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Published on: November 6, 2017
Retinal vascular density in CADASIL (Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and
Valérie Krivosic1, Michel Paques2, Dominique Hervé3
1Ophtalmology, APHP Hopital Lariboiisiere, Paris, France.
Insights
Retinal vascular density (VD) is reduced in Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) patients. This reduction progresses with age but does not correlate with disease severity or imaging markers.
Area of Science:
- Neuro-ophthalmology
- Vascular Neurology
- Medical Imaging
Background:
- Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) is a genetic cerebrovascular disorder.
- Retinal vascular density (VD) measured by optical coherence tomography angiography (OCTA) is a potential indicator of cerebrovascular health.
Purpose of the Study:
- To investigate the relationship between retinal vascular density (VD) and clinical/imaging manifestations in CADASIL patients.
Main Methods:
- Optical coherence tomography angiography (OCTA) was used to assess retinal VD in 104 CADASIL patients and 83 healthy controls.
- Clinical assessments included stroke history, modified Rankin Scale, and Mini-Mental Status Examination.
- Brain MRI was performed to evaluate lesion load.
Main Results:
- Retinal VD decreased with age in both patients and controls.
- CADASIL patients exhibited significantly lower retinal VD compared to controls, even after age adjustment.
- No significant association was found between retinal VD and stroke history, functional scores, or MRI-detected white matter lesions.
Conclusions:
- Retinal vascular density is reduced in CADASIL patients and declines with age.
- Retinal VD does not appear to be a reliable biomarker for the severity of clinical or imaging features in CADASIL.
Background And Objective:
Retinal vascular density (VD) measured using optical coherence tomography with angiography (OCTA) has been suggested as a potential marker of intracerebral vascular changes in Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL). We aimed to determine whether VD is related to the clinical and imaging manifestations of the disease.
Methods:
OCTA was performed in 104 CADASIL patients (parallel to their clinical and imaging assessment) and in 83 healthy individuals.
Results:
A significant reduction of VD related to age was detected in patients and controls in the superficial and deep vascular plexus of the whole foveal or parafoveal retinal area (p<0.0001). After adjustment for age, these parameters were found significantly lower in patients than in controls (p<0.03). Multivariable analysis did not show any association between retinal VD and history of stroke, modified Rankin Scale or Mini-Mental Status Examination scores. No significant association was found with MRI lesions either.
Conclusion:
In CADASIL, retinal VD is decreased early and progresses with ageing but does not appear related to the severity of clinical or imaging manifestations.

