March 28, 2009
FSH Society Initiated Nutrition Research Describes a New MRI/MRS Imaging Method to Provide Biomarkers for Clinical Trials and To Evaluate Muscle Damage in FSHD
Researchers in Nijmegen, the Netherlands theorized that quantitative assessment of MR imaging observations found in previously funded FSH Society Sam E. and Mary F. Roberts Nutrition Research projects on MRI by Dr. Kemp at Liverpool, England, could provide an objective non-invasive biomarker for individual muscle involvement in FSHD. Drs. Kan and Heerschaap, developed a new MR method to “quantitatively separate muscular and fat content in different muscles of the lower leg in healthy volunteers and patients with FSHD, using differences in T2 relaxation times of fat and muscle tissue, to provide an objective biomarker for individual muscle involvement in FSHD.” The researchers acknowledged the Society’s role in developing this much needed non-invasive tool -- ”Acknowledgements. The authors gratefully acknowledge Frank Vergeldt (Wageningen University, Wageningen, The Netherlands) for his contribution to the fitting algorithm and the FSH Society Sam E. and Mary F. Roberts Nutrition Research Fellowship Grant FSHS-SMRF-003 and Prinses Beatrixfonds WAR06-0217 for funding.”
The research helps us to understand the difficulties in FSHD research and how major differences can occur within the same muscle. “Typically,muscle involvement was non-uniform even within one muscle, indicating that MRI can be used as a valuable tool to study pathophysiology and therapy evaluation in FSHD….” “In five patients, muscle fractions were calculated separately for each slice and this revealed considerable differences in the degree of involvement within one muscle (up to 37%) in some muscles. Often, the degree of involvement was larger at the distal end of the muscle. This indicates that the degree of involvement can be non-uniform over the muscle, which could provide important clues for the patho-physiology of the disease. Furthermore, when evaluating therapies or assessing the disease progression, great care should be taken to assess a muscle at the same location every time, or to assess a muscle as a whole. Finally, when taking a muscle biopsy, MRI might be helpful to guide the optimal location as 20% of the muscle biopsies in FSHD are reported to be normal. Our method provides a quantitative assessment of fatty infiltration based on a straightforward multi-echo sequence which is available across MR vendors. Therefore, the method would be easily applicable in a multi center clinical trial. It could also be used to evaluate several muscle groups in different limbs if the appropriate hardware to image different parts of the body is available. Manual outlining of individual muscles is relatively time consuming at 2 min per slice, however, semi-automated procedures could be developed in the future, as the muscle boundaries are commonly well preserved in muscular dystrophies.”
Neuromuscul Disord. 2009 May;19(5):357-62. Epub 2009 Mar 28
Quantitative MR imaging of individual muscle involvement in facioscapulohumeral muscular dystrophy.
Kan HE, Scheenen TW, Wohlgemuth M, Klomp DW, van Loosbroek-Wagenmans I, Padberg GW, Heerschap A.
Department of Radiology, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands. h.e.kan@lumc.nl
The purpose of this study was to implement a quantitative MR imaging method for the determination of muscular and fat content in individual skeletal muscles of patients with facioscapulohumeral muscular dystrophy (FSHD). Turbo Inversion Recovery Magnitude (TIRM) and multiecho MR images were acquired from seven FSHD patients and healthy volunteers. Signal decay in the multiecho MR images was fitted to a biexponential function with fixed relaxation rates for muscle and fat tissue and used to calculate the degree of fatty infiltration in eight muscles in the lower leg. Considerable differences in fatty infiltration between different muscles were observed in FSHD patients, suggesting that this could be used as a biomarker for disease progression. TIRM imaging indicated an inflammatory component of the disease previously only observed in muscle biopsies. Typically, muscle involvement was non-uniform even within one muscle, indicating that MRI can be used as a valuable tool to study pathophysiology and therapy evaluation in FSHD.
PMID: 19329315 [PubMed - in process]
{Excerpt From Paper]
Acknowledgements
The authors gratefully acknowledge Frank Vergeldt (Wageningen University, Wageningen, The Netherlands) for his contribution to the fitting algorithm and the FSH Society Sam E. and Mary F. Roberts Nutrition Research Fellowship Grant FSHS-SMRF-003 and Prinses Beatrixfonds WAR06-0217 for funding.
