muscular weakness and atrophy, especially of the face and neck, cataract formation, early baldness, gonadal atrophy, abnormal glucose tolerance curve, and, frequently, mental deficiency.
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Some years ago, the detection of a form clinically very similar to myotonic dystrophy, but with proximal strength deficit and different genetic basis, resulted in the introduction of DM1 symbol to indicate Steinert's disease and DM2 to indicate this other form, also known as PROMM (Proximal Myotonic Myopathy).
Myotonic muscular dystrophy is often known simply as myotonic dystrophy and is occasionally called Steinert's disease, after a doctor who originally described the disorder in 1909.