Karlien Mul1,2, Chad Heatwole3, Katy Eichinger3, Nuran Dilek3, William B Martens3, Baziel G M Van Engelen2, Rabi Tawil3, Jeffrey M Statland1,3. 1. Department of Neurology, University of Kansas Medical Center, 4330 Shawnee Mission Parkway, Suite 323 Fairway, Kansas, 66205, USA. 2. Department of Neurology, Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Center, Nijmegen, The Netherlands. 3. Department of Neurology, University of Rochester Medical Center, Rochester, New York, USA.
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, 2018.
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 FSHDpatients over this period of time. Muscle Nerve 58: 213-218, 2018.
Authors: Karlien Mul; Sanne C C Vincenten; Nicol C Voermans; Richard J L F Lemmers; Patrick J van der Vliet; Silvère M van der Maarel; George W Padberg; Corinne G C Horlings; Baziel G M van Engelen Journal: Neurology Date: 2017-10-13 Impact factor: 9.910
Authors: Jeffrey M Statland; Bharati Shah; Don Henderson; Silvere Van Der Maarel; Stephen J Tapscott; Rabi Tawil Journal: Muscle Nerve Date: 2015-06-18 Impact factor: 3.217
Authors: Barbara H Janssen; Nicoline B M Voet; Christine I Nabuurs; Hermien E Kan; Jacky W J de Rooy; Alexander C Geurts; George W Padberg; Baziel G M van Engelen; Arend Heerschap Journal: PLoS One Date: 2014-01-14 Impact factor: 3.240
Authors: Samantha LoRusso; Nicholas E Johnson; Michael P McDermott; Katy Eichinger; Russell J Butterfield; Elena Carraro; Kiley Higgs; Leann Lewis; Karlien Mul; Sabrina Sacconi; Valeria A Sansone; Perry Shieh; Baziel van Engelen; Kathryn Wagner; Leo Wang; Jeffrey M Statland; Rabi Tawil Journal: BMC Neurol Date: 2019-09-10 Impact factor: 2.474