Retinal arterial abnormalities correlate with brain white matter lesions in cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy

Purpose: The aim of this study is to determine the relationship between retinal abnormalities and brain white matter hyper-intensities (WMH) in symptomatic patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL). Methods: Fifteen patients with CADASIL and fifteen age-matched healthy control individuals were enrolled in this study. Ophthalmological examinations were performed on all subjects and all patients with CADASIL underwent cerebral MRI scanning. Retinal artery abnormalities were graded according to the Keith-Wagener-Barker hypertensive retinopathy classification. WMH were classified as punctuate (Grade 1), nodular or early confluent (Grade 2) and diffusly confluent (Grade 3). Partial correlation was used to assess the relationship between retinal abnormalities and WMH, controlling for age. Results: Retinal arteriole narrowing existed in 15 cases and 2 controls, with Grade 2 and Grade 3 being the most common grading of retinal artery narrowing in patients with CADASIL. All patients had WMH on MRI, rated as Grade 1 in one patient, Grade 2 in five patients and Grade 3 in nine patients. The correlation coefficient of retinal arteriole narrowing and WMH was 0.546 (P < 0.05). Conclusion: Retinal arteriole narrowing may be associated with the severity of the cerebral lesions in CADASIL.