We found a match
Your institution may have rights to this item. Sign in to continue.
- Title
Ultrasonography of abdominal muscles: Differential diagnosis of late-onset Pompe disease and myotonic dystrophy type 1.
- Authors
Pei-Chen Hsieh; Chun-Wei Chang; Long-Sun Ro; Chin-Chang Huang; Jia-En Chi; Hung-Chou Kuo
- 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 relatively normal for the biceps and flexor digitorum groups). Patients with LOPD had significantly lower abdominal muscle thickness than patients with DM1. Abdominal muscle strength was significantly correlated with the muscle echogenicity, trunk impairment scale, and trunk control test. The extremities' sum score was correlated with the total Medical Research Council score. Discussion: The increased quantitative muscle score in abdominal muscles, sparing the biceps and flexor digitorumgroups, may offer differential diagnosis between LOPD and DM1. Ultrasound can easily access abdominal muscles and investigate muscle echogenicity and thickness. A quantitative approach usingmuscle echogenicity rather thanmuscle thicknessmay provide a greater correlation with trunk muscle function.
- Subjects
MEDICAL Research Council (Great Britain); ABDOMINAL muscles; MYOTONIA atrophica; ULTRASONIC imaging; MUSCLE strength; DIFFERENTIAL diagnosis
- Publication
Frontiers in Neurology, 2022, Vol 13, p1
- ISSN
1664-2295
- Publication type
Article
- DOI
10.3389/fneur.2022.944464