Retinal dysfunction in eyes of patients with BRCA1 gene mutation

PURPOSE: To assess the retinal function in BRCA1 gene mutation carriers. MATERIAL AND METHODS: Thirty unaffected patients (60 eyes) with constitutional BRCA1 gene mutation were studied. Flash ERG recordings were performed in accordance with the International Society for Clinical Electrophysiology of Vision (ISCEV) standards. RESULTS: In ERGs, in the maximal response, amplitude of a-wave (p<0.02) was reduced. In the cone single- flash response, the amplitude of a-wave (p<0.04) was also reduced. In the scotopic oscillatory potentials (OPs), we noted: increased amplitude of OP2 (p<0.0006), increased index of OP amplitude (01+02+03+04) (p<0.04), and increased latencies of OP1 (<0.05) and OP3 (p<0.004) and OP4 (p<0.03). CONCLUSIONS: Slight dysfunction of rods, cones and inner retinal layers is present in asymptomatic carriers of BRCA1 gene mutation.     

Electro-oculographic and electroretinographic studies in HNPCC gene mutation carriers

PURPOSE: To assess retinal function in HNPCC gene mutation carriers. PATIENTS: 19 carriers (38 eyes) of HNPCC genes and controls. METHODS: Electro-oculogram, standard flash electroretinogram and pattern electroretinogram (PERG) recordings were performed. RESULTS: In the total group of HNPCC gene mutation carriers examined by oscillatory potentials, reduced amplitude (p < 0.0004) and increased latency (p < 0.04) of the O3 wave and increased latency (p < 0.02) of the O4 wave were found. In the subgroup of carriers with hMLH1 gene mutation, reduced amplitudes of the O3 (p < 0.0005) and O4 (p < 0.04) waves were identified. In the total group of HNPCC gene mutation carriers examined by PERG, reduced amplitudes of the P50 (p < 0.003), N95 (p < 0.02) and abnormal N95/P50 ratio (p < 0.02) were revealed. In the subgroup of hMLH1 gene mutation carriers, reduced amplitude of the P50 (p < 0.04) and abnormal N95/P50 ratio (p < 0.02) were observed, whereas in the hMSH2 gene mutation carrier subgroup, reduced amplitude (p < 0.03), shortened latency of the P50 wave (p < 0.02) and reduced amplitude of the N95 wave (p < 0.03) were found. CONCLUSION: Constitutional dysfunction of the inner retina appears to be a characteristic feature of HNPCC gene mutation carriers.     

Retinal function in the von Hippel-Lindau disease

Purpose: To assess the retinal function in patients with von Hippel-Lindau disease (VHL). Patients: Studies were undertaken in 12 patients (17 eyes) with detected VHL gene mutation and 12 normal healthy controls (17 eyes). Methods: Pattern ERG (PERG), standard flash electroretinogram (ERG) recordings were performed in accordance with the International Society for Clinical Electrophysiology of Vision (ISCEV) standards. Results: In VHL patients, electrophysiological statistically significant changes were found. In PERG examination, increased latency of P50 was found in the total VHL group (p<0.02) and in the VHL subgroup with retinal angiomas (p<0.04). In ERG examination, photopic b-wave latency was increased in the total VHL group (p<0.03) and also in the VHL subgroup without retinal angiomas (p<0.05). In OPs, latency increase of OP2, OP3 waves and reduced amplitude of OP3 wave in the total VHL group (OP2 latency, p<0.05; OP3 latency, p<0.01; OP3 amplitude, p<0.03) and in the VHL subgroup with retinal angiomas (OP2 latency, p<0.02; OP3 latency, p<0.008; OP3 amplitude, p<0.02) were obtained. Conclusions: It can be hypothesized that dysfunction of the inner retinal layer is present in individuals with VHL disease even in patients without retinal angiomas.     

