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Retinal vasculopathy and cerebral leukodystrophy

Synonym(s) RVCL
Prevalence Unknown
Inheritance Autosomal dominant
Age of onset -
ICD-10 -
MeSH -
MedDRA -


Disease definition

Retinal vasculopathy and cerebral leukodystrophy (RVCL) is 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; see these terms); all exhibiting progressive visual impairment as well as variable cerebral dysfunction.

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