[The retinopathy of ophthalmoplegia plus (Kearn's syndrome)].
The retinopathy of ophthalmoplegia plus (Kearns syndrome) is characterised by an extreme variability and a relative mildness of the visual disturbances, contrasting with the gravity of the ophthalmoscopic lesions. A study of three new cases, as well as an analysis of the literature, tend to demonstrate that the retinal lesions are mainly localised in the pigment-epithelial cells (mitochondrial anomalies?). This peculiar pathogenesis could explain why the retinopathy in this syndrome differs from classical pigmentary retinopathy.
D000293:Adolescent; D000328:Adult; D002648:Child; D003623:Dark Adaptation; D004585:Electrooculography; D004596:Electroretinography; D005260:Female; D005451:Fluorescein Angiography; D006801:Humans; D007625:Kearns-Sayre Syndrome / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology; D008297:Male; D008875:Middle Aged; D008928:Mitochondria / Q000648:ultrastructure; D009886:Ophthalmoplegia / Q000175:diagnosis*; D010857:Pigment Epithelium of Eye / Q000648:ultrastructure; D012160:Retina / Q000473:pathology; D014794:Visual Fields
La rétinopathie de l'ophtalmoplégie plus ou syndrome de Kearns.