LOSS OF ELECTRORETINOGRAPHIC OSCILLATORY POTENTIALS, OPTIC ATROPHY, AND DYSPLASIA IN CONGENITAL STATIONARY NIGHT BLINDNESS
LOSS OF ELECTRORETINOGRAPHIC OSCILLATORY POTENTIALS, OPTIC ATROPHY, AND DYSPLASIA IN CONGENITAL STATIONARY NIGHT BLINDNESS
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DOI：
10.1016/s0002-9394(14)77917-6
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发表时间：
1983-01-01
影响因子：
4.2
通讯作者：
ROSENBAUM, AL
中科院分区：
文献类型：
--
作者：
HECKENLIVELY, JR;MARTIN, DA;ROSENBAUM, AL
诊断先天性静止性夜盲症的新标准包括明视和亮闪光暗适应视网膜电图中振荡电位的丢失，以及视神经乳头的萎缩或发育不良变化，或两者兼而有之。10例患者（7男3女，年龄6-19岁）有典型的先天性静止性夜盲表现，包括先天性非进行性夜盲，无色素性视网膜病变和符合近视的全视野。视力范围为20/30至20/60，但1例患者的视力为20/200。大多数患者有斜视史。明视视网膜电图低于正常。在男性患者中，5例视盘倾斜伴颞部神经缺失，2例神经头畸形。3例女性患者视盘苍白，无倾斜迹象。
New criteria for diagnosing congenital stationary night blindness include loss of the oscillatory potentials in the photopic and bright-flash dark-adapted electroretinogram, and atrophy or dysplastic changes, or both, in the optic nerve head. Ten patients (7 male and 3 female, ranging in age from 6-19 yr) had typical findings of congenital stationary night blindness including congenital nonprogressive nyctalopia, no pigmentary retinopathy and full visual fields consistent with myopia. Visual acuities ranged from 20/30 to 20/60, though 1 patient had a visual acuity of 20/200. Most patients had histories of strabismus. The photopic electroretinograms were subnormal. Of the male patients, 5 had tilted optic disks with temporal portions of the nerve missing and 2 had misshapen nerve heads. The 3 female patients had pallor of the optic disk without evidence of tilt.