Nickel B, Hoyt C S
Arch Ophthalmol. 1982 Jul;100(7):1089-92. doi: 10.1001/archopht.1982.01030040067010.
Thirty-one patients had Leber's congenital amaurosis. Only one was severely retarded, and three demonstrated hypoplasia of the cerebellar vermis on computed tomographic scanning. These findings are in contrast to those of previous investigators, who have emphasized the high incidence of psychomotor retardation associated with Leber's amaurosis. Much of the psychomotor retardation that has been reported is probably secondary to the sensory deprivation and not necessarily a sign of structural CNS dysfunction. The diagnosis of Leber's congenital amaurosis does not, therefore, portend severe intellectual impairment and poor educability.
31名患者患有莱伯先天性黑蒙。只有1名患者严重智力发育迟缓，3名患者在计算机断层扫描中显示小脑蚓部发育不全。这些发现与之前研究者的结果形成对比，他们强调莱伯黑蒙相关的精神运动发育迟缓发生率很高。之前报道的许多精神运动发育迟缓可能继发于感觉剥夺，不一定是中枢神经系统结构功能障碍的迹象。因此，莱伯先天性黑蒙的诊断并不预示着严重的智力损害和教育能力差。
