Abstract
Objective: To verify the association of midbrain-based MRI measures as well as cortical volumes with disease core features and progression in patients with Progressive Supranuclear Palsy (PSP).Methods: Sixty-seven patients (52.2% with Richardson's syndrome) were included in the present analysis. Available midbrain-based MRI morphometric assessments as well as cortical lobar volumes were computed. Ocular, gait and postural involvement at the time of MRI was evaluated with the PSP rating scale. Specific milestones or death were used to estimate disease progression up to 72 months follow up. Hierarchical regression models and survival analysis were used for analyzing cross-sectional and longitudinal data, respectively.Results: Multivariate models showed vertical supranuclear gaze palsy was associated with smaller midbrain area (OR: 0.02, 95% CI 0.00–0.175, p = 0.006). Cox regression adjusted for age, disease duration, and phenotype demonstrated that lower midbrain area (HR: 0.122, 95% CI 0.030–0.493, p = 0.003) and diameter (HR: 0.313, 95% CI 0.112–0.878, p = 0.027), higher MR Parkinsonism Index (HR: 6.162, 95% CI 1.790–21.209, p = 0.004) and larger third ventricle width (HR: 2.755, 95% CI 1.068–7.108, p = 0.036) were associated with higher risk of dependency on wheelchair.Conclusions: Irrespective of disease features and other MRI parameters, reduced midbrain size is significantly associated with greater ocular motor dysfunction at the time of MRI and more rapid disease progression over follow up. This is the first comprehensive study to systematically assess the association of available midbrain-based MRI measures and cortical volumes with disease severity and progression in a large cohort of patients with PSP in a real-world setting.
Highlights
Progressive Supranuclear Palsy (PSP) is a rare, rapidly progressive, neurodegenerative disease characterized by dysfunction in four core domains including ocular motor function, postural instability, akinesia, and cognition represented by a number of clinical features [1]
After adjusting for age and disease duration in the univariate model, vertical supranuclear gaze palsy (VSGP) was associated with PSP Richardson’s syndrome (PSP-RS) phenotype, midbrain area, midbrain diameter, pons diameter, length of midbrain tegmentum, MCP, SCP, MR Parkinsonism Index (MRPI), pons-to midbrain area ratio (P/M), pons-to-midbrain diameter ratio, occipital volume
Cox regression analysis adjusted for age, disease duration and phenotype demonstrated that lower midbrain area (HR: 0.122, 95% confidence interval (CI) 0.030–0.493, p = 0.003), midbrain diameter (HR: 0.313, 95% CI 0.112–0.878, p = 0.027), higher MRPI (HR: 6.162, 95% CI 1.790–21.209, p = 0.004) and larger third ventricle width (HR: 2.755, 95% CI 1.068–7.108, p = 0.036) but not length of midbrain tegmentum (HR: 0.431, 95% CI 0.176–1.056, p = 0.066)
Summary
Progressive Supranuclear Palsy (PSP) is a rare, rapidly progressive, neurodegenerative disease characterized by dysfunction in four core domains including ocular motor function, postural instability, akinesia, and cognition represented by a number of clinical features [1]. Irrespective of the phenotype, the presence of either slowing velocity of vertical saccades or vertical supranuclear gaze palsy (VSGP) is mandatory for the diagnosis of probable PSP [1]. Research studies on magnetic resonance imaging (MRI) measures in PSP have focused on supporting the clinical diagnosis of disease. Given the key role of midbrain in PSP-related pathological process, a number of midbrain-based MRI morphometric measures have shown adequate diagnostic accuracy in differentiating PSP-RS from healthy subjects and other parkinsonian disorders [2,3,4,5,6,7]
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