A new autosomal dominant vascular retinopathy syndrome

We describe a new syndrome with autosomal dominant transmission whose most striking feature is vascular retinopathy. The retinopathy is often associated with migraine, Raynaud's phenomenon and mental changes, mainly forgetfulness, aggression and depression. To define this syndrome we collected...

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Bibliographic Details
Published inEuropean journal of ophthalmology Vol. 1; no. 2; p. 73
Main Authors Storimans, C W, Van Schooneveld, M J, Oosterhuis, J A, Bos, P J
Format Journal Article
LanguageEnglish
Published United States 01.04.1991
Subjects
Adult
Aged
Female
Fluorescein Angiography
Fundus Oculi
Humans
Male
Middle Aged
Migraine Disorders - genetics
Pedigree
Raynaud Disease - genetics
Retinal Diseases - genetics
Retinal Neovascularization - genetics
Retinal Vessels
Syndrome
Visual Acuity
Vitreous Hemorrhage - genetics
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Summary:We describe a new syndrome with autosomal dominant transmission whose most striking feature is vascular retinopathy. The retinopathy is often associated with migraine, Raynaud's phenomenon and mental changes, mainly forgetfulness, aggression and depression. To define this syndrome we collected medical data on 110 family members. General ophthalmological examination and fluorescein angiography were performed in 61 persons. The retinopathy, as diagnosed in 22 persons, is characterized by central and peripheral microangiopathy, areas of capillary non-perfusion, haemorrhages, cotton wool spots and, in a more advanced stage, occlusion of large retinal vessels, which can induce a neovascular response. A vascular occlusive disorder may be the common aetiological factor of the various manifestation of this syndrome.
ISSN:1120-6721
DOI:10.1177/112067219100100204