Application of Digital Accelerometry for Swallow Imaging (DASI) for Dysphagia Evaluation and Treatment in a Series of Myotonic Dystrophy Type 1 (DM1) Patients (P05.189)

Objective: To use digital accelerometry for swallow imaging (DASI) for evaluation and treatment of dysphagia in DM1 patients. Background Dysphagia is a potentially life threatening complication of DM1 patients. Design/Methods: Patients with DM1 and history of aspiration or dysphagia underwent a formal swallow evaluation +/- modified barium swallow (MBS). We used noninvasive DASI technology with software designed for swallow imaging and calculation of key parameters of swallow biomechanics. Trials included spontaneous and instructed dry and wet swallows with different consistencies. We analyzed shape, amplitude, timing, frequency and jitter looking for swallow movement dysfunction. DASI biofeedback: Patients observed swallow traces on the computer screen and were coached in improving strength, duration and timing of swallow. Results: Case 1: A 24-year old man with congenital DM1, dysphagia, and repeated hospitalizations for aspiration pneumonia had a MBS showing aspiration of pyriform sinus residue. DASI evaluation showed excessive pre/post-swallow laryngeal movement, decreased pharyngeal phase control with multiple small amplitude peaks. DASI Treatment: Patient improved swallow strenght and control of pharyngeal movement. Case 2: A 46 year-old man with adult-onset DM1 and choking episodes was hospitalized with pneumonia and placed on a ventilator. MBS showed aspiration during/after the swallow with delayed cough. DASI evaluation showed low amplitude and frequency of swallow with increased jitter and frequent coughing. DASI Treatment: Patient improved swallow strength. Case 3: A 33 year-old female with adult-onset DM1 reported dysphagia. Clinical swallow evaluation showed oral residue, reduced hyolaryngeal excursion, and throat clearing. DASI evaluation showed reduced amplitude and increased jitter, with extraneous, inefficient lingual/hyolaryngeal movements. DASI Treatment: Reduced extraneous laryngeal movements and improved control of swallow. Conclusions: DASI is an adjunct in dysphagia evaluation in DM1. DASI biofeedback is helpful in gaining increased swallow function through Active Repetitive Motion Therapy which is instructive for reducing extraneous, inefficient laryngeal movements and can be personalized according to the patient physical and mental status. Supported by: DASI equipment on loan from Elixir Therapeutics. Disclosure: Dr. Farkas has nothing to disclose. Dr. Entrekin has nothing to disclose. Dr. Milstead has nothing to disclose. Dr. Mosman has nothing to disclose. Dr. Berggren has nothing to disclose. Dr. Sampson has received personal compensation for activities with PTC Therapeutics and Myotonic Dystrophy Foundation.