Intramuscular blood flow in Duchenne and Becker Muscular Dystrophy: Quantitative power Doppler sonography relates to disease severity

Alexander R Dietz, Anne Connolly, Amir Dori, Craig M Zaidman, Alexander R Dietz, Anne Connolly, Amir Dori, Craig M Zaidman

Abstract

Objective: Absent or truncated dystrophin in Duchenne (DMD) and Becker (BMD) muscular dystrophies results in impaired vasodilatory pathways and exercise induced muscle ischemia. Here, we used power Doppler sonography to quantify changes in intramuscular blood flow immediately following exercise in boys with D/BMD.

Method: We quantified changes in intramuscular blood flow following exercise using power Doppler sonography in 14 boys with D/BMD and compared changes in muscle blood flow to disease severity and to historic controls.

Result: Post exercise blood flow change in the anterior forearm muscles is lower in (1) DMD (median 0.25%; range -0.47 to 2.19%) than BMD (2.46%; 2.02-3.38%, p < 0.05) and historical controls (6.59%; 2.16-12.40%, p < 0.01); (2) in non-ambulatory (0.04%; -0.47 to 0.10%) than ambulatory DMD boys (0.71%; 0.07-2.19%, p < 0.05); and (3) in muscle with higher echointensity (rs = -0.7253, p = 0.005). The tibialis anterior showed similar findings. We estimate that a single sample clinical trial would require 19 subjects to detect a doubling of blood flow to the anterior forearm after the intervention.

Conclusion: Post-exercise blood flow is reduced in D/BMD and relates to disease severity.

Significance: Our protocol for quantifying post-exercise intramuscular blood flow is feasible for clinical trials in D/BMD.

Keywords: Blood flow; Doppler; Dystrophy; Exercise; Muscle; Ultrasound.

Conflict of interest statement

Conflict of interest

None.

Copyright © 2019. Published by Elsevier B.V.

Figures

Figure 1:. Post-Exercise blood flow in Dystrophinopathy…
Figure 1:. Post-Exercise blood flow in Dystrophinopathy and History Healthy Controls
Post-exercise blood flow is severely reduced in DMD compared to the less severely affected BMD and compared to healthy controls, including three children ages 6, 8, and 10 years (open triangles) in both the anterior forearm and tibialis anterior.
Figure 2:. Post exercise blood flow in…
Figure 2:. Post exercise blood flow in the Tibialis Anterior
Power Doppler images of the tibialis anterior show reduced blood flow after exercise in a 9 and 10 year old boy with DMD compared to a healthy 10 year old boy.
Figure 3.. Intramuscular blood flow compared to…
Figure 3.. Intramuscular blood flow compared to gray scale level
Post-exercise blood flow is more reduced in dystrophic anterior forearm muscles with worse (higher) grayscale echointensity.

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