Relationship of oscillatory potential amplitude to A-wave slope over a range of flash luminances in normal subjects

The effect of flash luminance on the relationship of oscillatory potential (OP) amplitude, as a measurement of inner retinal function, to a-wave slope, as a measurement of photoreceptor function, was evaluated in full-field electroretinograms recorded from normal subjects. The ratio of OP amplitude to a-wave slope was found to be independent of flash luminance over a 3000-fold luminance range. This finding raises the possibility that a reduction in the ratio of OP amplitude to a-wave slope in eyes with media opacities may be used as a sign of inner retinal malfunction. Selected cases are presented to illustrate application of this approach.     

Origin of electroretinogram amplitude growth during light adaptation in pigmented rats

We assessed the growth of the rat photopic electroretinogram (ERG) during light adaptation and the mechanisms underlying this process. Full field ERG responses were recorded from anesthetized adult Brown-Norway rats at each minute for 20 min of light adaptation (backgrounds: 1.8, 2.1, 2.4 log scotopic cd m(-2)). The rat photopic b-wave amplitude increased with duration of light adaptation and its width at 33% maximal amplitude narrowed (by approximately 40 ms). These effects peaked 12-15 min after background onset. The narrowing of the b-wave reflected steepening of the b-wave recovery phase, with little change in the rising phase. OP amplitudes grew in proportion to the b-wave. Inhibition of inner retinal responses using TTX resulted in a greater relative growth of b-wave and OP amplitude compared with fellow control eyes, and delayed the change in recovery phase by approximately 5 min. Inhibition of all ionotropic glutamate receptors with CNQX/D-AP7 delayed both rising and recovery phases equally (approximately 12 ms) without altering b-wave width or the time course of adaptation changes. These outcomes suggest that inner retinal light responses are not directly responsible for b-wave amplitude growth, but may contribute to the change in its recovery phase during adaptation. A TTX-sensitive mechanism may help to hasten this process. The cone a-wave was isolated using PDA/L-AP4 or CNQX/L-AP4. A-wave amplitude (35 ms after stimulus onset) also increased with time during light adaptation and reached a maximum (130 +/- 29% above baseline) 12-15 min after background onset. B-wave amplitude growth in fellow control eyes closely followed the course and relative magnitude of cone a-wave amplitude growth. Hence, the increase of the cone response during light adaptation is sufficient to explain b-wave amplitude growth.     

Ocular phenotype of CORD5, an autosomal dominant retinal dystrophy associated with PITPNM3 p.Q626H mutation

Purpose: To describe in detail the phenotype of CORD5 in two families segregating a mutation c.1878G>C (p.Q626H) in the PITPNM3 gene. Methods: The study included 35 individuals from two different families of Swedish origin, all heterozygous for a PITPNM3 p.Q626H mutation. All participants underwent ophthalmological examination including kinetic perimetry, and in selected cases adaptometry, colour vision tests and optical coherence tomography (OCT). Electrophysiological studies were also performed. In some cases, the data were obtained from medical records. Results: The majority of patients showed subnormal visual acuity and light sensitivity from childhood. Early signs of macular degeneration were also observed. There was a progressive decrease in visual acuity leading to legal blindness in early adulthood. Electrophysiological testing showed a progressive loss of photoreceptor function restricted mainly to the cones. OCT revealed decreased macular thickness with flattened and enlarged fovea. Conclusion: Our observations of the PITPNM3 p.Q626H mutation carriers confirm that CORD5 is a disease not to mix with other retinal degenerations mapped to 17p13. The results of our clinical evaluation so far indicate that CORD5 is characterized by predominant cone dysfunction without signs of general involvement of the retinal pigment epithelium. The rod system also seems to be unaffected. In this sense, CORD5 is different from other autosomal dominant CORDs where rod involvement is present to some degree in a late phase of the disease. Some intra‐ and inter‐familial differences regarding the severity of the clinical picture were observed.     

