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BackgroundDuchenne muscular dystrophy (DMD) is a genetic neuromuscular disorder that leads to mobility loss and life‐threatening cardiac or respiratory complications. Quantitative ultrasound (QUS) envelope statistics imaging, which characterizes fat infiltration and fibrosis in muscles, has been extensively used for DMD evaluations.PurposeNotably, changes in muscle microstructures also result in acoustic attenuation, potentially serving as another crucial imaging biomarker for DMD. Expanding upon the reference frequency method (RFM), this study contributes to the field by introducing the robust RFM (RRFM) as a novel approach for ultrasound attenuation imaging in DMD.MethodsThe RRFM algorithm was developed using an iterative reweighted least squares technique. We conducted standard phantom measurements with a clinical ultrasound system equipped with a linear array transducer to assess the improvement in attenuation estimation bias by RRFM. Additionally, 161 DMD patients, included in both a validation dataset (n = 130) and a testing dataset (n = 31), underwent ultrasound scanning of the gastrocnemius for RRFM‐based attenuation imaging. The diagnostic performances for ambulatory functions and discrimination between early and late ambulatory stages were evaluated and compared with those of QUS envelope statistics imaging (involving Nakagami distribution, homodyned K distribution, and entropy values) using the area under the receiver operating characteristic curve (AUROC).ResultsThe results indicated that the RRFM method more closely matched the actual attenuation properties of the phantom, reducing measurement bias by 50% compared to conventional RFM. The AUROCs for RRFM‐based attenuation imaging, used to discriminate between early and late ambulatory stages, were 0.88 and 0.92 for the validation and testing datasets, respectively. These performances significantly surpassed those of QUS envelope statistics imaging (p < 0.05).ConclusionsUltrasound attenuation imaging employing RRFM may serve as a sensitive tool for evaluating the progression of ambulatory function deterioration, offering substantial potential for the health management and follow‐up care of DMD patients.
BackgroundDuchenne muscular dystrophy (DMD) is a genetic neuromuscular disorder that leads to mobility loss and life‐threatening cardiac or respiratory complications. Quantitative ultrasound (QUS) envelope statistics imaging, which characterizes fat infiltration and fibrosis in muscles, has been extensively used for DMD evaluations.PurposeNotably, changes in muscle microstructures also result in acoustic attenuation, potentially serving as another crucial imaging biomarker for DMD. Expanding upon the reference frequency method (RFM), this study contributes to the field by introducing the robust RFM (RRFM) as a novel approach for ultrasound attenuation imaging in DMD.MethodsThe RRFM algorithm was developed using an iterative reweighted least squares technique. We conducted standard phantom measurements with a clinical ultrasound system equipped with a linear array transducer to assess the improvement in attenuation estimation bias by RRFM. Additionally, 161 DMD patients, included in both a validation dataset (n = 130) and a testing dataset (n = 31), underwent ultrasound scanning of the gastrocnemius for RRFM‐based attenuation imaging. The diagnostic performances for ambulatory functions and discrimination between early and late ambulatory stages were evaluated and compared with those of QUS envelope statistics imaging (involving Nakagami distribution, homodyned K distribution, and entropy values) using the area under the receiver operating characteristic curve (AUROC).ResultsThe results indicated that the RRFM method more closely matched the actual attenuation properties of the phantom, reducing measurement bias by 50% compared to conventional RFM. The AUROCs for RRFM‐based attenuation imaging, used to discriminate between early and late ambulatory stages, were 0.88 and 0.92 for the validation and testing datasets, respectively. These performances significantly surpassed those of QUS envelope statistics imaging (p < 0.05).ConclusionsUltrasound attenuation imaging employing RRFM may serve as a sensitive tool for evaluating the progression of ambulatory function deterioration, offering substantial potential for the health management and follow‐up care of DMD patients.
Purpose of review This review highlights recent developments in the field of muscle ultrasound (MUS) for the diagnosis and follow up of muscle disorders. Recent findings The diagnostic screening capacity of quantitative grayscale analysis is still sufficient to assess children suspected of a neuromuscular disorder. A combination of visual and quantitative assessment is advised for optimal interpretation. MUS was more sensitive but less specific than MRI for detecting pathology in limb girdle dystrophies and inflammatory myopathies. New techniques such as shearwave elastography and artificial intelligence algorithms for automated image segmentation show promise but need further development for use in everyday practice. Muscle ultrasound has high correlations with clinical measures of function in skeletal and respiratory muscles and the orofacial region, in most of the myopathies and dystrophies studied. Over time, imaging changes precede changes in clinical status, making them attractive for biomarker use in trials. In Duchenne muscular dystrophy MUS was also responsive to the effects of steroid treatment. Summary Muscle ultrasound is a sensitive technique to diagnose and follow up of skeletal, facial and respiratory muscles in neuromuscular disorders. Its role is both complementary to and partially overlapping with that of MRI.
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