[Retinal degeneration with blue cone hypersensitivity].
3
Citation
0
Reference
10
Related Paper
Abstract:
The clinical and electrophysiological findings are presented for five patients who had enhanced blue cone sensitivity. This new syndrome is characterized by night blindness, yellow, partly pigmented flecks at the posterior pole and variable visual loss. Maculopathy and peripheral retinoschisis may occur. The electroretinogram shows similar responses to dark and light-adapted conditions and long b-wave implicit times. This was the first time that it could successfully be demonstrated that the disease is really inherited and is probably of autosomal recessive origin. The main diagnostic test is an ERG recording with blue and red stimuli in the light-adapted state. These patients show enhanced sensitivity for blue instead of red when compared to normals and other retinal degenerations.Keywords:
Erg
Electroretinography
Retinoschisis
Cite
In Brief Purpose: To study retinal function after vitrectomy. Methods: Core vitrectomy was performed in 12 rabbits under standardized conditions using a vitreous cutting rate of either 600 or 1200 cuts/min. Full-field electroretinography (ERG) and multifocal electroretinography (mfERG) were performed pre- and postoperatively. Morphologic change was monitored by immunohistochemistry directed against glial fibrillary acidic protein (GFAP). Results: Three days postoperatively, the b-wave amplitudes of all cone and rod responses of the ERG were significantly reduced in all vitrectomized eyes. At 28 days, the rod response was still reduced, but returned to normal by 58 days. No correlation was found between vitreous cutting speed and ERG findings. No reduction in the central cone function was detected in the mfERG. GFAP upregulation was found in the entire retina of vitrectomized eyes 3 days after surgery. GFAP expression was present after 28 and 58 days in eyes in which the vitreous cutting rate had been set to 600 cuts/min, but not in the 1200 cuts/min eyes. Conclusion: Pars plana vitrectomy transiently affects retinal function in rabbit eyes. Vitreous cutting speed is not related to the reduced function but appears inversely correlated to Müller cell activation, indicating that high-speed vitreous cutters are more lenient to the retina. Retinal function and morphology was studied in rabbit eyes after vitrectomy using high- and low- speed vitreous cutting. Reduced electroretinogram amplitudes and upregulation of glial fibrillary acidic protein (GFAP) were found in all eyes after 3 days. Retinal function was restituted after 58 days, but GFAP upregulation persisted in eyes where a low cutting rate was used.
Electroretinography
Erg
Cite
Citations (16)
Purpose: Recent evidence suggests structural changes distal to the inner retina in multiple sclerosis (MS) patients. The functional correlates of these proposed structural abnormalities remain unclear. We investigated outer retinal function and structure in MS patients, and quantified to what extent outer retinal structure influenced function in these patients. Methods: Outer retinal function was assessed using the full-field and multifocal electroretinogram (ERG/MF-ERG), whereas retinal structure was assessed using spectral-domain optical coherence tomography (OCT). Results were compared with preexisting normative data. The relationships between electrophysiology parameters and the OCT values corresponding to the proposed cellular origins of the ERG and MF-ERG were analyzed. Results: Most electrophysiological responses were delayed in MS patients, independently of optic neuritis (ON). Inner retinal thickness and volumes were reduced, and inner nuclear layer volume marginally increased, in eyes with previous ON; all other OCT parameters were normal. OCT results correlated with ERG amplitudes, but not with ERG peak times or any MF-ERG parameters. Conclusions: We recorded outer retinal dysfunction without detectable abnormalities of the corresponding retinal layers in MS patients, not ascribable to retrograde degeneration following ON. The findings complement a growing body of literature reporting primary retinal abnormalities distal to the ganglion cell–inner plexiform layer complex in MS patients, with our data suggesting that this may be a more widespread phenomenon than previously thought. ERG may be of more utility in detecting retinal dysfunction in MS patients than MF-ERG. Analysis of peak times, rather than response amplitudes, is recommended.