Modulation of the scotopic electroretinogram and oscillatory potentials with systemic hyperoxia and hypercapnia in humans

PURPOSE: Systemic hyperoxia reduces blood flow to the retina while systemic hypercapnia has the opposite effect. However, the effect this modification in blood flow has on neuroretinal function in humans has not been documented yet. The purpose of this study was to evaluate the effect of pure oxygen and carbogen breathing on scotopic electroretinograms (ERGs) and oscillatory potentials (OPs) in humans. METHODS: Thirty-five healthy adults volunteered for this study. The ERGs and OPs were recorded: 1) during room air breathing, 2) after a period of pure oxygen (O(2)) or carbogen breathing, 3) in room air just after the flow of gas was interrupted, and 4) 10 minutes after the end of the gas administration. RESULTS: The amplitude and latency of the a- and b-waves were not altered during systemic hyperoxia. The amplitude of OP3 increased during hyperoxia while the amplitude of the other OPs was not altered. The latency of all OPs remained stable throughout the O(2) session. Ten minutes after the end of pure O(2) breathing, the a- and b-wave latencies were delayed. No change was found in the amplitude of the a-wave during the carbogen session that increased the end-tidal carbon dioxide by 7.1%, whereas the b-wave was reduced ten minutes after the end of carbogen breathing. The amplitude of OP5 was reduced during carbogen breathing, as well as 10 minutes later. The amplitude of all other OPs, as well as the latencies of all ERG and OP components remained stable throughout the carbogen session. CONCLUSIONS: Breathing pure O(2) or carbogen did not compromise retinal function in any major way, likely due to adequate retinal and choroidal regulatory mechanisms. Further investigations are required to better delineate the impact and temporal characteristics of such physiological challenges on retinal function.     

Predominant loss of the photopic negative response in central retinal artery occlusion

PURPOSE: To determine how the photopic negative response (PhNR) is affected in central retinal artery occlusion (CRAO). DESIGN: Observational case series. METHODS: Seven patients with unilateral CRAO were included. Full-field scotopic and photopic electroretinograms (ERGs) including the PhNR were recorded. Each ERG amplitude in the affected eye was expressed as a percentage of amplitude of the corresponding wave in the unaffected eye. RESULTS: Mean of the PhNR amplitude was reduced to 12.3 +/- 11.7% of that of unaffected eyes whereas the cone b-wave amplitude was attenuated to only 73.4 +/- 30.4%. This reduction of the PhNR amplitude was more significant than that of other waves including the rod b-wave, maximum a-wave and b-wave, cone a-wave and b-wave, and 30 Hz flicker ERG (P <.005). CONCLUSIONS: The PhNR was severely affected in CRAO despite relative preservation of the cone b-wave, implicating massive loss of ganglion cells and their axons.     

Genetic analysis of Chinese families reveals a novel truncation allele of the retinitis pigmentosa GTPase regulator gene

AIM: To make comprehensive molecular diagnosis for retinitis pigmentosa (RP) patients in a consanguineous Han Chinese family using next generation sequencing based Capture-NGS screen technology. METHODS: A five-generation Han Chinese family diagnosed as non-syndromic X-linked recessive RP (XLRP) was recruited, including four affected males, four obligate female carriers and eleven unaffected family members. Capture-NGS was performed using a custom designed capture panel covers 163 known retinal disease genes including 47 RP genes, followed by the validation of detected mutation using Sanger sequencing in all recruited family members. RESULTS: Capture-NGS in one affected 47-year-old male reveals a novel mutation, c.2417_2418insG:p.E806fs, in exon ORF15 of RP GTPase regulator (RPGR) gene results in a frameshift change that results in a premature stop codon and a truncated protein product. The mutation was further validated in three of four affected males and two of four female carriers but not in the other unaffected family members. CONCLUSION: We have identified a novel mutation, c.2417_2418insG:p.E806fs, in a Han Chinese family with XLRP. Our findings expand the mutation spectrum of RPGR and the phenotypic spectrum of XLRP in Han Chinese families, and confirms Capture-NGS could be an effective and economic approach for the comprehensive molecular diagnosis of RP.     

