Orofacial muscles may be affected in early stages of Becker muscular dystrophy: A preliminary study

• Published in: Muscle & nerve Vol. 61; no. 2; pp. 213 - 217
• Main Authors: Lagarde, Marloes L.J., Alfen, Nens, Geurts, Alexander C.H., Groot, Imelda J.M., Engel‐Hoek, Lenie
• Format: Journal Article
• Language: English
• Published: Hoboken, USA John Wiley & Sons, Inc 01.02.2020; Wiley Subscription Services, Inc
• Subjects: Algorithms; Becker muscular dystrophy; Becker's muscular dystrophy; Bite Force; Child; Clinical; Clinical s; Deglutition Disorders - etiology; Duchenne muscular dystrophy; Dysphagia; Dystrophy; Facial Muscles - diagnostic imaging; Facial Muscles - physiopathology; Humans; Male; Mastication; Masticatory Muscles - diagnostic imaging; Masticatory Muscles - physiopathology; Muscle contraction; Muscles; Muscular dystrophy; Muscular Dystrophy, Duchenne - complications; Muscular Dystrophy, Duchenne - physiopathology; orofacial muscles; Skeletal muscle; Swallowing; Temporalis muscle; Ultrasonography; Ultrasound; Walk Test; Walking; dysphagia; Becker muscular dystrophy; mastication; Duchenne muscular dystrophy; orofacial muscles; ultrasound
• ISSN: 0148-639X; 1097-4598; 1097-4598
• DOI: 10.1002/mus.26771

Background Dysphagia is reported in patients with Duchenne or Becker muscular dystrophy. Our clinical experience suggests that, compared with Duchenne patients, impaired mastication and swallowing occur early in Becker patients relative to their skeletal muscle involvement. The aim of this study was to assess dysphagia in Duchenne and Becker patients in relation to ambulatory capacity. Methods In patients in the early ambulatory stage, clinical symptoms, quantitative muscle ultrasound of the orofacial muscles, and maximum bite force were assessed. The 6‐Minute Walk Test (6MWT) was used to measure ambulatory capacity. Results Eleven Duchenne and 11 Becker patients were included. Although Becker patients had a greater 6MWT distance than Duchenne patients, the occurrence of mastication and swallowing difficulties was similar. The temporalis muscle was significantly thicker in Becker patients. Conclusions Clinicians should be aware of dysphagia in both groups, even when ambulation is still well preserved.