Swallowing assessment in myotonic dystrophy type 1 using fiberoptic endoscopic evaluation of swallowing (FEES)

• Published in: Neuromuscular disorders : NMD Vol. 24; no. 12; pp. 1054 - 1062
• Main Authors: Pilz, Walmari, Baijens, Laura W.J., Passos, Valéria Lima, Verdonschot, Rob, Wesseling, Frederik, Roodenburg, Nel, Faber, Catharina G., Kremer, Bernd
• Format: Journal Article
• Language: English
• Published: England Elsevier B.V 01.12.2014
• Subjects: Adult; Aged; Deglutition; Deglutition - physiology; Deglutition disorders; Deglutition Disorders - diagnosis; Deglutition Disorders - physiopathology; Dysphagia; Endoscopy - methods; Female; Fiber Optic Technology - methods; Fiberoptic endoscopic evaluation of swallowing; Humans; Male; Middle Aged; Multivariate Analysis; Myotonic Dystrophy - diagnosis; Myotonic Dystrophy - physiopathology; Myotonic dystrophy type 1; Neurology; Pharynx - physiopathology; Physical Stimulation - methods; Reproducibility of Results; Severity of Illness Index; Young Adult; Myotonic dystrophy type 1; Deglutition; Fiberoptic endoscopic evaluation of swallowing; Dysphagia; Deglutition disorders
• ISSN: 0960-8966; 1873-2364; 1873-2364
• DOI: 10.1016/j.nmd.2014.06.002

•The swallowing function of patients with myotonic dystrophy type 1 is described.•Pharyngeal pooling and piecemeal deglutition are important determinants of dysphagia.•Dysphagia is already present in the early stages of the muscular disease.•The magnitude of the swallowing impairment depends on the bolus consistency.•Swallowing assessment should include different bolus consistencies. This study describes the swallowing function of patients with myotonic dystrophy type 1 (DM1) and the effect of bolus consistency on swallowing in this group. The aim of the study is twofold: (a) to identify which (and to what extent) swallowing variables change for DM1 patients relative to healthy control subjects and (b) to examine whether the degree of oropharyngeal dysphagia is associated with disease severity. Forty-five consecutive DM1 patients and ten healthy subjects underwent a swallowing assessment, at Maastricht University medical Center in the Netherlands. The assessment included a standardized fiberoptic endoscopic evaluation of swallowing (FEES) protocol using different bolus consistencies. Clinical severity of the disease was assessed using the muscular impairment rating scale (MIRS). Significant differences were found between patients and controls for all FEES variables. The magnitude of these differences depended on the bolus consistency. The odds of a more pathological swallowing outcome increased significantly with higher MIRS levels. In conclusion, swallowing function is found to be significantly altered in DM1 patients. The results emphasize the importance of conducting a detailed swallowing assessment in all patients, even those with mild muscle weakness.