The contribution of white matter changes to clinical phenotype in progressive supranuclear palsy

Abstract

MRI; Phenotype; Progressive supranuclear palsy

Ressonància magnètica; Fenotip; Paràlisi supranuclear progressiva

Resonancia magnética; Fenotipo; Parálisis supranuclear progresiva

White matter hyperintensities (WMH) are considered magnetic brain imaging (MRI) biomarkers of cerebral small vessel disease but their clinical role in neurodegenerative-related disorders is poorly understood. This study describes the distribution of WMH on brain MRI in Progressive Supranuclear Palsy (PSP) in comparison with Parkinson’s disease (PD) and explores their possible impact on disease’s features. Sixty PSP and 33 PD patients were included. Motor symptoms, cardiovascular risk factors and the age-related white matter changes (ARWMC) score was computed to rate WMH for both groups. Pearson’s correlation and linear or logistic regression analysis were used to check for relationships between ARWMC and PSP clinical scores. The mean (standard deviation) ARWMC total score in the PSP cohort was 4.66 (3.25). Any degree of WMH was present in 68% of PSP (ARWMC +). Compared to ARWMC-, ARWMC + did not have greater disease severity or more cardiovascular risk factors. WMH were frequently localized in fronto-parietal lobes and were mild in severity. Linear regression analysis showed that ARWMC total score was related to the PSP-rating scale, irrespective of age, disease duration and the Charlson modified comorbidity index. Logistic regression analysis confirmed that ARWMC total score was related to the use of wheelchair, irrespective of above-mentioned covariates. Vascular risk factors as well as severity and distribution of WMH did not have an impact on the PSP phenotype. No differences were found with PD patients. Our results suggest that WMH in PSP might be markers of neurodegenerative-related pathology rather than being simple expression of atherosclerotic cerebrovascular changes.

Open access funding provided by Università degli Studi di Salerno within the CRUI-CARE Agreement. This work was supported by the University of Salerno FARB2022. Dr Marina Picillo is supported by the Michael J Fox Foundation for Parkinson’s research, the Italian Ministry of Health and Fondazione della Società Italiana di Neurologia and received speaker honoraria by Italfarmaco; Prof Paolo Barone received consultancies as a member of the advisory board for Zambon, Lundbeck, UCB, Chiesi, Abbvie and Acorda; the other authors report no financial disclosures.