The photopic negative response of the flash electroretinogram in retinal vein occlusion

The photopic negative response (PhNR) has recently been shown to be severely affected in central retinal artery occlusion (CRAO), despite relative preservation of the cone b-wave compared to that in the healthy unaffected fellow eye. The aim of this study was to test how the PhNR of the flash electroretinogram (ERG) is affected in human retinal vein occlusion. PhNR was elicited with red stimuli (1 cd s/m², 5 cd s/m², and 7 cd s/m² with 4 ms duration) and blue background (10 cd/m²). Standard Ganzfeld flash ERG was produced according to the ISCEV standard for the clinical electroretinogram (2004). Sixteen patients with central retinal vein occlusion (CRVO), 14 patients with branch retinal vein occlusion (BRVO), and 16 controls were analyzed. The amplitude of the PhNRs was significantly smaller in the CRVO and BRVO eyes than those in the unaffected fellow or control eyes (p = 0.000). There was a significantly greater reduction of PhNR amplitudes than that of other waves including the OPs, rod b-wave, combined a-wave and b-wave, cone a-wave and b-wave, and 30 Hz flicker ERG. Thus, PhNR amplitude in retinal vein occlusion is severely affected. There is a potential role for PhNR in assessing inner retinal damage and evaluating the effect of treatment.     

Clinical and electroretinographic findings of female carriers and affected males in a progressive X-linked cone-rod dystrophy (COD-1) pedigree

OBJECTIVE: To study the clinical and electroretinographic findings of affected males and female carriers in a family with X-linked cone-rod dystrophy (COD-1). DESIGN: Observational case series. PARTICIPANTS: Twenty-five members of a five-generation pedigree were examined. METHODS: A history of visual impairment including age at onset, loss of acuity, color vision abnormalities, photophobia, and nyctalopia was obtained. A complete ophthalmologic examination was performed, including kinetic perimetry with a Goldmann perimeter, FM 100-hue testing, and standardized Ganzfeld electroretinography following the ISCEV protocol. MAIN OUTCOME MEASURES: Patients were classified as affected or unaffected on the basis of the clinical examination. All carrier females had affected sons. RESULTS: Nine affected males and seven female carriers were identified. Affected males noted decreased visual acuity and poor color vision within the first two decades of life. Early in the disease, macular retinal pigment epithelial (RPE) changes were found that progressed to an atrophic macular scar by the fifth decade. Evidence of progression from macular pigment mottling to an atrophic macular lesion over a 13-year period was identified in one patient. The photopic, single-flash, b-wave amplitude was low in all affected males and declined with age. The 30-Hz flicker b-wave implicit times were abnormally prolonged in all affected males. Female carriers were asymptomatic although three had slightly abnormal color vision and small paracentral field defects and subtle RPE defects were found in three carriers. Carriers demonstrated prolongation of the 30-Hz flicker b-wave implicit time and interocular asymmetry. Five of seven carriers and two affected males demonstrated reduced oscillatory potentials and an abnormal-appearing flattened photopic a-wave. Five men and two women demonstrated a characteristic tapetal-like retinal sheen. CONCLUSIONS: Affected patients in this pedigree demonstrate early loss of visual acuity and poor cone function with late rod involvement. Female carriers may appear clinically normal or may be identified by subtle color vision defects, fundus abnormalities, prolongation of the 30-Hz flicker implicit time with interocular asymmetry, or an abnormal flattened photopic a-wave. Genetic linkage analysis of this family was recently reported and the disease-causing gene has been mapped to an approximately 1-Mb interval on chromosome Xp11.4.     

The effect of diphenylhydantoin on the electroretinogram

Acute administration of diphenylhydantoin (DPH) in rabbits produces a significant increase in the amplitude of the a-wave. A marked increase in the amplitude of the b-wave is also noted but the time course is slower than that for the a-wave. While in controls the oscillatory potential (OP) recordings essentially consist of three major types, recordings taken after DPH injection consist of one major OP (OP₂), which appears to be a result of the fusion of the original OP₂ with another OP produced by the DPH injection. A similar blend of OPs was also seen in ERGs recorded from three human subjects on DPH therapy.     

