Longitudinal study of MRI and functional outcome measures in facioscapulohumeral muscular dystrophy.

ABSTRACT:

Background

Facioscapulohumeral muscular dystrophy (FSHD) is a patchy and slowly progressive disease of skeletal muscle. For MRI to be a useful biomarker in an FSHD clinical trial, it should reliably detect changes over relatively short time-intervals (~ 1 year). We hypothesized that fatty change over the study course would be most likely in muscles already demonstrating disease progression, and that the degree of MRI burden would be correlated with function.

Methods

We studied 36 patients with FSHD and lower-extremity weakness at baseline. Thirty-two patients returned in our 12-month longitudinal observational study. We analyzed DIXON MRI images of 16 lower-extremity muscles in each patient and compared them to quantitative strength measurement and ambulatory functional outc

SUBMITTER: Wang LH 

PROVIDER: S-EPMC7948347 | biostudies-literature | 2021 Mar

REPOSITORIES: biostudies-literature