BACKGROUND: Myopathies present with a broad diagnostic spectrum which may ultimately require muscle biopsy. MRI has been established as a non-invasive method in diagnosing adult myopathies; not only does MRI reveal characteristic findings which point in a diagnostic direction, but also aids in determining optimal biopsy sites and controlling therapeutic interventions. Muscle MRI is increasingly finding application to pediatric myopathies, especially dystrophies and myositides. The following paper serves to illustrate the use of MRI using exemplary clinical vignettes. PATIENTS/ METHODS: From 1999 until 2006, 180 children with myopathies of unknown aetiology, ages 10 months to 18 years, were examined with a standardised MRI protocol (axial T1-SE and T2-weighted TIRM sequences). The protocol included imaging of the lower extremities whereas sequences displaying the upper extremities were only acquired in selected patients. Furthermore, intravenous contrast agent was only administered in selected children. RESULTS: All investigations could be performed without sedation due to an examination time of 12 to 15 minutes. The illustrated cases of limb-girdle muscular dystrophy, Duchenne's muscular dystrophy, dermatomyositis, pyomyositis, and chronic neurogenic disease with secondary myopathy all showed disease-characteristic MRI patterns which substantially helped to reach the ultimate diagnosis. CONCLUSIONS: Muscle MRI is a non-invasive and effective instrument in helping to diagnose pediatric myopathies of unknown aetiology. It may facilitate muscle biopsy and serves to control therapeutical effects and disease course. Furthermore, muscle MRI may be applicated even to children of less than 4 years of age without sedation.
BACKGROUND:Myopathies present with a broad diagnostic spectrum which may ultimately require muscle biopsy. MRI has been established as a non-invasive method in diagnosing adult myopathies; not only does MRI reveal characteristic findings which point in a diagnostic direction, but also aids in determining optimal biopsy sites and controlling therapeutic interventions. Muscle MRI is increasingly finding application to pediatric myopathies, especially dystrophies and myositides. The following paper serves to illustrate the use of MRI using exemplary clinical vignettes. PATIENTS/ METHODS: From 1999 until 2006, 180 children with myopathies of unknown aetiology, ages 10 months to 18 years, were examined with a standardised MRI protocol (axial T1-SE and T2-weighted TIRM sequences). The protocol included imaging of the lower extremities whereas sequences displaying the upper extremities were only acquired in selected patients. Furthermore, intravenous contrast agent was only administered in selected children. RESULTS: All investigations could be performed without sedation due to an examination time of 12 to 15 minutes. The illustrated cases of limb-girdle muscular dystrophy, Duchenne's muscular dystrophy, dermatomyositis, pyomyositis, and chronic neurogenic disease with secondary myopathy all showed disease-characteristic MRI patterns which substantially helped to reach the ultimate diagnosis. CONCLUSIONS: Muscle MRI is a non-invasive and effective instrument in helping to diagnose pediatric myopathies of unknown aetiology. It may facilitate muscle biopsy and serves to control therapeutical effects and disease course. Furthermore, muscle MRI may be applicated even to children of less than 4 years of age without sedation.
Authors: Claudia P Cejas; Maria M Serra; David F Gonzalez Galvez; Eliana A Cavassa; Ana L Taratuto; Gabriel A Vazquez; Mario E L Massaro; Angeles V Schteinschneider Journal: Pediatr Radiol Date: 2017-01-19