Hitomi E, Simpkins AN, Luby M, Latour LL, Leigh RJ, Leigh R. Blood-ocular barrier disruption in patients with acute stroke. Neurology. 2018;90:e915–e23.です。
In conclusion, in patients with acute ischemic stroke due to ICA stenosis or occlusion, GLOS(Gadolinium Leakage in Ocular Structures) is a frequent finding, commonly unilateral or bilateral asymmetrical and in a subset of patients associated with pre-existing signal abnormalities in the vitreous body. Asymmetrical appearance of GLOS might be caused by an insufficient collateral blood flow through the circle of Willis and collateral blood flow from the ECA resulting in a reversed blood flow in the central retinal artery and possibly development of venous stasis retinopathy.
In 2007, dominant heterozygous frameshift mutations in the C‑terminal end of TREX1 were detected in families with a fatal vascular disorder affecting the brain, retinas and kidneys. As a result, the clinical syndromes cerebroretinal vasculopathy (CRV), hereditary vascular retinopathy (HVR) and hereditary endotheliopathy, retinopathy, nephropathy and stroke (HERNS), which were previously thought to be distinct entities, were summarized under the term retinal vasculopathy with cerebral leukodystrophy. Recently the syndrome was renamed retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations (RVCL-S) in a consensus paper from 2016.
Periventricular patchy white matter lesions in the frontal lobes as well as rim-enhancing lesions with prolonged diffusion restriction and long-lasting contrast enhancement are characteristic imaging findings in RCVL-S and can be helpful in the differential diagnosis.