Nakamura M, Sekiya Y, Yamamoto M
Department of Ophthalmology, Kobe University School of Medicine, Japan.
Invest Ophthalmol Vis Sci. 1996 Dec;37(13):2736-43.
To examine whether the early response of photic blink reflex (PBR) is spared in patients with Leber's hereditary optic neuropathy (LHON).
Twenty-six patients with bilateral optic neuropathy (visual acuity < or = 0.1) and central scotomata were divided into LHON group with one of three mitochondrial DNA mutations at nucleotide position of 3460, 11778, or 14484 and non-LHON group without them. Latencies of the PBR early response and those of the electrically evoked blink reflex (EBR) response were compared among the 26 patients and 20 healthy volunteers.
In controls, average latency of the PBR response was 50.3 +/- 2.4 msec and was in accordance with previous reports. Ten of eleven patients with LHON had normal PBR responses, whereas 12 of 15 controls without LHON had abnormal responses (P = 0.0005). Latencies of EBR responses were normal in all but one patient with LHON.
The afferent fibers of the PBR early response, as well as those of the light reaction, are reported to terminate presumably in the pretectum. Together with the reported evidence of the preserved light reaction in patients with LHON, we presume that W retinal ganglion cells may project common afferent fibers of these two neuronal pathways and may be preferentially spared in patients with LHON.
研究莱伯遗传性视神经病变（LHON）患者的光性眨眼反射（PBR）早期反应是否保留。
26例双侧视神经病变（视力≤0.1）且有中心暗点的患者被分为LHON组（在核苷酸位置3460、11778或14484处存在三种线粒体DNA突变之一）和无这些突变的非LHON组。比较了这26例患者和20名健康志愿者的PBR早期反应潜伏期以及电诱发眨眼反射（EBR）反应潜伏期。
在对照组中，PBR反应的平均潜伏期为50.3±2.4毫秒，与先前报道一致。11例LHON患者中有10例PBR反应正常，而15例非LHON对照组中有12例反应异常（P = 0.0005）。除1例LHON患者外，所有患者的EBR反应潜伏期均正常。
据报道，PBR早期反应的传入纤维以及光反应的传入纤维可能终止于顶盖前区。结合LHON患者保留光反应的报道证据，我们推测W视网膜神经节细胞可能投射这两条神经元通路的共同传入纤维，并且在LHON患者中可能优先保留。
