Objective: Assess sex differences in disease severity, quality of life (QOL) and caregiver burden in early- and late-onset progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS)

Background: PSP and CBS are frontotemporal lobar degeneration (FTLD) syndromes characterized by progressive motor, cognitive, and functional decline. While sex differences have been reported in other neurodegenerative diseases, sex-specific patterns of disease severity and caregiver burden in PSP and CBS are understudied. Age of onset of PSP or CBS (early-onset: ≤65 years, late-onset:>65 years) may also influence clinical manifestations and their impact differentially between sexes.

Method: People diagnosed with PSP (31 females, 37 males) or CBS (43 females, 44 males) enrolled in ALLFTD, an FTLD natural history study with 28 North American centers, between 2015 and 2025 with at least one longitudinal visit were included. Disease severity (PSP Rating Scale, MoCA, NPI-Q), quality of life (DEMQOL), and caregiver burden (Zarit Burden Interview, ZBI) were assessed annually. For each clinical outcome, linear mixed-effects models were fit including a three-way interaction of sex, onset age group, and time since baseline visit, along with corresponding lower-order interactions. All models were adjusted for years of education and age at visit.

Results: The percentage of people with early- and late-onset within PSP and CBS groups did not differ by sex. Age and years of education were similar for the sexes at baseline. Females were more likely to have male caregivers, while males were more likely to have female caregivers. At baseline, females with early-onset CBS had lower NPI-Q scores than males with early-onset CBS (β=-19.3, p=.003), and caregivers of females with early-onset PSP had lower ZBI scores than caregivers of males with early-onset PSP (β=-4.9, p=.003). No other significant interactions with sex were found.

Conclusion: Females with early-onset CBS may experience less severe behavioral symptoms, and males with early-onset PSP may have more caregiver burden despite similar disease severity. These differences were present only in caregiver-reported measures, suggesting that sex differences in PSP and CBS may be dependent on caregiver perception and experience. Types of caregiving relationships and caregiver characteristics including gender may differ between sex groups and can impact disease related outcomes.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/sex-differences-in-disease-severity-and-impact-for-progressive-supranuclear-palsy-and-corticobasal-syndrome/