Effects of different regional cerebral blood flow on white matter hyperintensity in CADASIL patients

Abstract

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an early-onset inherited small vessel disease. Decreased cerebral blood flow (CBF) may contribute to white matter hyperintensity (WMH) severity in CADASIL, but more evidence is needed to support this hypothesis. This study comprised six patients with CADASIL who harbored mutations in the coding sequence of NOTCH3 and twelve age-matched neurologically healthy controls. We collected clinical and imaging data from patients with CADASIL and divided the brain into four regions: WMH, normal-appearing white matter (NAWM), gray matter (GM), and global brain. We analyzed the relationship between CBF of each region and the WMH volume. Compared with the control group, CBF was significantly decreased in all four regions in the CADASIL group. Lower CBF in these regions was correlated with higher WMH volume in CADASIL. CBF in the NAWM, GM and global regions was positively correlated with that in WMH region. However, after correction tests, only CBF in the WMH region but not in NAWM, GM and global regions was associated with WMH volume. Our findings suggest that CBF in the WMH region is an influencing factor of the WMH severity in CADASIL.

A multicenter, single-arm, phase II clinical trial of adrenomedullin in patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy

“…Importantly, the WM lesions are not pathologically uniform conditions but rather diverse abnormalities in the brain [2] . Reduced cerebral blood flow (CBF) is a contributing factor to the development of WM lesions [3] . Furthermore, disruptions in the differentiation of oligodendrocyte precursor cells (OPCs) into mature oligodendrocytes also play a role in the formation of WM lesions.…”

Editorial commentary on the special issue of glia and neurological diseases

“…Wang et al reported a clinical study of six patients of CADASIL and twelve healthy controls, they found that the severity of hypoperfusion correlates with the white matter hyperintensity volume. It highlighted the mutual links between the elements of the glio-vascular unit (G-unit) [ 8 ] . We hope the readers find this special issue interesting and intriguing.…”