title: Practice guideline update summary: Corticosteroid treatment of Duchenne muscular dystrophy [RETIRED]: Report of the Guideline Development Subcommittee of the American Academy of Neurology.
abstract: To update the 2005 American Academy of Neurology (AAN) guideline on corticosteroid treatment of Duchenne muscular dystrophy (DMD).
  
  
  
  We systematically reviewed the literature from January 2004 to July 2014 using the AAN classification scheme for therapeutic articles and predicated recommendations on the strength of the evidence.
  
  
  
  Thirty-four studies met inclusion criteria.
  
  
  
  In children with DMD, prednisone should be offered for improving strength (Level B) and pulmonary function (Level B). Prednisone may be offered for improving timed motor function (Level C), reducing the need for scoliosis surgery (Level C), and delaying cardiomyopathy onset by 18 years of age (Level C). Deflazacort may be offered for improving strength and timed motor function and delaying age at loss of ambulation by 1.4-2.5 years (Level C). Deflazacort may be offered for improving pulmonary function, reducing the need for scoliosis surgery, delaying cardiomyopathy onset, and increasing survival at 5-15 years of follow-up (Level C for each). Deflazacort and prednisone may be equivalent in improving motor function (Level C). Prednisone may be associated with greater weight gain in the first years of treatment than deflazacort (Level C). Deflazacort may be associated with a greater risk of cataracts than prednisone (Level C). The preferred dosing regimen of prednisone is 0.75 mg/kg/d (Level B). Over 12 months, prednisone 10 mg/kg/weekend is equally effective (Level B), with no long-term data available. Prednisone 0.75 mg/kg/d is associated with significant risk of weight gain, hirsutism, and cushingoid appearance (Level B).
authors.affiliation: From the Department of Neurology (D.G.), Geisinger Health System, Danville, PA; Department of Neurology (R.T.M.), University of Rochester School of Medicine and Dentistry, NY; Department of Neurology (S.A.), Loma Linda University Medical Center, CA; and Departments of Pediatric and Neurology/Neurosurgery (M.O.), McGill University, Montréal, Canada.
authors.forename: David
authors.initials: D
authors.lastname: Gloss
authors.affiliation: From the Department of Neurology (D.G.), Geisinger Health System, Danville, PA; Department of Neurology (R.T.M.), University of Rochester School of Medicine and Dentistry, NY; Department of Neurology (S.A.), Loma Linda University Medical Center, CA; and Departments of Pediatric and Neurology/Neurosurgery (M.O.), McGill University, Montréal, Canada.
authors.forename: Richard T
authors.initials: RT
authors.lastname: Moxley
authors.affiliation: From the Department of Neurology (D.G.), Geisinger Health System, Danville, PA; Department of Neurology (R.T.M.), University of Rochester School of Medicine and Dentistry, NY; Department of Neurology (S.A.), Loma Linda University Medical Center, CA; and Departments of Pediatric and Neurology/Neurosurgery (M.O.), McGill University, Montréal, Canada.
authors.forename: Stephen
authors.initials: S
authors.lastname: Ashwal
authors.affiliation: From the Department of Neurology (D.G.), Geisinger Health System, Danville, PA; Department of Neurology (R.T.M.), University of Rochester School of Medicine and Dentistry, NY; Department of Neurology (S.A.), Loma Linda University Medical Center, CA; and Departments of Pediatric and Neurology/Neurosurgery (M.O.), McGill University, Montréal, Canada.
authors.forename: Maryam
authors.initials: M
authors.lastname: Oskoui
mesh_terms: D000047:Academies and Institutes / Q000592:standards*; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D006801:Humans; D020388:Muscular Dystrophy, Duchenne / Q000175:diagnosis / Q000188:drug therapy* / Q000453:epidemiology; D009462:Neurology / Q000379:methods / Q000592:standards*; D017410:Practice Guidelines as Topic / Q000592:standards*; D011282:Pregnenediones / Q000627:therapeutic use; D058028:Research Report / Q000592:standards*; D016896:Treatment Outcome; D014481:United States / Q000453:epidemiology
chemical_list: D000305:Adrenal Cortex Hormones; D011282:Pregnenediones; C021988:deflazacort
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