Ultrasound Findings in Duchenne Muscular Dystrophy Disease

ABSTRACT

The real-time ultrasonography is a simple, noninvasive procedure that is most suitable for application in pediatric practice. The ultrasonographic appearance of various disorders in children such as progressive muscular dystrophies, infantile spinal muscular atrophy, congenital myopathies, and motor neuropathies has been found to be strikingly abnormal. We have done a pilot study using real-time ultrasonography in children with Duchenne muscular dystrophy in an attempt to correlate their clinicopathologic profiles with scan findings. Echogenicity and delineation of fascia at midthigh and midcalf muscle were measured using a real-time linear array ultrasound scanner in 12 Duchenne mucular dystrophy patients attending our Muscle Clinic, as a double-blind pilot study matched against 10 controls. The ultrasonic scan findings in normal children revealed no echogenicity of muscle, distinct echogenicity of bone and delineation of fascia. But all Duchenne muscular dystrophy patients had increased echogenicity of muscle and decreased echogenicity of bone, and some patients had interruption of delineation of fascia. Duchenne muscular dystrophy patients who were unable to raise from standard height chair showed higher grade of echogenicity at midthigh level than the patients who were able to raise from standard height chair. But this result was not applicable at midcalf level. We concluded that the real-time ultrasonography was useful diagnosis method in Duchenne muscular dystrophy. In addition, when the real-time B ultrasonography was applied to midthigh level, the ultrasonic scan findings could reflect indirectly the functional ability of Duchenne muscular dystrophy patients.