Erg
Electroretinography
Ganglion cell layer
Outer nuclear layer
Optic neuritis
Cite
Citations (32)
iii ACKNOWLEDGEMENTS iv TABLE OF CONTENTS v LIST OF FIGURES viii LIST OF ABBREVIATIONS x I. LITERATURE REVIEW 1 1.1. Development and anatomy of the vertebrate eye 1 1.1.1. Development of the retina: RPE 1 1.1.2. Development of the retina: Neural retina 2 1.2. Development of neural retinal cell types in chickens 3 1.2.1. Retinal ganglion cells 3 1.2.2. Photoreceptors: rods and cones 5 1.2.3. Muller cells 6 1.2.4. Amacrine, horizontal and bipolar cells 7 1.3. Degenerative diseases of the retina 8 1.3.1. Retinitis pigmentosa (RP) 8 1.3.2. Leber congenital amaurosis (LCA) 8 1.3.3. Age-related macular degeneration (AMD) 9 1.4. Electroretinography (ERG) 9 1.4.1. Oscillatory potentials (OPs) 12 1.4.2. Multi-focal electroretinography (mfERG) 13 1.4.3. Pattern electroretinography (pERG) 13 1.4.4. Flicker ERG (fERG) and Scotopic Threshold Response (STR) 14 vi 1.5. Mutant chick strains of inherited retinal degeneration diseases 14 1.5.1. Retinopathy globe enlarged (rge) 15 1.5.2. Blindness enlarged globe (beg) 15 1.5.3. Retinal dysplasia and degeneration (rdd) 15 1.5.4. Retinal degeneration (rd) in the Rhode Island Red 16 1.5.5. Delayed amelanosis (DAM) 17 I
Electroretinography
Erg
Cite
Citations (0)
Erg
Electroretinography
Cite
Citations (26)
Electroretinography
Erg
Photoreceptor cell
Cite
Citations (6)
Abstract Retinal degeneration 10 (rd10) mice are a model of autosomal recessive retinitis pigmentosa (RP), identified by Chang et al. in 2002 (Vision Res. 42:517–525). These mice carry a spontaneous mutation of the rod‐phosphodiesterase (PDE) gene, leading to a rod degeneration that starts around P18. Later, cones are also lost. Because photoreceptor degeneration does not overlap with retinal development, and light responses can be recorded for about a month after birth, rd10 mice mimic typical human RP more closely than the well‐known rd1 mutants. The aim of this study is to provide a comprehensive analysis of the morphology and function of the rd10 mouse retina during the period of maximum photoreceptor degeneration, thus contributing useful data for exploiting this novel model to study RP. We analyzed the morphology and survival of retinal cells in rd10 mice of various ages with quantitative immunocytochemistry and confocal microscopy; we also studied retinal function with the electroretinogram (ERG), recorded between P18 and P30. We found that photoreceptor death (peaking around P25) is accompanied and followed by dendritic retraction in bipolar and horizontal cells, which eventually undergo secondary degeneration. ERG reveals alterations in the physiology of the inner retina as early as P18 (before any obvious morphological change of inner neurons) and yet consistently with a reduced band amplification by bipolar cells. Thus, changes in the rd10 retina are very similar to what was previously found in rd1 mutants. However, an overall slower decay of retinal structure and function predicts that rd10 mice might become excellent models for rescue approaches. J. Comp. Neurol. 500:222–238, 2007. © 2006 Wiley‐Liss, Inc.
Electroretinography
Erg
Retinal regeneration
Cite
Citations (456)
Electroretinography
Erg
Fundus (uterus)
Fundus Photography
Cite
Citations (22)
Erg
Knockout mouse
Electroretinography
Aquaporin 4
Outer nuclear layer
Light sensitivity
Cite
Citations (13)
Outer nuclear layer
Erg
Electroretinography
Cite
Citations (34)
Erg
Multielectrode array
Electroretinography
Cite
Citations (3)