Corticosteroid therapy in Duchenne muscular dystrophy.
In recent years, various clinical trials have documented the benefit of glucocorticoid therapy in the palliation of Duchenne muscular dystrophy (DMD). Prednisone therapy, daily or on alternate days, has been confirmed to be of value in enhancing muscle strength and function in DMD for up to two years. However, there is evidence that corticosteroid treatment results in muscle weakness and degeneration. This review, therefore, examines the available studies and addresses various possible mechanisms involved in the efficacy of prednisone therapy and amelioration of DMD. The progression of DMD is known to be associated with profound changes in structure, biochemistry and physiology of the affected muscles. It is hypothesized, therefore, that these very changes offer a fortunate set of circumstances, and it is owing to these alterations, as well as the well known anti-inflammatory/immunosuppressive action of steroid, that muscles in DMD are rendered responsive resulting in significant improvement of muscle bulk and function.
Khan
M A
MA
Department of Anatomy & Cell Biology, State University of New York, Health Science Center at Brooklyn 11203.
D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000494:pharmacology / Q000627:therapeutic use*; D000818:Animals; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D009132:Muscles / Q000378:metabolism / Q000503:physiopathology; D009136:Muscular Dystrophies / Q000188:drug therapy* / Q000378:metabolism / Q000503:physiopathology; D009420:Nervous System / Q000187:drug effects; D009424:Nervous System Physiological Phenomena
D000305:Adrenal Cortex Hormones