Positive effect of the combination of multilevel contracture release and glucocorticoid treatment in Duchenne muscular dystrophy

Abstract Purpose In the 1980s the first results of an early multilevel contracture release (MLCR) in patients suffering from progressive Duchenne muscular dystrophy (DMD) showed a positive effect on ambulation. Despite the demonstrated positive effects of prolongation of walking this treatment is not part of current guidelines. The aim of our study was to evaluate the effect of MLCR as well as its combination with glucocorticoid (GC) treatment on ambulation. Methods Data of all boys (n = 86) with DMD treated in our outpatient department were analyzed regarding the treatment and loss of independent ambulation. In all, 23 were treated with GC only, ten were operated on, 21 received GC and underwent MLCR and 32 received neither of the two treatments. Results The analysis of the loss of independent ambulation in our cohort showed a comparable extension of the ambulatory period between the GC-treated and MLCR-treated boys (p = 0.008 and p = 0.005, respectively). Furthermore, an additive effect of both therapies was found; patients with DMD who had both treatments were able to walk two years longer than those with only one of the two treatment options (p<0.001). Conclusion Standard GC treatment and early MLCR in lower limbs have an independent positive effect on prolongation of ambulation in patients with DMD. In our cohort, the combination of both therapies is significantly more effective than each therapy alone. We suggest both should be offered to all DMD patients eligible. Level of evidence: III

[1]  B. Alman,et al.  Orthopedic and Surgical Management of the Patient With Duchenne Muscular Dystrophy , 2018, Pediatrics.

[2]  S. Pandya,et al.  Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and neuromuscular, rehabilitation, endocrine, and gastrointestinal and nutritional management , 2018, The Lancet Neurology.

[3]  K. Bushby,et al.  Clinical Outcomes in Duchenne Muscular Dystrophy: A Study of 5345 Patients from the TREAT-NMD DMD Global Database , 2017, Journal of neuromuscular diseases.

[4]  A. Manzur,et al.  Corticosteroids for the treatment of Duchenne muscular dystrophy. , 2016, The Cochrane database of systematic reviews.

[5]  J. Forst,et al.  Surgical treatment of Duchenne muscular dystrophy patients in Germany: the present situation , 2012, Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology.

[6]  C. Richelme,et al.  Lower extremity surgery in muscular dystrophy. , 2011, Orthopaedics & traumatology, surgery & research : OTSR.

[7]  S. Weiss,et al.  Darstellung eines Konzeptes zur Behandlung der Duchenne Muskeldystrophie (DMD) mit dem Ziel der Verbesserung und Wiedererlangung der Geh- und Stehfähigkeit , 2008 .

[8]  U. Werlauff,et al.  A randomized comparative study of two methods for controlling Tendo Achilles contracture in Duchenne muscular dystrophy , 2000, Neuromuscular Disorders.

[9]  J. Forst,et al.  Lower limb surgery in Duchenne muscular dystrophy , 1999, Neuromuscular Disorders.

[10]  T. Voit,et al.  Clinical results of early orthopaedic management in Duchenne muscular dystrophy. , 1995, Neuropediatrics.

[11]  L. Merlini,et al.  Early surgery in Duchenne muscular dystrophy. Experience at Istituto Ortopedico Rizzoli, Bologna, Italy , 1994, Neuromuscular Disorders.

[12]  W. Puhl,et al.  [Presentation of a treatment concept of Duchenne muscular dystrophy with the aim at improvement and restoration of walking and standing ability]. , 1994, Zeitschrift fur Orthopadie und ihre Grenzgebiete.

[13]  G. Fenichel,et al.  Prolongation of Ambulation in Children with Duchenne Muscular Dystrophy by Subcutaneous Lower Limb Tenotomy , 1993, Journal of pediatric orthopedics.

[14]  G. Bentley,et al.  A randomized controlled trial of early surgery in duchenne muscular dystrophy , 1992, Neuromuscular Disorders.

[15]  J. McKeon,et al.  ORTHOPEDIC SURGERY AND REHABILITATION FOR THE PROLONGATION OF BRACE‐FREE AMBULATION OF PATIENTS WITH DUCHENNE MUSCULAR DYSTROPHY , 1991, American journal of physical medicine & rehabilitation.

[16]  Y. Rideau,et al.  Prolongation of ambulation in the muscular dystrophies. , 1983, Acta neurologica.