The main pathologic process centers on small blood vessels that prematurely “drop out” and disappear. The retina of the eye and white matter of the brain appear to be among the most sensitive to this pathologic process. Over a five to ten-year period, this<unk>  (blood vessel pathology) results in vision loss and destructive brain lesions with neurologic deficits and death. Although brain and eye disease are universally present in patients with RVCL, the disease is truly a multi-system disorder characterized by chronic kidney disease, liver disease, and frequently other<unk>  including gastrointestinal disease, osteonecrosis, and hypothyroidism.
Retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations
Multifocal choroiditis and panuveitis
Diffuse unilateral subacute neuroretinitis
Cerebroretinal microangiopathy with calcifications and cysts
Uveitis–Glaucoma–Hyphema syndrome
Chronic relapsing inflammatory optic neuropathy
Acute posterior multifocal placoid pigment epitheliopathy
Late congenital syphilitic oculopathy