Electrical impedance myography in facioscapulohumeral muscular dystrophy: A 1‐year follow‐up study
- 15 March 2018
- journal article
- research article
- Published by Wiley in Muscle & Nerve
- Vol. 58 (2), 213-218
- https://doi.org/10.1002/mus.26127
Abstract
Introduction: Electrical impedance myography (EIM) is a noninvasive technique for measuring muscle composition and a potential physiological biomarker for facioscapulohumeral muscular dystrophy (FSHD). Methods: Thirty‐two participants with genetically confirmed and clinically affected FSHD underwent EIM in 7 muscles bilaterally. Correlations between EIM and baseline clinical measures were used to select EIM variables of interest in FSHD, and EIM and clinical measures were followed for 1 year. Results: There were no significant changes in the EIM variables. Although 50‐kHZ reactance correlated the strongest with clinical measures at baseline, the 50–211‐kHZ phase ratio demonstrated lower within‐subject 12‐month variability, potentially offering sample size savings for FSHD clinical trial planning. Discussion: EIM did not identify significant disease progression over 12 months. It is currently unclear whether this is because of limitations of the technology or the slow rate of disease progression in this cohort of FSHD patients over this period of time. Muscle Nerve 58: 213–218, 2018Keywords
Funding Information
- FSH Society (FSHS‐22013, FSHS‐82012)
- National Institute of Arthritis and Musculoskeletal and Skin Diseases (U01AR065119)
- University of Rochester (UL1 RR024160)
- Prinses Beatrix Spierfonds
This publication has 25 references indexed in Scilit:
- Facioscapulohumeral Muscular DystrophyNeurologic Clinics, 2014
- Distinct Disease Phases in Muscles of Facioscapulohumeral Dystrophy Patients Identified by MR Detected Fat InfiltrationPLOS ONE, 2014
- Optimizing Electrode Configuration for Electrical Impedance Measurements of Muscle via the Finite Element MethodIEEE Transactions on Biomedical Engineering, 2013
- Digenic inheritance of an SMCHD1 mutation and an FSHD-permissive D4Z4 allele causes facioscapulohumeral muscular dystrophy type 2Nature Genetics, 2012
- Electrical impedance myography in spinal muscular atrophy: A longitudinal studyMuscle & Nerve, 2012
- Electrical impedance myography as a biomarker to assess ALS progressionAmyotrophic Lateral Sclerosis, 2012
- A Unifying Genetic Model for Facioscapulohumeral Muscular DystrophyScience, 2010
- Statistical power analyses using G*Power 3.1: Tests for correlation and regression analysesBehavior Research Methods, 2009
- Electrical impedance myography: Background, current state, and future directionsMuscle & Nerve, 2009
- A Prospective, Quantitative Study of the Natural History of Facioscapulohumeral Muscular Dystrophy (FSHD)Neurology, 1997