DUCHENNE MUSCULAR DYSTROPHY AND STEROID THERAPY: EVALUATION OF PATIENTS' MOTOR PERFORMANCE

Objectives: To define a simple and quick physiotherapic assessment of muscle strength and motor ability in boys with Duchenne muscular dystrophy (DMD) treated with steroids.

Methods: Thirty-two boys were evaluated along a period of 14 months, according to physiotherapic tests based on Scott et al, 1982 (MRC scale, Hammersmith motor ability score, time for rising from the floor and for walking 9 meters) and on lifting weight tests. Results: Eighteen boys maintained independent walking (Group 1), 8 lost it, and 6 abandoned the study due to surgical procedure (2), irregular treatment (2) or lack of follow-up (2). In Group 1, the final MRC score (for only 18 muscles of lower limbs and trunk) was above 70%, against a value below 70% observed for the natural history of DMD. Regarding the tests Hammersmith score, lifting weight and time for walking 9 meters we found a statistically significant greater number of patients showing improvement. At the end of the follow-up our patients demonstrated a trend for muscle strength and functional score decrease from 80 to 70% and from 31 to 25, respectively, against 72 to 51% and 31 to 17, respectively, in Scott's study. Along the first year of treatment, 4 evaluations are enough for obtaining an objective assessment.

Conclusion: A practical and quick evaluation includes MRC scale for lower limbs and trunk muscles, Hammersmith motor ability score, time for walking 9 meters and test of weight lifting; in boys treated with steroids the loss of muscle strength and functional ability is slower then that observed in the natural history.