Imaging

Oculopharyngeal muscular dystrophy (OPMD) is a rare, late onset, progressive muscle disorder with ptosis, dysphagia and limb weakness as the most common features. We aimed to assess whether quantitative muscle ultrasound (QMUS) can detect muscle abnormalities and disease progression in OPMD and can therefore be used as an imaging biomarker. A longitudinal study of 20 months was performed with forty-three genetically confirmed OPMD patients. The study protocol included QMUS of orofacial muscles, muscles of the upper and lower extremities and clinical measures of muscle strength (fixed dynamometry, MRC grading, maximum bite force (MBF) and maximum isometric tongue strength (MITP)) and functional capacity (Motor Function Measure (MFM), 10-step stair test, maximum chewing, swallowing and speaking capacity).

Source: Neuromuscular Disorders - September 19, 2021 Category: Neurology Authors: R. Kroon, J. Kalf, B. de Swart, B. van Engelen, C. Horlings Source Type: research