Objective: We sought to evaluate PSP-Richardson syndrome (PSP-RS) for uniformity of progression over nearly the entire disease course. Rather than comparing scores to the simple passage of time, we used progression of downgaze palsy, the most specific feature central to PSP, as milestones.

Background: There has been insufficient study of the progression of progressive supranuclear palsy (PSP), particularly for periods of more than 12 months and in the later disease stages.

Method: We used a longitudinal database of 462 patients with probable PSP-RS followed from 1994 to 2020, assessing 37 variables (representing the comprehensive symptoms and signs) comprising the 28 PSP Rating Scale (PSPRS) items plus 9 derived variables. For 120 patients (mean onset age of 68.0 years old, 49% female) with multiple visits and known dates of death, we compared progression rates among intervals defined by symptom onset date, the visit with first downgaze palsy (mean 3.3 years post-onset), and the last visit within 1 year of death (mean 5.2 years post-onset, 0.4 years before death). We also similarly analyzed a subgroup of 42 patients using an interim visit where downgaze palsy reached the maximum possible score on the PSPRS.

Results: Progression rates for dysphagia for solids and dysphagia for liquids accelerated later in the course (p<0.001 for each). No other variable accelerated or decelerated to this degree or with clinical significance.

Conclusion: These observations may provide useful clinical counseling information, comparator data for long-term treatment trials, and insight into the pathogenetic process underlying PSP-RS.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/progression-of-the-clinical-features-of-progressive-supranuclear-palsy-richardson-syndrome-in-early-and-advanced-stages/