[Effects of corticosteroids in the management of Duchenne muscular dystrophy: our experience].
Objective To evaluate the clinical course in patients with Duchenne muscular dystrophy admitted to our department who received corticosteroid treatment and to compare their course with that in patients who did not receive corticosteroid treatment.Patients and methodsWe performed a retrospective study of 20 pediatric patients with a diagnosis of Duchenne muscular dystrophy who were offered corticosteroid treatment: 10 patients received deflazacort and 10 refused the treatment. The MRC muscular strength scale and Vignos' functional scale were used to evaluate clinical course, which was compared in both groups.ResultsUntreated patients showed progressive worsening. Corticosteroid-treated patients showed disease stabilization both in muscular strength and functional performance. In addition, muscular balance improved in 70 % of these patients, but only 2 % showed functional improvement. The positive effect of steroid treatment had a mean duration of 12 months. Loss of independent gait occurred at similar ages in both groups (10.3 vs. 10.5 years). The results of Achilles' tendon surgery were poor.ConclusionsCorticosteroids produced clinical stabilization and improved muscular strength. Functional improvement was not significant, including loss of gait, probably because this loss also depends on an increase in joint contracture. Good coordination among multiprofessional teams is essential to achieve optimal results.
Rafia
S
S
Servicio de Neurología Pediátrica. Hospital Universitario La Paz. Madrid. España.
Pascual-Pascual
S I
SI
Martínez-Granero
M A
MA
Pascual-Castroviejo
I
I
D000305:Adrenal Cortex Hormones; D019468:Disease Management; D006801:Humans; D009132:Muscles; D020388:Muscular Dystrophy, Duchenne / Q000175:diagnosis; D012189:Retrospective Studies
D000305:Adrenal Cortex Hormones