视网膜中央静脉阻塞性黄斑水肿的OCT与ERG的对比分析

目的：观察视网膜中央静脉阻塞性黄斑水肿的黄斑区视网膜厚度与视网膜电图( electroretinogram, ERG )各项参数(Cone-a,Cone-b和30Hz)变化的关系。
　　方法：随机选择视网膜中央静脉阻塞患者25例25眼及25只对侧眼分别行明视闪光视网膜电图及光学相干断层扫描( optical coherence tomography,OCT)检查,明视闪光视网膜电图检查测各项参数的振幅和潜伏期, OCT测量黄斑区九部分的视网膜厚度,分析黄斑区形态参数与明视闪光视网膜电图各参数变化之间的关系。
　　结果：黄斑区除颞侧外七个部位视网膜厚度与 ERG 的Cone-b和30 Hz潜伏期相关。
　　结论：研究发现视网膜中央静脉阻塞患者的黄斑区视网膜厚度与内层视网膜功能密切相关。     

Ophthalmic findings in a family with early-onset isolated ectopia lentis and the p.Arg62Cys mutation of the fibrillin-1 gene (FBN1)

Purpose: The purpose of this paper is to describe ophthalmic findings in a family with isolated ectopia lentis (EL) caused by a specific FBN1 mutation.

Methods: Detailed family histories and clinical data were recorded for six isolated EL patients of 11 family members. The ophthalmological and systematic examinations were performed on patients and unaffected members of the investigated family. The detailed ocular examinations included visual acuity, anterior chamber depth, pupil size, lens location, optometry, central corneal thickness, keratometry, slitlamp examination, fundus examination, axial length, ocular B-ultrasound, gonioscope checking, ultrasound biomicroscopy (UBM) and intraocular pressure (IOP; Goldmann applanation tonometer). Systematic examinations included the measurement of echocardiogram, height, arm span, skull, face, jaw, tooth, breast bone, spinal column, and skin. Genomic DNA was extracted using the phenol-chloroform extraction method for all subjects, and sequencing was carried out on an ABI Prism 3730 Genetic Analyzer.

Results: A heterozygous mutation, c.184C>T (p.Arg62Cys) in exon 2 of FBN1 was identified in all affected members but was not found in any unaffected member of the family. Our study presented detailed clinical manifestations, including some novel ophthalmic findings, such as pupillary abnormality, different types of glaucoma, and progressive hyperopia.

Conclusions: Ophthalmic findings and the p.Arg62Cys mutation of FBN1 gene were reported in a family with early-onset isolated ectopia lentis.     

Association of electroretinogram and morphological findings in branch retinal vein occlusion with macular edema

The objective of this study is to evaluate the relations among electroretinogram parameters (cone a-wave, cone b-wave, and 30-Hz flicker), retinal thickness, and retinal volume in patients with branch retinal vein occlusion (BRVO) and macular edema. We prospectively examined 33 patients (33 eyes) with BRVO and macular edema. The amplitude and implicit time of the a-wave cone, b-wave cone, and 30-Hz flicker were calculated automatically from the ERG. Retinal thickness and volume were measured by optical coherence tomography (OCT) in nine macular subfields. Then, correlations between the ERG parameters and morphological parameters were analyzed. The 30-Hz flicker amplitude was significantly smaller in the eyes with BRVO and macular edema than in the unaffected contralateral eyes. Thirty-hertz flicker and cone b-wave implicit times were significantly longer in the eyes with macular edema than in the unaffected eyes. The implicit time of the cone b-wave was correlated with both retinal thickness and retinal volume in the temporal subfields. Thirty-hertz flicker amplitude was correlated with both retinal thickness and volume in the temporal and superior outer (site of occlusion) subfields, while 30-Hz flicker implicit time was correlated with retinal thickness and volume in the outer temporal subfield. Multiple regression analysis demonstrated that the retinal thickness and volume of the temporal subfields were significant “determinants” of the implicit time for the cone b-wave and 30-Hz flicker, as well as the 30-Hz flicker amplitude. These findings suggest that OCT parameters of the temporal region may reflect postreceptoral cone pathway function in BRVO patients with macular edema.     

