Use Of The Motor Function Measure Scale To Analyze Effects Of Steroid Therapy On Patients With Duchenne Muscular Dystropy

Duchenne Muscular Dystrophy (DMD) is a severe recessive X-linked form of muscular dystrophy characterized by rapid progression of muscle degeneration leading to loss of ambulation and pulmonary function impairment. Motor Function Measure (MFM) is a new measurement tool for assessing motor function in different neuromuscular disorders. It includes 32 items that evaluate three dimensions of motor performance: D1, standing posture and transfers; D2, axial and proximal motor capacity; and D3, distal motor capacity. The items are scored and summed to comprise a total score in which the maximum represents normal motor function. The objective of this study was to assess the evolution of motor function in patients with DMD treated with steroids (prednisolone or deflazacort) through the MFM. Thirty-three patients with DMD (22 ambulant, six non-ambulant and five who lost the capacity to walk during the period of the study) were assessed using the MFM scale on six times over a period of 18 months. Motor functions remained stable during a period of 14 months and in ambulant patients D2 improved during a period of 6 months; an improvement in D3 was noted during the total follow-up. D1 and total score were useful to predict the loss of the ability to walk. The use of the MFM in DMD patients confirms the benefits of the steroid treatment for slowing the progression of the disease (Supported by CAPES).