Gender differences in progressive supranuclear palsy.

The gender differences in progressive supranuclear palsy (PSP) are not extensively studied. The objective of this study was to determine the gender differences in the phenotypic expression and progression in PSP. We did a retrospective review of medical records of patients diagnosed with PSP over a 21-year period. The interval between disease onset and attainment of the five clinical disability milestones namely wheel chair dependency, unintelligible speech, severe dysphagia, severe cognitive impairment and urinary catheterization was used to determine the progression. Data was analysed from the case records of 334 patients with PSP. 209 patients (62.2%) were male and 125 (37.4%) among the patients were women (male:female ratio = 1.6:1). Males had older age at onset with longer duration of illness at time of presentation. Tremors were more common, PSP-P phenotype was more frequent and time to attain wheelchair dependency was earlier in males. Falls within 1 year of disease onset, apathy and executive dysfunction were more frequent and time to attain unintelligible speech, severe dysphagia and cognitive impairment were earlier in females. This study in a large cohort of clinically diagnosed cases of PSP has showed that gender differences exist in PSP in terms of clinical characteristics, progression of the disease.

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.