Seward B Rutkove1, Jim S Wu2, Craig Zaidman3, Kush Kapur4, Sung Yim5, Amy Pasternak4, Lavanya Madabusi4, Heather Szelag4, Tim Harrington4, Jia Li5, Adam Pacheck5, Basil T Darras4. 1. Department of Neurology, Beth Israel Deaconess Medical Center, Harvard Medical School, 330 Brookline Ave, Boston, MA 02215, USA. Electronic address: srutkove@bidmc.harvard.edu. 2. Department of Radiology, Beth Israel Deaconess Medical Center, Harvard Medical School, 330 Brookline Ave, Boston, MA 02215, USA. 3. Department of Neurology, Washington University in St. Louis, 660 S Euclid Ave, St. Louis, MO 63110, USA. 4. Department of Neurology, Boston Children's Hospital, Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115, USA. 5. Department of Neurology, Beth Israel Deaconess Medical Center, Harvard Medical School, 330 Brookline Ave, Boston, MA 02215, USA.
Abstract
OBJECTIVE: We sought to understand the alteration in the anisotropic, or direction dependent, character of muscle as measured by electrical impedance myography (EIM) in subjects with Duchenne muscular dystrophy (DMD) and its potential to serve as a biomarker of disease status. METHODS: Thirty-six boys with DMD and 27 healthy controls were measured with EIM, with electrical current applied both parallel and perpendicular to the major muscle fiber direction. In addition, muscle extracted from 10 mdx and 10 wild-type mice were measured analogously. RESULTS: Normalized reactance anisotropy, a direction-dependent measure of membrane charge storage capability, was significantly lower in the four muscles of DMD subjects as compared to controls (p<0.01). Normalized reactance anisotropy also decreased with increasing age in DMD subjects (r=-0.36, p=0.031), but not in healthy boys. Analogous changes were observed in mdx mouse gastrocnemius as compared to wild type (p=0.019). CONCLUSION: These results support that loss of electrical anisotropy is a previously unrecognized feature of dystrophic muscle. SIGNIFICANCE: Anisotropic alterations may offer novel indices to assist in neuromuscular disease diagnosis and to serve as easy-to-obtain biomarkers in clinical therapeutic trials.
OBJECTIVE: We sought to understand the alteration in the anisotropic, or direction dependent, character of muscle as measured by electrical impedance myography (EIM) in subjects with Duchenne muscular dystrophy (DMD) and its potential to serve as a biomarker of disease status. METHODS: Thirty-six boys with DMD and 27 healthy controls were measured with EIM, with electrical current applied both parallel and perpendicular to the major muscle fiber direction. In addition, muscle extracted from 10 mdx and 10 wild-type mice were measured analogously. RESULTS: Normalized reactance anisotropy, a direction-dependent measure of membrane charge storage capability, was significantly lower in the four muscles of DMD subjects as compared to controls (p<0.01). Normalized reactance anisotropy also decreased with increasing age in DMD subjects (r=-0.36, p=0.031), but not in healthy boys. Analogous changes were observed in mdx mouse gastrocnemius as compared to wild type (p=0.019). CONCLUSION: These results support that loss of electrical anisotropy is a previously unrecognized feature of dystrophic muscle. SIGNIFICANCE: Anisotropic alterations may offer novel indices to assist in neuromuscular disease diagnosis and to serve as easy-to-obtain biomarkers in clinical therapeutic trials.
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Authors: Evellyn C Grilo; Thais A Cunha; Ádila Danielly S Costa; Bárbara G M Araújo; Márcia Marília G D Lopes; Bruna L L Maciel; Camila X Alves; Karina M Vermeulen-Serpa; Mário Emílio T Dourado-Júnior; Lucia Leite-Lais; José Brandão-Neto; Sancha Helena L Vale Journal: PLoS One Date: 2020-11-20 Impact factor: 3.240