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Ultrasound Med Biol. 2017 Nov;43(11):2537-2545. doi: 10.1016/j.ultrasmedbio.2017.06.016. Epub 2017 Jul 29.

Ultrasound Imaging of Muscle Contraction of the Tibialis Anterior in Patients with Facioscapulohumeral Dystrophy.

Author information

1
Orthopaedic Research Laboratory, Department of Orthopaedics, Radboud university medical center, Nijmegen, The Netherlands. Electronic address: Kaj.Gijsbertse@radboudumc.nl.
2
Department of Neurology, Donders Centre for Neuroscience, Radboud university medical center, Nijmegen, The Netherlands.
3
Medical Ultrasound Imaging Center (MUSIC), Department of Radiology and Nuclear Medicine, Radboud university medical center, Nijmegen, The Netherlands.
4
Orthopaedic Research Laboratory, Department of Orthopaedics, Radboud university medical center, Nijmegen, The Netherlands; Laboratory of Biomechanical Engineering, University of Twente, Enschede, The Netherlands.
5
Medical Ultrasound Imaging Center (MUSIC), Department of Radiology and Nuclear Medicine, Radboud university medical center, Nijmegen, The Netherlands; Physics of Fluids Group, MESA+ Institute for Nanotechnology and MIRA Institute for Biomedical Technology and Technical Medicine, University of Twente, Enschede, The Netherlands.

Abstract

A need exists for biomarkers to diagnose, quantify and longitudinally follow facioscapulohumeral muscular dystrophy (FSHD) and many other neuromuscular disorders. Furthermore, the pathophysiological mechanisms leading to muscle weakness in most neuromuscular disorders are not completely understood. Dynamic ultrasound imaging (B-mode image sequences) in combination with speckle tracking is an easy, applicable and patient-friendly imaging tool to visualize and quantify muscle deformation. This dynamic information provides insight in the pathophysiological mechanisms and may help to distinguish the various stages of diseased muscle in FSHD. In this proof-of-principle study, we applied a speckle tracking technique to 2-D ultrasound image sequences to quantify the deformation of the tibialis anterior muscle in patients with FSHD and in healthy controls. The resulting deformation patterns were compared with muscle ultrasound echo intensity analysis (a measure of fat infiltration and dystrophy) and clinical outcome measures. Of the four FSHD patients, two patients had severe peroneal weakness and two patients had mild peroneal weakness on clinical examination. We found a markedly varied muscle deformation pattern between these groups: patients with severe peroneal weakness showed a different motion pattern of the tibialis anterior, with overall less displacement of the central tendon region, while healthy patients showed a non-uniform displacement pattern, with the central aponeurosis showing the largest displacement. Hence, dynamic muscle ultrasound of the tibialis anterior muscle in patients with FSHD revealed a distinctively different tissue deformation pattern among persons with and without tibialis anterior weakness. These findings could clarify the understanding of the pathophysiology of muscle weakness in FSHD patients. In addition, the change in muscle deformation shows good correlation with clinical measures and quantitative muscle ultrasound measurements. In conclusion, dynamic ultrasound in combination with speckle tracking allows the study of the effects of muscle pathology in relation to strength, force transmission and movement generation. Although further research is required, this technique can develop into a biomarker to quantify muscle disease severity.

KEYWORDS:

Deformation imaging; Facioscapulohumeral muscular dystrophy; Skeletal muscle; Speckle tracking; Ultrasound

[Indexed for MEDLINE]

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