title: Electrical impedance myography in individuals with collagen 6 and laminin α-2 congenital muscular dystrophy: a cross-sectional and 2-year analysis.
abstract: BACKGROUND
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Mark O. Hatfield Clinical Research Center, National Institutes of Health, Bethesda, Maryland, USA.|University of Chicago School of Medicine, Chicago, Illinois, USA.
authors.forename: Carmel
authors.initials: C
authors.lastname: Nichols
authors.affiliation: Mark O. Hatfield Clinical Research Center, National Institutes of Health, Bethesda, Maryland, USA.
authors.forename: Minal S
authors.initials: MS
authors.lastname: Jain
authors.affiliation: Tissue Injury Branch, National Institute of Nursing Research, National Institutes of Health, Bethesda, Maryland, USA.
authors.forename: Katherine G
authors.initials: KG
authors.lastname: Meilleur
authors.affiliation: Clinical Trials Unit, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland, USA.
authors.forename: Tianxia
authors.initials: T
authors.lastname: Wu
authors.affiliation: Mercy Clinic Pediatric Neurology, Springfield, Missouri, USA.
authors.forename: James
authors.initials: J
authors.lastname: Collins
authors.affiliation: Mark O. Hatfield Clinical Research Center, National Institutes of Health, Bethesda, Maryland, USA.
authors.forename: Melissa R
authors.initials: MR
authors.lastname: Waite
authors.affiliation: Columbia University, New York, New York, USA.
authors.forename: Jahannaz
authors.initials: J
authors.lastname: Dastgir
authors.affiliation: EMG Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Building 10/Room 7-5680, Bethesda, Maryland, 20892-1404, USA.|Mercy Hospital, Baltimore, Maryland, USA.
authors.forename: Anam
authors.initials: A
authors.lastname: Salman
authors.affiliation: Neuromuscular and Neurogenetic Disorders of Childhood Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland, USA.
authors.forename: Sandra
authors.initials: S
authors.lastname: Donkervoort
authors.affiliation: Stanford Children's Health, Palo Alto, California, USA.
authors.forename: Tina
authors.initials: T
authors.lastname: Duong
authors.affiliation: Rady Children's Hospital, San Diego, California, USA.
authors.forename: Katherine
authors.initials: K
authors.lastname: Keller
authors.affiliation: Neuromuscular and Neurogenetic Disorders of Childhood Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland, USA.
authors.forename: Meganne E
authors.initials: ME
authors.lastname: Leach
authors.affiliation: University of Florida, Gainesville, Florida, USA.
authors.forename: Donovan J
authors.initials: DJ
authors.lastname: Lott
authors.affiliation: Cincinnati Children's Hospital, Cincinnati, Ohio, USA.
authors.forename: Michelle N
authors.initials: MN
authors.lastname: McGuire
authors.affiliation: University of Texas Southwestern Medical Center, Dallas Texas, USA.
authors.forename: Leslie
authors.initials: L
authors.lastname: Nelson
authors.affiliation: Kaiser Permanente SCPMG Cure CMD, Los Angeles, California, USA.
authors.forename: Anne
authors.initials: A
authors.lastname: Rutkowski
authors.affiliation: Hospices Civils de Lyon, Hôpital femme Mère enfant, Bron, France.|Université de Lyon, Lyon, France.
authors.forename: Carole
authors.initials: C
authors.lastname: Vuillerot
authors.affiliation: Neuromuscular and Neurogenetic Disorders of Childhood Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland, USA.
authors.forename: Carsten G
authors.initials: CG
authors.lastname: Bönnemann
authors.affiliation: EMG Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Building 10/Room 7-5680, Bethesda, Maryland, 20892-1404, USA.
authors.forename: Tanya J
authors.initials: TJ
authors.lastname: Lehky
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D024142:Collagen Type VI / Q000235:genetics*; D003430:Cross-Sectional Studies; D017097:Electric Impedance; D005260:Female; D006801:Humans; D007797:Laminin / Q000235:genetics*; D008297:Male; D053580:Muscle Strength; D009136:Muscular Dystrophies / Q000151:congenital* / Q000235:genetics*; D009213:Myography / Q000379:methods*; D009460:Neurologic Examination / Q000379:methods; D012420:Running; D012680:Sensitivity and Specificity; D012720:Severity of Illness Index
chemical_list: D024142:Collagen Type VI; D007797:Laminin; C102645:laminin alpha 2
keywords: COL6 related dystrophies; LAMA2 related dystrophies; congenital muscular dystrophy; electrical impedance myography; neuromuscular disorders