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- W2138695957 abstract "Myotonic dystrophy (MD), or Steinert disease, is an autosomal dominant, neuromuscular multisystemic disease that is characterized by a progressive muscular weakness as well as respiratory, cardiac, ophthalmologic, endocrine, and gastrointestinal ailments. 1 Russell S.H. Hirsh N.P. Anaesthesia and myotonia. Br J Anaesth. 1994; 72: 210 Crossref PubMed Scopus (118) Google Scholar It is estimated currently that approximately 30,000 people are affected in the United States with an incidence of 2.4 to 5.5 cases per 100,000. 2 Steinert H. Myopathologische Beiträge I. Uber das klinishce und anatomische. Bild des Mukelschwunds der Myotoniker. Deutsch Z Nervenheilkd. 1909; 37: 58 Crossref Scopus (118) Google Scholar , 3 Aldridge L.M. Anaesthetic problems in myotonic dystrophy. Br J Anaesth. 1985; 57: 1119 Crossref PubMed Scopus (101) Google Scholar It has variable expressivity and complete penetrance. It is the most common form of muscular dystrophy. The onset of symptoms generally occurs in the second decade of life and the severity of symptoms worsens as the patient ages. Average life expectancy is 45 to 50 years and death generally occurs from cardiac or respiratory failure. 4 Dalal F.Y. Bennett E.J. Raj P.P. Dystrophia myotonica: A multisystem disease. Can Anaesth Soc J. 1972; 19: 436 Crossref PubMed Scopus (13) Google Scholar" @default.
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- W2138695957 title "Orthognathic Surgery in a Patient With Myotonic Dystrophy: Review of Literature and Report of a Case" @default.
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