Swallowing assessment in myotonic dystrophy type 1 using fiberoptic endoscopic evaluation of swallowing (FEES) |
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| Institution: | 1. Department of Otorhinolaryngology, Head and Neck Surgery, Maastricht University Medical Center, Maastricht, The Netherlands;2. Department of Methodology and Statistics, Maastricht University, Maastricht, The Netherlands;3. Emergency Department, Erasmus Medical Center, Rotterdam, The Netherlands;4. Department of Radiation Oncology (MAASTRO), GROW-School for Oncology and Developmental Biology, Maastricht University Medical Center, The Netherlands;5. Department of Neurology, Maastricht University Medical Center, Maastricht, The Netherlands;1. Unit of Neuromuscular Disorders, Laboratory of Molecular Medicine, Bambino Gesu’ Children’s Research Hospital, Rome, Italy;2. Folkhälsan Institute of Genetics and Department of Medical Genetics, Haartman Institute, University of Helsinki, Helsinki, Finland;3. Pediatric Neurology Unit, Catholic University School of Medicine, Rome, Italy;4. Institute of Neurology, Catholic University School of Medicine, Rome, Italy;5. Neuromuscular Research Center, University of Tampere and Tampere University Hospital, Tampere, Finland;6. Neurology Department, Vaasa Central Hospital, Vaasa, Finland |
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| Abstract: | 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. |
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| Keywords: | Dysphagia Myotonic dystrophy type 1 Deglutition Deglutition disorders Fiberoptic endoscopic evaluation of swallowing |
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