1. MRI change metrics of facioscapulohumeral muscular dystrophy: Stir and T1
- Author
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Daniel G. Miller, Leo H. Wang, Gregory T. Carter, Dennis W. W. Shaw, Seth D. Friedman, Christopher B. Budech, Nancy E. Gove, Sandra L. Poliachik, and Mark R. Ferguson
- Subjects
Physiology ,Fatty replacement ,Adipose tissue ,Thigh ,complex mixtures ,030218 nuclear medicine & medical imaging ,03 medical and health sciences ,Cellular and Molecular Neuroscience ,0302 clinical medicine ,Physiology (medical) ,Edema ,Medicine ,Facioscapulohumeral muscular dystrophy ,Muscular dystrophy ,Pathological ,medicine.diagnostic_test ,business.industry ,digestive, oral, and skin physiology ,Magnetic resonance imaging ,equipment and supplies ,medicine.disease ,medicine.anatomical_structure ,Neurology (clinical) ,medicine.symptom ,business ,Nuclear medicine ,030217 neurology & neurosurgery - Abstract
Introduction MRI evaluation in facioscapulohumeral muscular dystrophy (FSHD) demonstrates fatty replacement and inflammation/edema in muscle. Our previous work demonstrated short T1 inversion recovery (STIR)-hyperintense (STIR+) signal in muscle 2 years before fatty replacement. We evaluated leg muscle STIR changes and fatty replacement within 14 months. Methods FSHD subjects received 2 MRI scans of thigh and calf over a 6.9- to 13.8-month interval. Quality of life measures were collected. One Radiologist rated muscle changes on a semi-quantitative scale. Results Fifteen subjects completed longitudinal imaging. Four STIR + muscles and 3 STIR-normal (STIR-) muscles were rated as progressing to fatty tissue over the study period. Discussion STIR + muscles with confluent regions of fat at baseline increased more in fat, while STIR- muscles had increases in septal-fat over the study period. These changes may reflect two phases of FSHD, demonstrating MRI sensitivity is weighted toward gross pathological phases of the disease. Muscle Nerve 57: 905-912, 2018.
- Published
- 2018