François J
Klin Monbl Augenheilkd. 1979 Dec;175(6):715-34.
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.
在各种青少年黄斑变性中，可以区分出主要累及以下部位的类型：1. 神经上皮：斯塔加特病；显性青少年变性；中心或中心周围色素性视网膜病变、视锥细胞进行性营养不良和黄斑囊样水肿。2. 色素上皮：黄斑卵黄样变性、眼底黄斑病变、干燥综合征网状色素性营养不良、黄斑蝴蝶状色素营养不良和黄斑区色素沉着。3. 布鲁赫膜：玻璃膜透明变性或玻璃膜疣以及显性进行性黄斑中心凹营养不良。4. 脉络膜：脉络膜中央晕状萎缩和索斯比假炎症性营养不良。——所有这些黄斑遗传性变性的特点是双侧对称。对于诊断和鉴别诊断，不仅眼底镜检查结果很重要，视觉功能检查也很重要，其中必须包括视网膜电图和眼电图检查，以及对受累家庭进行基因研究。
