Validity of Skeletal Muscle Ultrasound in Assessment of Suspected Limb Girdle Muscular Dystrophy Patients

Abstract

Abstract Background Limb-girdle muscular dystrophies (LGMDs) are a clinically and genetically heterogeneous group of disorders characterized by progressive muscle weakness and degenerative muscle changes. Studies have shown that ultrasound can be useful both for diagnosis and follow-up of LGMDs patients. Objectives This study aims to measure the sensitivity and the specificity of muscle ultrasound in assessment of suspected limb girdle muscular dystrophy patients. Subjects and Methods This cross-sectional descriptive study was conducted on Fifty-five patients with suspected LGMD from neuromuscular unit, myology clinic, Ain Shams University hospitals and eight healthy subjects. Age was above 2 years. Both sexes were included in the study. They underwent real-time B-mode ultrasonography performed with using Logiq p9 General Electric ultrasound machine and General Electric 7-11.5 MHZ linear array ultrasound probe. All ultrasound images have been obtained and scored by a single examiner and muscle echo intensity was visually graded semiquantitative according to Heckmatt's scale. The examiner was blinded to the muscle biopsy results and clinical evaluations. Results Statistical analysis revealed that the diagnostic performance of muscle US (Heckmatt’s score) in LGMD is most sensitive when calculated in all examined upper limb and lower limb muscles, followed by lower limb muscles alone. US of upper limb was found to be the least sensitive. Conclusions Muscle ultrasound is a practical and reproducible and valid tool that can be used in assessment of suspected LGMD patients.