Objective To test the hypothesis that the macular pigment may be a marker of foveal cone function and consequently the structural integrity of foveal cones.Methods Sixteen patients (32 eyes) diagnosed to have Stargardt dystrophy and three patients with full thickness macular holes by clinical criteria were studied with a scanning laser ophthalmoscopy (SLO) comparing argon laser blue and infrared images for the presence or absence of macular pigment (MP) in the fovea. An C++ computer based program was used to evaluate the density of MP. Eyes were graded into three categories: those without foveal macular pigment, those with partial pigment and those with normal amounts of macular pigment. These categories were compared with visual acuity determined by the Snellen chart. Results Thirteen eyes with a visual acuity of 20/200 or worse had no macular pigment in the fovea. Eleven eyes with visual acuity of 20/40 or better had a normal amount of macular pigment in the fovea and 1 eye had partial macular pigment. Eleven eyes with partial macular pigment had intermediary acuity value.Conclusions Foveal macular pigment is closely related to foveal cone acuity and therefore may be a marker for the presence of foveal cones. Infrared light is a sensitive indicator of early macular diseases.(Chin J Ocul Fundus Dis，2003，19：201-268)
Purpose To observe the features of multi-focal electroretinogram (mERG) in Stargardtprime;s disease, and evaluate the validity of mE RG on diagnosis of this disease. Methods mERG had been recorded in 7 cases (14 eyes) of Stargardtprime;s disease with VERIS 4.0，and the findings were compared with normal individuals. Results The mERG were remarkably abnormal in all cases of the disease, as the amplitudes of N1 and P1 waves were seriously decreased and the latencies were prolonged in all the 6 regions (Plt;0.01). The degrees of the amplitude changes of N1, P1 waves were not the same in those areas,and the most decreased part was in fovea. The responses of N1, P1waves from the fovea of patients were about 19 and 10 percent respectively of which in controls. As the eccentricity enlarged, these differences had a tendency of reduction. The mERG topography of this disease could be divided to two types, i.e. central decreased and diffuse decreased types. Conclusion There are remarkably abnormalities on mERG in Stargardtprime;s disease,and the most damaged location of macular function is in fovea. (Chin J Ocul Fundus Dis, 2001,17:271-273)
Stargardt disease (STGD) is one of the most prevalent inherited macular dystrophy, and most often occurs in child or adolescence. Irreversible vision loss is observed in almost all cases. Type 1 (STGD1) is one of the most common type. It is an autosomal recessive condition, caused by mutations in the Abca4 gene. In recent years, encouraging progress has been made in the treatment of STGD1. C20-D3-retinyl acetate (ALK-001), fenretinide and ICR-14967 (A1120) as visual cycle modulators, StarGen as gene supplementation therapies, and the stem cell transplantation of human embryonic stem cell-derived retinal pigment epithelium cells are the most promising therapies. With the development of studies and clinical trials, the clinical application of various treatments of STGD1 are expected in the near feature, which are expected to save the vision of most patients.
Purpose To investigate the clinical characteristics of the Stargardt is disease and to evaluate the value of the fundus fluorescein angiography (ICGA) in Stargardt is disease. Methods Routine eye examination,VEP,ERG,EOG,and perimetry were performed in 25 cases(30 eyes) of Stargardtprime;s disease (15 men and 10 women) aged 12-41,on an average of 23.FFA was done in 15 cases (30eyes),and ICGA was done in 10 cases (20 eyes). Results Visual acuity was below 0.1 for 90.0% of the cases.Every eye showed macular lesions that wrere horizontal ellipse in shape,1.3-3.2PD in horizontal diameter and tinged with metal or beated copper color.Fundus flavimaculatus was found in 84.0% of the cases.On fluorescein angiography,window defect hyperfluorescence was found in the macular lesion of ocular fundus,with typical quot;bullprime;s eyequot; pattern.A quot; dark choroidquot; was revealed in 91.70% of the whole cases.On indocyanine green angigraphy,hypofluorescent area appeared at the macula in the early phase,even in very early stage of the Stargardtprime;s disease.Areas of fundus flavimaculatus were also hypoflurescent that became more apparent in the later phase. Conclusion Fluorescein angiography combined with indocyanine green angiography was useful for studying the clinical features and the pathological mechnism of the Stargardtprime;s disease. (Chin J Ocul Fundus Dis,1998,14:72-75)