Electrical impedance myography in individuals with collagen 6 and laminin α-2 congenital muscular dystrophy: a cross-sectional and 2-year analysis

Muscle Nerve. 2018 Jan;57(1):54-60. doi: 10.1002/mus.25629. Epub 2017 Apr 25.

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.

Keywords: COL6 related dystrophies; LAMA2 related dystrophies; congenital muscular dystrophy; electrical impedance myography; neuromuscular disorders.

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Cohort Studies
  • Collagen Type VI / genetics*
  • Cross-Sectional Studies
  • Electric Impedance*
  • Female
  • Humans
  • Laminin / genetics*
  • Male
  • Muscle Strength
  • Muscular Dystrophies / congenital*
  • Muscular Dystrophies / genetics*
  • Myography / methods*
  • Neurologic Examination / methods
  • Running
  • Sensitivity and Specificity
  • Severity of Illness Index

Substances

  • Collagen Type VI
  • Laminin
  • laminin alpha 2