/retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations

retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations

Rare Disease

MONDO:0008641

Also Known As

RVCLRVCL-Shereditary vascular retinopathyretinal vasculopathy and cerebral leukoencephalopathyvasculopathy, retinal, with cerebral leukoencephalopathy and systemic manifestationsADRVCLCRVHVRautosomal dominant retinal vasculopathy with cerebral leukodystrophycerebroretinal vasculopathycerebroretinal vasculopathy, hereditarygrand Kaine fulling syndromegrand-Kaine-fulling syndromeretinal vasculopathy with cerebral leukodystrophyretinopathy, vascular, with cerebral and renal involvement and Raynaud and migraine phenomenavasculopathy, retinal, with cerebral leukodystrophy

Definition

An inherited group of small vessel diseases comprised of cerebroretinal vasculopathy (CRV), hereditary vascular retinopathy (HRV) and hereditary endotheliopathy with retinopathy, nephropathy and stroke (HERNS); all exhibiting progressive visual impairment as well as variable cerebral dysfunction.