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White Matter Hyperintensities of the Corpus Callosum Are Associated With Clinical Severity in CADASIL
Elisa Ouin1, Ana Dimitrovic2, Lina Grosset2,3
1Department of Neurology, Amiens University Hospital, France (E.O.).
Insights
White matter hyperintensities in the corpus callosum (WMHCC) in Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy (CADASIL) are linked to secondary degeneration. These WMHCC correlate with worse cognitive function and disability in patients.
Area of Science:
- Neurology
- Neuroimaging
- Genetics
Background:
- Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy (CADASIL) clinical severity doesn't correlate with overall white matter hyperintensities (WMH) burden.
- This suggests heterogeneous tissue alterations underlie WMH in CADASIL.
Purpose of the Study:
- Investigate if WMH in the corpus callosum (WMHCC) result from secondary degeneration.
- Determine the relationship between WMHCC and clinical severity in CADASIL patients.
Main Methods:
- Analyzed 3D FLAIR MRI data from 228 CADASIL patients.
- Blindly assessed WMHCC and lacunes for signs of secondary degeneration.
- Correlated WMHCC with Mattis Dementia Rating Scale and modified Rankin Scale scores.
Main Results:
- WMHCC were present in 46% of patients, often crossing nearby lacunes (74%).
- Patients with WMHCC showed significantly worse cognitive performance and higher disability scores.
- WMHCC were independently associated with poorer cognitive function after adjustments.
Conclusions:
- WMHCC in CADASIL are likely due to secondary degeneration.
- WMHCC are independently associated with clinical severity, unlike the total WMH burden.
Background:
In CADASIL (Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy), clinical severity is not related to the total burden of white matter hyperintensities (WMHs), presumably because of heterogeneous underlying tissue alterations. We aimed to investigate whether WMHs in the corpus callosum (WMHCC) are due to secondary degeneration and related to clinical severity.
Methods:
We evaluated data from 228 CADASIL patients included in an ongoing prospective cohort with available 3-dimensional fluid-attenuated inversion recovery magnetic resonance imaging sequences. We analyzed in a blind manner WMHCC and lacunes in presumably connected areas to determine whether WMHCC are related to secondary degeneration. We evaluated the links between WMHCC and the Mattis dementia rating scale and the modified Rankin Scale-widely used measures of global cognitive performances and disability, respectively. Linear regression models were adjusted for age, sex, level of education, brain volume, number of lacunes, and volume of WMH.
Results:
Among 228 patients, only 105 (46%) had WMHCC while all had WMH in the rest of the white matter. In 74% of cases, WMHCC crossed a presumably connected nearby lacune, which was significantly higher than the expected value if the spatial distributions of WMHCC and nearby lacunes were unrelated (11%; P<0.001). Patients with WMHCC had worse Mattis dementia rating scale (median [P25-P75], 138 [122-142] versus 143 [140-143]; P<0.001) and worse modified Rankin Scale (2 [1-3] versus 1 [0-1]; P<0.001). In adjusted models, Mattis dementia rating scale was significantly associated with WMHCC (estimate, -6.2 [95% CI, -11.8 to -0.1]).
Conclusions:
In CADASIL, WMHCC are likely related to secondary degeneration and are independently related to clinical severity, in contrast to the total burden of WMH.
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