Effects of hypoxemia on the a- and b-waves of the electroretinogram in the cat retina

PURPOSE: Slow components of the electroretinogram (ERG) are sensitive to even mild hypoxemia (60 < P(a)O(2) < 100 mm Hg) in the cat eye. However, the electrical responses of the inner retina remain unchanged until P(a)O(2) is below 40 mm Hg. In this study, the effects of hypoxemia on photoreceptors, on which both slow ERG components and inner retinal activity depend, were examined by recording the a-wave of the ERG. METHODS: The ERG of dark-adapted, anesthetized cats was recorded between an Ag-AgCl electrode in the vitreous humor and a reference electrode near the eye. Responses to bright flashes of diffuse white light were recorded at 3-minute intervals during hypoxemic episodes lasting 15 minutes to 2 hours. RESULTS: The cat a-wave was well described by the Lamb and Pugh a-wave model during normoxia and hypoxemia. During mild hypoxemia (P(a)O(2) of 50-60 mm Hg), small changes in a-wave amplitude were detected but did not become greater during severe hypoxemia. The mean decrease in the a-wave amplitude during severe hypoxemia (P(a)O(2) of 20-30 mm Hg) was 8.9% from the mean amplitude during air breathing. The effects of hypoxemia were more severe on the b-wave amplitude. The mean decrease in the b-wave was 35% at P(a)O(2) of 20-30 mm Hg. CONCLUSIONS: The a-wave is more resistant to severe hypoxemia than the b-wave. This implies that photoreceptor transduction works almost normally during hypoxemia and that failure of inner retinal PO(2) regulation causes the decrease in the b-wave. Previously observed changes in the amplitudes of slow ERG components during hypoxemia may result from changes in the ionic environment, rather than a failure of photoreceptor energy metabolism.     

Oscillatory potential analysis and ERGs of normal and diabetic rats

PURPOSE: To identify and characterize the early alterations of the ERG oscillatory potentials (OPs) under conditions of poor glycemic control associated with diabetes in an animal model. To characterize and correlate the a- and b-wave properties of the ERGs of diabetic animals in parallel with the changes in oscillatory potentials. METHODS: Blood sugars, weights, and ERGs were measured for a group of rats each week for 3 weeks to obtain baseline information. A single injection of streptozotocin was given to the experimental animals. Weights, blood sugar, glycosylated hemoglobin, and detailed ERGs were recorded weekly for up to 12 weeks in control and experimental animals. RESULTS: OP kinetics were found to be inherently related to amplitude. Amplitude-matched OPs were delayed in diabetic animals when compared with baseline data from the same animal. The kinetics of OPs in control animals stayed the same or were slightly accelerated relative to their baseline values. For a given recording condition, OP kinetics were very stable over time and this stability was not disturbed in diabetic animals. Diabetic animals showed a small but significant delay in the a-wave, but no change in b-wave timing. The sensitivity of the b-wave was reduced twofold, but that of the a-wave was not changed. CONCLUSIONS: OPs have been used to evaluate retinal function in both diabetic models and patients. The comparison of amplitude-matched OPs is a robust determinant of changes in kinetics. Researchers and clinicians who use OPs may wish to consider the relationship between OP amplitude and kinetics to avoid confounding assessments of abnormalities. The amplitude versus kinetics relationship does not change form in diabetic animals; it is merely shifted (delayed) on the time axis.     

