Evaluation of Dysphagia in Myositis and Muscular Dystrophy Using Real-time MRI and Quantitative Muscle Ultrasound
Rachel Zeng1, Anke Rietveld5, Omar Al Bourini2, Rosemarie Kroon6, Arno Olthoff3, Matthias Weidenmueller3, Per-Ole Carsten4, Isabel Kommerell4, Saskia Schuetz4, Corinne Horlings5, Hanneke Kalf6, Bert De Swart6, Baziel Van Engelen5, Tim Friede1, Sabine Hofer1, Jens Frahm1, Joachim Lotz1, Ali Seif Amir Hosseini2, Jens Schmidt7, Christiaan Saris5
1University Medical Center Göttingen, 2Department of Diagnostic and Interventional Radiology, 3Department of Otorhinolaryngology, Phoniatrics and Pedaudiology, 4Department of Neurology, University Medical Center Göttingen, 5Department of Neurology, 6Department of Rehabilitation, Radboudumc, 7University Hospital Brandenburg Medical School / Immanuel Klinik Rüdersdorf
Background:
Swallowing dysfunction -dysphagia- is a frequent and debilitating symptom in neuromuscular disorders, leading to malnutrition, cachexia, aspiration pneumonia and death. Identification of the underlying pathophysiological mechanisms is important for diagnosis and treatment. As standard assessments have limitations, novel imaging techniques are needed. In the present study, we investigated the utility of real-time MRI and quantitative muscle ultrasound for characterizing dysphagia in two different neuromuscular disorders.
Design/Methods:
This prospective cohort study included 18 patients with inclusion body myositis (IBM, 33% female, age 68.9±7.7 years) and 13 with oculopharyngeal muscular dystrophy (OPMD, 62% female, age 55.9±7.0 years) from two European Neuromuscular research centers (Nijmegen, NL; Göttingen, DE). Swallowing function was studied using real-time MRI (RT-MRI), FEES (flexible endoscopic evaluations of swallowing) and clinical assessments. T1-mapping and quantitative muscle ultrasound (QMUS) were used to analyze tissue properties in swallowing muscles. Outcomes were compared between the two muscle diseases. RT-MRI values were also compared to 22 age- and sex-matched non-myopathic controls.
Results:
RT-MRI revealed significantly prolonged oral transit times in OPMD vs. controls (difference between means=581.2 ms, 95% CI 225.9-936.4, p=0.002). Pharyngeal transit time was significantly prolonged in IBM vs. controls (difference between means=1132.8 ms, 95% CI 482.2-1783, p=0.001). A cricopharyngeal bar as a well-established morphological indicator of dysphagia was identified in 80% patients with IBM compared to 53% in OPMD. Fatty degeneration of the tongue in OPMD significantly correlated between MRI-T1 values and ultrasound echogenicity (Spearman`s ρ=-0.52, p=0.005). ROC revealed excellent discrimination between diseases by combining RT-MRI, T1-mapping and QMUS (AUC=0.95, 95% CI 0.86-1.00), while FEES and clinical assessments failed to differentiate specific patterns of dysphagia.
Conclusions:
This study supports the value of novel MRI and ultrasound techniques for clinical use by identifying the pathophysiology and severity of impaired swallowing. Differentiating the phenotypes of dysphagia can aid in the diagnosis and treatment of affected patients.
Disclaimer: Abstracts were not reviewed by Neurology® and do not reflect the views of Neurology® editors or staff.