Impact of MRI markers in subcortical vascular dementia: a multi-modal analysis in CADASIL

Anand Viswanathan1, Ophelia Godin, Eric Jouvent

  • 1Department of Neurology, CHU Lariboisière, Assistance Publique des Hôpitaux de Paris, Paris, France.

Neurobiology of Aging
|October 18, 2008
PubMed

Insights

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is linked to brain atrophy. Brain parenchymal fraction (BPF) significantly impacts cognitive function and disability in CADASIL patients.

Area of Science:

  • Neurology
  • Genetics
  • Radiology

Background:

  • CADASIL is a genetic arteriopathy caused by NOTCH3 gene mutations.
  • MRI reveals white matter hyperintensities (WMH), lacunar lesions (LL), cerebral microhemorrhages (CM), and brain atrophy in CADASIL.
  • Understanding the impact of these MRI markers on clinical outcomes is crucial.

Purpose of the Study:

  • To investigate the relative impact of lesion burden and location of MRI markers on cognitive impairment and disability in CADASIL.
  • To determine the independent influence of various MRI markers on clinical outcomes.

Main Methods:

  • Multi-modal MRI was performed on 147 patients with CADASIL.
  • Quantified lesion burden (LL, WMH, CM) and brain atrophy (brain parenchymal fraction - BPF).
  • Measured whole-brain mean apparent diffusion coefficient (mean-ADC).

Main Results:

  • Lacunar lesions (LL), mean-ADC, and BPF independently influenced cognitive function and disability.
  • Brain parenchymal fraction (BPF) explained the largest variation in cognitive and disability scores (35-38%).
  • Brain atrophy demonstrated the strongest independent influence on clinical impairment.

Conclusions:

  • Brain atrophy, specifically measured by BPF, plays a principal role in clinical impairment in CADASIL.
  • Cerebral tissue loss is a key factor in this genetic model of subcortical ischemic vascular dementia.
  • Integrated multi-modal MRI analysis provides critical insights into CADASIL pathophysiology.