Changes in the blood–brain barrier status closely correlate with the rate of disease progression in patients with multiple system atrophy: A longitudinal study

Abstract

BACKGROUND: The pathomechanisms responsible for disease progression in multiple system atrophy are unknown. The blood-brain barrier status may act as a modifier of disease progression in neurodegenerative diseases. METHODS: We evaluated the 12-month longitudinal change of the blood-brain barrier in 16 multiple system atrophy patients and analyzed its correlation with changes in clinical severity. RESULTS: The baseline blood-brain barrier index did not correlate significantly with change in disease severity. However, changes in the blood-brain barrier indices over 12 months had significant positive correlations with changes in total unified multiple system atrophy rating scale (r = 0.56, p = 0.024) and part II scores (r = 0.56, p = 0.025). These correlation coefficients were higher after adjusting for baseline neurological deficits. CONCLUSIONS: These data suggest that changes in the blood-brain barrier status are closely coupled with the rate of disease progression in multiple system atrophy, potentially acting as a contributor to disease progression.