Mayer D L, Fulton A B, Hansen R M
Ophthalmic Paediatr Genet. 1985 Feb;5(1-2):51-6. doi: 10.3109/13816818509007855.
Visual acuity for gratings was studied in 18 infants and children with generalized retinal degenerations using preferential looking (PL) procedures. Diagnoses were Leber's congenital amaurosis (12), Laurence-Moon-Bardet-Biedl-like syndromes (4) and metabolic disorders (2). ERG's were extinguished in 11 patients and much attenuated in seven patients. Acuities at all ages (two months to 12 years) were significantly poorer than normal, and patients with extinguished ERG's had the poorest acuity. Neurological abnormality or mental retardation, present in ten patients, was as likely in patients with 6/60 or poorer grating acuity as in patients with better than 6/60 acuity. A comparison group of 12 infants and children with oculocutaneous albinism showed significantly better grating acuities than the patients with retinal degeneration. Relatively good grating acuity in infants with no anatomic fovea (oculocutaneous albinism) and much poorer acuities of infants with generalized retinal degeneration suggest that parafoveal or peripheral retina is necessary and sufficient for normal, behaviorally-obtained grating acuity in infancy.
使用优先注视（PL）程序，对18名患有广泛性视网膜变性的婴幼儿和儿童的光栅视力进行了研究。诊断结果为莱伯先天性黑矇（12例）、劳伦斯-穆恩-巴德-比德尔样综合征（4例）和代谢紊乱（2例）。11例患者的视网膜电图（ERG）熄灭，7例患者的ERG明显减弱。所有年龄段（2个月至12岁）的视力均显著低于正常水平，ERG熄灭的患者视力最差。10例患者存在神经异常或智力迟钝，光栅视力为6/60或更差的患者与视力优于6/60的患者出现神经异常或智力迟钝的可能性相同。一组由12名患有眼皮肤白化病的婴幼儿和儿童组成的对照组，其光栅视力明显优于视网膜变性患者。没有解剖学上的中央凹的婴儿（眼皮肤白化病）的光栅视力相对较好，而患有广泛性视网膜变性的婴儿的视力则差得多，这表明在婴儿期，旁中央凹或周边视网膜对于通过行为获得的正常光栅视力是必要且充分的。
