Subtypes of PSP and Prognosis: A Retrospective Analysis

Abstract

Background: Progressive supranuclear palsy (PSP) is a clinically heterogeneous disease characterized by supranuclear gaze palsy and varying combinations of Parkinsonism, gait disturbances, postural instability, and fronto-limbic cognitive dysfunction. A major challenge in clinical diagnosis is the existence of subtypes whose clinical features overlap with those of other Parkinsonian disorders. Objectives: To categorize patients of PSP into its using the recently proposed movement disorder society criteria (2017) and to determine the prognosis of the PSP subtypes. Methods: Demographic and clinical data of patients diagnosed with PSP over a 21 year period were collected by review of medical records and categorized into its subtypes. Subtype prognosis was assessed from the interval between disease onset and attainment of the first of 5 clinical disability milestones namely wheelchair dependency, unintelligible speech, severe dysphagia, severe cognitive impairment, and urinary catheterization. Results: When categorized into subtypes, out of the 334 patients with PSP, PSP-RS predominated (72%), followed by PSP-parkinsonism (PSP-P) (13.5%), PSP-corticobasal syndrome (PSP-CBS) (5.1%), PSP-frontal (PSP-F) (4.2%), PSP-progressive gait freezing (PSP-PGF) (4.2%), PSP-postural instability (PSP-PI) (0.6%), and PSP-speech/language (PSP-SL) (0.3%). PSP-P reaches the milestones of wheelchair dependency, unintelligible speech, and dysphagia later than other subtypes. Conclusion: PSP-RS was the commonest and PSP-OM the rarest PSP subtype in our retrospective PSP cohort analysis. PSP-P had a better prognosis than all other subtypes of PSP. A large proportion of these cases would remain unclassified using NINDS-SPSP (1996) criteria.