Ultrasound in the Assessment of Myopathic Disorders

Neuromuscular ultrasound (US) augments a careful physical examination and electrodiagnostic evaluation in the evaluation of suspected myopathy. Ultrasound evaluation of muscle can identify abnormal echo intensity, size, and movement. Because it is painless and noninvasive, US can be used to evaluate multiple muscles to direct the electrodiagnostic examination or muscle biopsy. Some patterns of muscle involvement can suggest specific etiologies. Most muscular dystrophies show homogenously increased muscle echo intensity with attenuation of the US signal, likely resultant from increased intramuscular fat and fibrosis. Inflammatory myopathies can also show homogenously increased echogenicity but lack the signal attenuation seen in muscular dystrophies. In contrast, denervation can show "moth-eaten," atrophic muscles with fasciculations. Advanced age and obesity also impacts muscle size and echo intensity and can hamper efforts to detect mild pathologies. The sensitivity and specificity of US for detecting neuromuscular disease have been best studied in children and depend on the type and severity of the disorder. In general, muscle US yields sensitivities and specificities of 67% to 100% for detecting neuromuscular disorders in children and is similar to electromyogram for detection of myopathy. Ultrasound is most sensitive for detecting muscular dystrophies and is less sensitive in metabolic myopathies and very young children.