Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy: decrease in regional
R Bruening1, M Dichgans, C Berchtenbreiter
1Institute of Clinical Radiology, Klinikum Grossadern, Ludwig-Maximilians University, Marchioninstrasse 15, D-81377 Munich, Germany.
Insights
Regional cerebral blood volume (rCBV) is reduced in the white matter of individuals with Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL). This decrease in rCBV correlates with cognitive impairment and disability.
Area of Science:
- Neurology
- Radiology
- Genetics
Background:
- Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) is a genetic arteriopathic syndrome.
- Characteristic white matter changes are visible on T2-weighted MRI in CADASIL patients.
- The genetic defect is linked to chromosome 19.
Purpose of the Study:
- To measure regional cerebral blood volume (rCBV) changes in CADASIL patients using dynamic contrast-enhanced MRI.
- To correlate these rCBV changes with patient disability and cognitive performance.
Main Methods:
- rCBV was measured in 24 individuals with confirmed CADASIL using a 1.5-T MRI unit.
- Susceptibility-weighted imaging and bolus tracking were employed.
- Absolute rCBV values were calculated using indicator dilution principles.
- Disability was assessed using the Rankin scale, and cognitive function with the Mini-Mental State Examination.
Main Results:
- Mean rCBV was significantly lower in T2-hyperintense subcortical white matter (2.7 mL/100 g) compared to normal-appearing white matter (4.4 mL/100 g) (P < .05).
- Gray matter rCBV was within the normal range (8.3 mL/100 g).
- Lower rCBV in hyperintense white matter correlated with cognitive impairment and disability (P < .05).
- No correlation was found between rCBV and age.
Conclusions:
- rCBV is decreased in the affected subcortical white matter of CADASIL patients.
- Reduced rCBV in CADASIL is inversely correlated with disability and cognitive impairment.
Background And Purpose:
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an arteriopathic syndrome related to a genetic defect on chromosome 19. Characteristic changes in CADASIL can be observed onT2-weighted MR images in the subcortical white matter. The purpose of this study was to measure changes of regional cerebral blood volume (rCBV) with dynamic contrast-enhanced MR imaging and to correlate the changes to disability and cognitive performance.
Methods:
We obtained rCBV measurements of 24 individuals with proven CADASIL on a 1.5-T MR imaging unit. A susceptibility-weighted MR imaging sequence was used for bolus tracking. Principles of the indicator dilution theory were applied to estimate values of absolute rCBV (mL/100 g). Disability was determined by using the Rankin scale, and overall cognitive performance was assessed by using the Mini-Mental State Examination.
Results:
The mean rCBV in the subcortical white matter that was hyperintense on the T2-weighted images (2.7 +/- 0.8 mL/100 g) was significantly lower than the rCBV in the white matter that appeared normal on the T2-weighted images (4.4 +/- 1.3 mL/100 g) (P <.05). The mean rCBV in the gray matter was within the normal range (8.3 +/- 1.7 mL/100 g). Both cognitive impairment and disability negatively correlated with rCBV in the subcortical white matter that was hyperintense (P <.05) but not with rCBV in the normal appearing white matter. rCBV did not correlate with age.
Conclusion:
rCBV measured in the hyperintense subcortical white matter in individuals with CADASIL was decreased and inversely correlated with disability and cognitive impairment.
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