Structural and functional maturation of the retina of the albino Hartley guinea pig

PURPOSE: Altricial animals, such as rats and mice, are born with their eyes closed, compared to precocial animals, such as guinea pigs and humans, which have their eyes opened at birth. The purpose of this study was to investigate if the retina of guinea pigs (precocial animal) is subjected to a postnatal maturation process similar to that previously reported for rodents. METHODS: Photopic and scotopic electroretinograms (ERG) and retinal histology were obtained from albino guinea pigs aged P1 to P75. RESULTS: Photopic ERG responses reached maximal amplitudes at P5 (a-and b-waves), that is 5 days (b-wave) to 10 days (a-wave) earlier than scotopic responses. However, the postnatal gain in b-wave amplitude was significantly (P < 0.05) more important for the cone (73.38 +/- 4.4%) signal than for the rod (15.23 +/- 3.96%), suggesting that the rod function is more mature at birth. Similarly, the short latency photopic oscillatory potential (ie: OP2) reached its maximal value 5 days (P10) earlier than its scotopic equivalent (P15), while the long latency OPs (ie: OP3, OP4), reached their maximal values nearly 20 days sooner in scotopic condition. Finally retinal histology revealed a thinning of the retina with age, the latter being most pronounced at the level of the ganglion cell layer (GCL). CONCLUSION: Our results thus confirm that despite its relative maturity at birth (compared to rodents), the retina of newborn albino guinea pigs undergoes significant postnatal maturation modifying its structure as well as its function, albeit not as extensive as that previously documented for altricial animals.     

Postural modifications of the oscillatory potentials of the electroretinogram in primary open-angle glaucoma

The postural variations in the retinal microcirculation in glaucomatous patients were studied by evaluation of the oscillatory potentials (OPs) of the ERG. The OPs in scotopic adaptation were examined in subjects with primary open-angle glaucoma and in an age- and sex-matched normal control group in different body positions (seated, supine, anti-Trendelenburg, Trendelenburg). In the seated position, the difference of mean OP amplitude between the control group and the glaucomatous patients was highly significant (p < 0.001). In the normal subjects the OP amplitude in the Trendelenburg position was statistically lower with respect to the values obtained in all other positions (p < 0.05). In the glaucomatous patient group, the OP amplitude in the anti-Trendelenburg position was increased as compared to the other positions (p < 0.05). The study showed a reduction in amplitude of OPs in glaucomatous patients and their different behaviour in both groups with changes in body position.     

The electroretinogram components in Abyssinian cats with hereditary retinal degeneration

PURPOSE: To examine phototransduction using the a-wave and other aspects of retinal function with the intraretinal b- and c-waves at different stages of an inherited photoreceptor degeneration in Abyssinian cats. METHODS: Vitreal and intraretinal ERGs were recorded from eight dark-adapted, anesthetized Abyssinian cats. Brief bright flashes were used to elicit vitreal a- and b-waves. Longer, weaker flashes were used to elicit intraretinal b- and c-waves. Stages 1 through 4 of the disease were characterized ophthalmoscopically. Parameters of the Lamb and Pugh a-wave model (a(max), A, and t(eff)) for the Abyssinian cats were compared with those for normal cats. Light microscopy was used to count photoreceptor nuclei. RESULTS: The maximum a-wave amplitude, a(max), was significantly smaller in stage 1, and continued to decrease (stage 1: 50% of normal, stage 2: 28%, stage 3: 27%; and stage 4: unrecordable). There was a small, but not significant, decrease in the amplification constant A from 0.24 +/- 0.11 s(-2) in normal cats to 0.16 +/- 0.08 s(-2) in Abyssinian cats. The intraretinal b- and c-wave amplitudes decreased most dramatically during the early stage of the disease. Affected animals had fewer photoreceptors than unaffected Abyssinians or control animals. The number of photoreceptors declined most rapidly in the inferior periphery. CONCLUSIONS: The amplitudes of all ERG components were already reduced significantly by stage 1 and progressively declined. The lack of major changes in a-wave model parameters indicates that the degeneration is probably not due to a mutation in transduction proteins. Losses of photoreceptor function were larger than losses of photoreceptor nuclei.