Subtypes of PSP and Prognosis: A Retrospective Analysis.

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.