Carmel Nichols1,2, Minal S Jain1, Katherine G Meilleur3, Tianxia Wu4, James Collins5, Melissa R Waite1, Jahannaz Dastgir6, Anam Salman7,8, Sandra Donkervoort9, Tina Duong10, Katherine Keller11, Meganne E Leach9, Donovan J Lott12, Michelle N McGuire13, Leslie Nelson14, Anne Rutkowski15, Carole Vuillerot16,17, Carsten G Bönnemann9, Tanya J Lehky7. 1. Mark O. Hatfield Clinical Research Center, National Institutes of Health, Bethesda, Maryland, USA. 2. University of Chicago School of Medicine, Chicago, Illinois, USA. 3. Tissue Injury Branch, National Institute of Nursing Research, National Institutes of Health, Bethesda, Maryland, USA. 4. Clinical Trials Unit, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland, USA. 5. Mercy Clinic Pediatric Neurology, Springfield, Missouri, USA. 6. Columbia University, New York, New York, USA. 7. EMG Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Building 10/Room 7-5680, Bethesda, Maryland, 20892-1404, USA. 8. Mercy Hospital, Baltimore, Maryland, USA. 9. Neuromuscular and Neurogenetic Disorders of Childhood Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland, USA. 10. Stanford Children's Health, Palo Alto, California, USA. 11. Rady Children's Hospital, San Diego, California, USA. 12. University of Florida, Gainesville, Florida, USA. 13. Cincinnati Children's Hospital, Cincinnati, Ohio, USA. 14. University of Texas Southwestern Medical Center, Dallas Texas, USA. 15. Kaiser Permanente SCPMG Cure CMD, Los Angeles, California, USA. 16. Hospices Civils de Lyon, Hôpital femme Mère enfant, Bron, France. 17. Université de Lyon, Lyon, France.
Abstract
INTRODUCTION: Electrical impedance myography (EIM) is a noninvasive electrophysiological technique that characterizes muscle properties through bioimpedance. We compared EIM measurements to function, strength, and disease severity in a population with congenital muscular dystrophy (CMD). METHODS: Forty-one patients with CMD, either collagen 6 related disorders (COL6-RD; n = 21) or laminin α-2-related disorders (LAMA2-RD; n = 20), and 21 healthy pediatric controls underwent 2 yearly EIM exams. In the CMD cohorts, EIM was compared with functional and strength measurements. RESULTS: Both CMD cohorts exhibited change over time and had correlation with disease severity. The 50-kHZ phase correlated well with function and strength in the COL6-RD cohort but not in the LAMA2-RD cohort. DISCUSSION: EIM is a potentially useful measure in clinical studies with CMD because of its sensitivity to change over a 1-year period and correlation with disease severity. For COL6-RD, there were also functional and strength correlations. Muscle Nerve 57: 54-60, 2018.
INTRODUCTION: Electrical impedance myography (EIM) is a noninvasive electrophysiological technique that characterizes muscle properties through bioimpedance. We compared EIM measurements to function, strength, and disease severity in a population with congenital muscular dystrophy (CMD). METHODS: Forty-one patients with CMD, either collagen 6 related disorders (COL6-RD; n = 21) or laminin α-2-related disorders (LAMA2-RD; n = 20), and 21 healthy pediatric controls underwent 2 yearly EIM exams. In the CMD cohorts, EIM was compared with functional and strength measurements. RESULTS: Both CMD cohorts exhibited change over time and had correlation with disease severity. The 50-kHZ phase correlated well with function and strength in the COL6-RD cohort but not in the LAMA2-RD cohort. DISCUSSION: EIM is a potentially useful measure in clinical studies with CMD because of its sensitivity to change over a 1-year period and correlation with disease severity. For COL6-RD, there were also functional and strength correlations. Muscle Nerve 57: 54-60, 2018.
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