Abstract
BACKGROUND: Dysphagia can lead to fatal aspiration pneumonia in progressive supranuclear palsy (PSP). Little is known about the longitudinal progression of dysphagia or whether it differs across PSP clinical variants. OBJECTIVES: To characterize longitudinal changes in dysphagia across PSP variants and determine relationships with disease severity and rate of atrophy in PSP-related regions. METHODS: Sixty-one PSP patients were recruited and underwent at least two serial evaluations, comprising neurological and videofluorographic exams, and MRI. Variables-of-interest were the oral and pharyngeal total sum scores (OTSS/PTSS) from the Modified Barium Swallow Impairment Profile, the Penetration Aspiration Scale (PAS), Functional Oral Intake Scale (FOIS), and lingual pressure assessments. Rates of change were compared across PSP variants and related to baseline scores, PSP Rating Scale and regional brain atrophy rates. RESULTS: Dysphagia progressed longitudinally, particularly with worsening in OTSS and anterior and posterior lingual pressures. Rate of change in OTSS was fastest in PSP with predominant speech-language disorder. Negative correlations were observed between rates of change and baseline scores for OTSS, PTSS and PAS. Rates of change in lingual pressure measures correlated with rates of change in the PSP Rating Scale. Rate of change in the FOIS correlated with rates of change in OTSS and lingual pressure. Rates of brain atrophy were associated with baseline OTSS, PTSS, and PAS. CONCLUSIONS: Dysphagia and lingual pressure worsen over time in PSP, with variability in rates of change in the OTSS across PSP variants and evidence that dysphagia is related to rates of atrophy.