MRI change metrics of facioscapulohumeral muscular dystrophy: Stir and T1.

Document Type

Article

Publication Date

6-1-2018

Publication Title

Muscle & nerve

Keywords

MRI; facioscapulohumeral muscular dystrophy; fat; inflammation; longitudinal; muscle imaging

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.

Clinical Institute

Neurosciences (Brain & Spine)

Department

Rehabilitation

Department

Neurosciences

Department

Diagnostic Imaging

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