Objective: This study aimed to compare longitudinal clinical progression between patients with progressive supranuclear palsy–Richardson syndrome (PSP-RS) and those with PSP-subcortical phenotypes.
Background: PSP is a heterogeneous neurodegenerative disorder with various clinical phenotypes. PSP-RS is generally considered the most rapidly progressive form, whereas subcortical variants often show a milder or slower course. However, longitudinal data directly comparing progression trajectories between PSP-RS and PSP-subcortical phenotypes remain limited, particularly in Asian populations.
Method: We analyzed longitudinal data from our PSP cohort including patients classified as PSP-RS or PSP-subcortical. Clinical progression was assessed using the PSP Rating Scale (PSPRS), the PSP Clinical Deficits Scale (PSP-CDS), and their respective subscores. Longitudinal changes in PSPRS and PSP-CDS total scores and subscores were analyzed using linear mixed models adjusted for age and sex and annualized progression rates were estimated.
Results: The mean age was similar between groups (70.9 ± 6.8 years in RS vs 71.2 ± 7.2 in subcortical), and disease duration did not differ significantly (median 3.58 years [IQR 2.50–5.00] vs 4.00 years [2.50–6.42]). At baseline, PSP-RS showed greater clinical severity, including higher median PSPRS total scores (37.0 [28.0–45.0] vs 21.0 [18.0–26.0], p<0.001) and higher PSP-CDS total scores (10.0 [8.0–13.0] vs 8.0 [6.0–9.0], p<0.001). Both groups showed progressive worsening over time, and overall progression rates were not significantly different between PSP-RS and PSP-subcortical phenotypes (PSPRS: 6.01 vs 4.42; PSP-CDS: 1.53 vs 0.77 points/year). Among PSPRS subscores, the history domain showed a significant between-group difference in progression (1.98 vs 0.79 points/year, p=0.032). Among PSP-CDS subscores, dexterity also differed significantly between groups (0.36 vs 0.03 points/year,p=0.007), whereas other subscores were not significant after multiple comparison correction.
Conclusion: Overall progression rates did not differ significantly between PSP-RS and PSP-subcortical phenotypes. However, phenotype-related differences were observed in specific domains, particularly the PSPRS history and PSP-CDS dexterity subscores. These findings highlight clinical heterogeneity across PSP phenotypes and suggest that domain-specific measures may be useful outcome markers in longitudinal studies.
To cite this abstract in AMA style:
SM. Lee, R. Kim, JY. Lee. Clinical progression of PSP-Richardson’s syndrome and other PSP variants in an East Asian cohort [abstract]. Mov Disord. 2026; 41 (suppl 1). https://www.mdsabstracts.org/abstract/clinical-progression-of-psp-richardsons-syndrome-and-other-psp-variants-in-an-east-asian-cohort/. Accessed October 1, 2026.« Back to 2026 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/clinical-progression-of-psp-richardsons-syndrome-and-other-psp-variants-in-an-east-asian-cohort/