PURPOSE:To study the retinal pathologic changes and pathogenesis of relinhis pigmenlosa(RP). METHODS:The relina from a patient with autosomal dominant RP was examined by light and electron microscopy. RESULTS:Degeneration and structure disturbance almost involved in every layer of retina and were accompanied hy regional differenecs:Posterior region was more than periphery one in severity. Degeneration of retinal pigment epithelium(RPE)closely eorrelaled to that of the phmoreceplor. The uhraslrneture of the retina showed extensive and severe degeneration in the photoreeeptors ,particularly ill omer segments and mitoehondrlas. Lipofusein gramdes were accumuhtted in the cytoplagm. CONClUSIONS:These changes suggested that self-energizing system and self engulfing system of the photoreceptols were disfunctloned. （Chin J Ocul Fundus Dis,1997,13: 24-26）
ObjectiveTo use flash electroretinogram (F-ERG) and optical coherence tomography (OCT) to examine patients with primary retinitis pigmentosa (RP), and analyze the specificity of the disease on F-ERG and OCT. MethodsThirty-seven patients (74 eyes) diagnosed with primary retinitis pigmentosa in the Department of Ophthalmology, West China Hospital between September 2013 to October 2014 and 38 normal volunteers (76 eyes) were included in this study. F-ERG and OCT examinations were performed on all the patients. Then, we analyzed the differences between the two groups of subjects. ResultsFor RP patients undergoing P-ERG examination with the dark adaptation of 0.01 ERG, the latency of b wave was (73.24±6.42) ms and the amplitude of b wave was (22.87±22.48) μV; when dark adaptation of 3.0 ERG was adopted, the latency of a wave was (24.57±6.30) ms, the amplitude of a wave was (35.45±25.54) μV, the latency of b wave was (48.19±8.18) ms, and the amplitude of b wave was (119.47±50.89) μV; with the light adaptation of 3.0 ERG, the latency of a wave was (21.01±4.86) ms, the amplitude of a wave was (12.59±13.43) μV, the latency of b wave was (38.43±5.00) ms, and the amplitude of b wave was (27.19±38.12) μV. For normal volunteers undergoing F-ERG examination with the dark adaptation of 0.01 ERG, the latency of b wave was (72.63±3.49) ms and the amplitude of b wave was (86.36±21.57) μV; when the dark adaptation was 3.0 ERG, the latency of a wave was (22.88±1.62) ms, the amplitude of a wave was (210.74±43.57) μV, the latency of b wave was (42.59±2.60) ms, and the amplitude of b wave was (398.29±62.42) μV; when the light adaptation of 3.0 ERG was adopted, the latency of a wave was (16.61±0.87) ms, the amplitude of a wave was (54.26±19.64) μV, the latency of b wave was (33.29±1.11) ms, and the amplitude of b wave was (176.98±63.44) μV. There were no significant differences between the two groups when dark adaptation ERG was 0.01 (P=0.48), but for other adaptations, there were significant differences in the latency and amplitude of a and b wave between the two groups (P<0.05). The results of OCT showed that the retinal thickness of the RP patients with a range of 1 mm diameter centered on macular center concave was (218.66±74.14) mm, 3 mm diameter was (275.03±47.85) mm, and 6 mm diameter was (247.37±46.44) mm. For normal volunteers, OCT showed that the retinal thickness with a 1 mm range centered on macular center concave was (250.38±15.79) mm, 3 mm was (323.64±17.26) mm, and 6 mm was (283.44±12.50) mm. The differences between the two groups were statistically significant for each range (P<0.01). ConclusionFor patients with RP, F-ERG shows latency delay and amplitude decrease for each response, while OCT displays a thinning thickness of macular fovea. Therefore, F-ERG and OCT can not only effectively evaluate the functions of macular and the surrounding retina, but can also be used as an effective method for the diagnosis of RP.