[Juvenile macula degeneration]. 1979

J François

Among the various forms of juvenile macular degeneration, one can distinguish those that primarily involve: 1. The neuro-epithelium: Stargardt's disease; dominant juvenile degeneration; central or pericentral pigmentary retinopathy, progressive dystrophy of the cones and cystoid macular oedema. 2. The pigment epithelium: vitelliform degeneration of the macula, fundus flavimaculatus, Sjögren's reticulated pigmentary dystrophy, butterfly shaped pigment dystrophy of the macula and grouped pigmentation of the macular region. 3. Bruch's membrane: the hyaline dystrophies or drusen of the lamina vitrea and dominant progressive foveal dystrophy. 4. The choroid: central areolar atrophy of the choroid and Sorsby's pseudo-inflammatory dystrophy. -- All these macular heredo-degenerations are characterized by the fact that they are bilateral and symmetrical. For diagnosis and differential diagnosis not only the ophthalmoscopic appearance is important, but also the examination of the visual functions, which must include an electroretinographic and electro-oculographic examination, as well as the genetic study of the affected families.

UI MeSH Term Description Entries
D008268 Macular Degeneration Degenerative changes in the RETINA usually of older adults which results in a loss of vision in the center of the visual field (the MACULA LUTEA) because of damage to the retina. It occurs in dry and wet forms. Maculopathy,Maculopathy, Age-Related,Age-Related Macular Degeneration,Age-Related Maculopathies,Age-Related Maculopathy,Macular Degeneration, Age-Related,Macular Dystrophy,Maculopathies, Age-Related,Age Related Macular Degeneration,Age Related Maculopathies,Age Related Maculopathy,Age-Related Macular Degenerations,Degeneration, Macular,Dystrophy, Macular,Macular Degeneration, Age Related,Macular Degenerations,Macular Dystrophies,Maculopathies,Maculopathy, Age Related
D009887 Ophthalmoscopy Examination of the interior of the eye with an ophthalmoscope. Ophthalmoscopies
D010857 Pigment Epithelium of Eye The layer of pigment-containing epithelial cells in the RETINA; the CILIARY BODY; and the IRIS in the eye. Eye Pigment Epithelium
D012160 Retina The ten-layered nervous tissue membrane of the eye. It is continuous with the OPTIC NERVE and receives images of external objects and transmits visual impulses to the brain. Its outer surface is in contact with the CHOROID and the inner surface with the VITREOUS BODY. The outer-most layer is pigmented, whereas the inner nine layers are transparent. Ora Serrata
D012162 Retinal Degeneration A retrogressive pathological change in the retina, focal or generalized, caused by genetic defects, inflammation, trauma, vascular disease, or aging. Degeneration affecting predominantly the macula lutea of the retina is MACULAR DEGENERATION. (Newell, Ophthalmology: Principles and Concepts, 7th ed, p304) Degeneration, Retinal,Degenerations, Retinal,Retinal Degenerations
D002829 Choroid The thin, highly vascular membrane covering most of the posterior of the eye between the RETINA and SCLERA. Choriocapillaris,Haller Layer,Haller's Layer,Sattler Layer,Sattler's Layer,Choroids
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D012174 Retinitis Pigmentosa Hereditary, progressive degeneration of the retina due to death of ROD PHOTORECEPTORS initially and subsequent death of CONE PHOTORECEPTORS. It is characterized by deposition of pigment in the retina. Pigmentary Retinopathy,Tapetoretinal Degeneration,Pigmentary Retinopathies,Retinopathies, Pigmentary,Retinopathy, Pigmentary,Tapetoretinal Degenerations

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