Abstract Details

Evaluation of Dysphagia in Myositis and Muscular Dystrophy Using Real-time MRI and Quantitative Muscle Ultrasound
Neuromuscular and Clinical Neurophysiology (EMG)
S16 - Updates on Nerve and Muscle Disorders (1:36 PM-1:48 PM)
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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. 
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.
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.
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. 
Authors/Disclosures
Christiaan Saris, MD, PhD (Radboudumc)
PRESENTER 		Dr. Saris has nothing to disclose.
Rachel Zeng, MD Dr. Zeng has nothing to disclose.
Anke Rietveld, MD Dr. Rietveld has nothing to disclose.
Omar Al-Bourini, MD Dr. Al-Bourini has nothing to disclose.
Rosemarie Kroon, MA Mrs. Kroon has nothing to disclose.
Arno Olthoff, MD Prof. Olthoff has nothing to disclose.
Matthias Weidenmueller, MD Dr. Weidenmueller has nothing to disclose.
Per-Ole Carstens, MD Dr. Carstens has nothing to disclose.
Isabel Kommerell, MD Mrs. Kommerell has nothing to disclose.
Saskia Schütz, MD Dr. Schütz has nothing to disclose.
Corinne G. HORLINGS, MD, PhD Dr. HORLINGS has nothing to disclose.
Johanna G. Kalf, PhD The institution of Dr. Kalf has received research support from Netherlands Organisation for Health Research and Development (ZonMw).
Bert de Swart Bert de Swart has nothing to disclose.
Baziel G. van Engelen, MD, PhD, MA (Radboud University Nijmegen Medical Centre) The institution of Dr. van Engelen has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Fulcrum . The institution of Dr. van Engelen has received research support from Stichting Spieren voor Spieren. The institution of Dr. van Engelen has received research support from Prinses Beatrix Fonds. The institution of Dr. van Engelen has received research support from Dutch FSHD society. Dr. van Engelen has received intellectual property interests from a discovery or technology relating to health care.
Tim Friede, PhD Prof. Friede has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Novartis. Prof. Friede has received personal compensation in the range of $500-$4,999 for serving as a Consultant for CSL Behring. Prof. Friede has received personal compensation in the range of $500-$4,999 for serving as a Consultant for RECARDIO. Prof. Friede has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Daiichi Sankyo. Prof. Friede has received personal compensation in the range of $500-$4,999 for serving as a Consultant for BMS. Prof. Friede has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Prof. Friede has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Prof. Friede has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Bayer. Prof. Friede has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for BiosenseWebster. Prof. Friede has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Galapagos. Prof. Friede has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Enanta. Prof. Friede has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for VICO Therapeutics. Prof. Friede has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Fresenius Kabi Deutschland GmbH.
Sabine Hofer, PhD Dr. Hofer has nothing to disclose.
Jens Frahm, PhD Prof. Frahm has nothing to disclose.
Joachim Lotz, MD The institution of Prof. Lotz has received research support from DFG. The institution of Prof. Lotz has received research support from BMBF.
Ali Seif Amir Hosseini, MD Dr. Seif Amir Hosseini has nothing to disclose.
Jens Schmidt, MD, FAAN (University Hospital Brandenburg Medical School / Immanuel Klinik Rüdersdorf) Dr. Schmidt has received personal compensation in the range of $500-$4,999 for serving as a Consultant for CSL Behring and UCB. Dr. Schmidt has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for CSL Behring and LFB. Dr. Schmidt has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for CSL Behring. Dr. Schmidt has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for UCB. The institution of Dr. Schmidt has received research support from Kezar, Novartis and AFM.