Neuroimaging Biomarkers of Disease Progression and Cognitive Change in Patients With Retinal Vasculopathy With Cerebral Leukoencephalopathy - PubMed
a day ago
- #Cerebral Small Vessel Disease
- #Cognitive Decline
- #Neuroimaging
- The study focuses on retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations (RVCL-S), a monogenic, age-related cerebral small vessel disease (cSVD) leading to vascular dementia, vision loss, and premature death.
- Researchers examined the natural history of RVCL-S progression using neuroimaging and cognitive endpoints in a prospective cohort, hypothesizing that cerebral blood flow (CBF) and oxygen extraction fraction (OEF) would correlate with disease progression.
- Sequential brain MRI and cognitive tests were conducted on 25 participants aged 23-68 years over a median of 2.2 years, tracking neuroimaging and cognitive changes.
- Key findings include significant progression in white matter hyperintensity (WMH) volume (+31.3%/year) and declines in cognitive functions, particularly processing speed (DSST), free recall, and category fluency.
- OEF, but not CBF, was independently associated with WMH growth and changes in white matter microstructure, suggesting tissue hypoxia-ischemia plays a role in RVCL-S pathophysiology.
- The study suggests cerebral OEF could serve as a predictive biomarker for RVCL-S and other cSVD forms, aiding in patient risk stratification.