Ultrasonography of abdominal muscles: Differential diagnosis of late-onset Pompe disease and myotonic dystrophy type 1.

ABSTRACT:

Introduction

Axial muscles are involved earlier and to a greater extent in late-onset Pompe disease (LOPD) than in myotonic muscular dystrophy type 1 (DM1). We aimed to evaluate abdominal muscles in LOPD compared in DM1 using muscle ultrasonography.

Methods

Patients with LOPD (n = 3), DM1 (n = 10), and age- and gender-matched healthy subjects (n = 34) were enrolled for muscle ultrasonography. Patients with LOPD and DM1 were 20 to 59 years of age with a disease duration ranging between 7 and 30 years. A multifrequency linear transducer was used to evaluate quality and thickness in the abdominal muscles and extremities.

Results

The quantitative muscle echo score revealed a higher Z score in abdominal muscles in Patients with LOPD (scores were relati

SUBMITTER: Hsieh PC 

PROVIDER: S-EPMC9488967 | biostudies-literature | 2022

REPOSITORIES: biostudies-literature