Muscle ultrasound is a sensitive biomarker in oculopharyngeal muscular dystrophy.

ABSTRACT:

Introduction/aims

Oculopharyngeal muscular dystrophy (OPMD) is a late-onset, progressive muscle disease. Quantitative muscle ultrasound (QMUS) assesses structural changes in muscles and is a sensitive biomarker in neuromuscular disorders. Our aim of this study was to determine whether QMUS can detect muscle pathology and can be used as longitudinal imaging biomarker in OPMD.

Methods

Genetically confirmed OPMD patients, recruited by their treating physicians or from the national neuromuscular database, were examined twice, 20 months apart, using QMUS of orofacial and limb muscles, and measurements of functional capacity and muscle strength. Absolute echo intensity (AEI) and muscle thickness of all muscles were analyzed and correlated with clinical data.

Results

The ton

SUBMITTER: Kroon RHMJM 

PROVIDER: S-EPMC9804746 | biostudies-literature | 2022 Oct

REPOSITORIES: biostudies-literature