LAUSR

Dysphagia is a frequent symptom of multiple system atrophy (MSA). Current dysphagia management is compensatory, involving thickening liquids, postural maneuvers, or enteral feeding. There are no studies of dysphagia rehabilitation in MSA. We found that skill-training improved swallowing function in a 44-year-old man who developed progressive walking difficulty, urinary urgency, and orthostatic hypotension at age 41. No evidence of cognitive disturbance was found. Based on the clinical features of cerebellar ataxia, parkinsonism, and autonomic disturbance, a movement disorder specialist (SHK) diagnosed him with MSA cerebellar subtype. His scale for the assessment and rating for ataxia score was 15, unified Parkinson’s disease scale part III score was 17, unified multiple system atrophy rating scale (UMSARS)-1 was 14, and UMSARS-2 was 20. Prominent cerebellar signs were wide-based gait with variable stance. Magnetic resonance imaging showed prominent pontocerebellar atrophy with hot cross bun sign. He developed dysphagia (choking on liquids) within one year of the onset of gait imbalance. He had no history of aspiration pneumonia. Clinical swallowing evaluation revealed adequate bulbar muscle function. However, endoscopic evaluation revealed delayed swallowing, with the bolus passing over the epiglottis before swallowing onset (see Supporting Video 1). There was aspiration below the vocal folds and diffuse post-swallow pharyngeal residue, suggesting deficits in coordinating pharyngeal swallowing events and airway protection. Biofeedback in strength and skill training (BiSSkiT) is a novel dysphagia treatment approach that has been studied previously in Parkinson’s disease. BiSSkiT targets motor control and swallowing precision by providing feedback regarding the timing and strength of muscle contractions. This treatment differs from traditional swallowing rehabilitation, as it is task-specific, provides biofeedback, and calibrated increases in skill-requirement as proficiency improves. The patient completed six one-hour BiSSkiT sessions over the course of six weeks. Surface electromyography provided biofeedback on a monitor regarding contractions of the suprahyoid muscles. The BiSSkiT software generated a target for the patient to “hit” during swallowing (see Supporting Video 1). The size of the target changed according to the patient’s success, requiring the patient to monitor and modify swallowing muscle contractions. This has been hypothesized to activate parieto-premotor pathways, bypassing the basal ganglia and/or cerebellum and facilitating movement control. In addition to weekly therapy, the patient undertook daily home practice that included a smartphone video module with variably timed, audiovisual prompts to swallow saliva with a specific force (i.e., “swallow hard” or “swallow soft”), a skill targeted with BiSSkiT. As the goal was to facilitate increased cortical control over swallowing, we did not monitor for accuracy. The patient reported completing (on average) 60 cued swallows per week. Swallowing-related outcomes were measured preand posttreatment. BiSSkiT performance improved post-treatment from 68% accuracy (target: 118 pixels) to 78% accuracy (target: 27 pixels), indicating improvements even as the task became more difficult. The patient had subjective improvement in swallowing symptoms such as decreased choking and coughing (per the SWAL-QOL). We also observed robust improvements in swallowing physiology (Table 1; see Supporting Video 1). Premature spillage and aspiration were eliminated, and post-swallow residue was reduced, allowing the patient to remain on an unrestricted diet. This research is the first report of dysphagia rehabilitation in MSA-C. Although exploratory, results suggest potential benefit for others with MSA-C and warrants further investigation. By therapeutically targeting swallowing precision, the patient appears