Electrophysiologic evaluation of retinal function in patients with psoriasis and vitiligo

Purpose

To investigate the relation between psoriasis and vitiligo with the electrophysiologic function of the retinal photoreceptors.

Methods

Patients with psoriasis or vitiligo referred for PUVA therapy were enrolled. Complete eye examination was performed. Patients with any drug or familial history or abnormal eye examination that might affect the retinal function were excluded. Standardized full-field electroretinogram (ERG) elicited with Ganzfeld stimuli using the commercial ERG system (Retiport32; Roland Consult) according to International Society for Clinical Electrophysiology of Vision guidelines was performed. The outcome measures were the difference between the mean rod response, standard combined response, single-flash cone response and 30-Hz flicker wave amplitudes of the patients and normal population.

Results

Seventy-six eyes of 38 patients (vitiligo: 21; psoriasis: 17) and 40 eyes of 20 normal subjects were enrolled in this study. The mean age of patients was 31.3 ± 11.3 years (range 16–54 years). Twenty-two patients (58 %) were female. The mean rod response b-wave, standard combined a- and b-waves, single-flash cone response b-wave and the 30-Hz flicker (N1-P1) amplitudes were significantly lower than the normal population in the same range of age as the study group. There was no significant difference between the patients with vitiligo and those with psoriasis in all wave amplitudes (P = 0.094).

Conclusion

This study showed that overall retinal electrophysiologic function in patients with vitiligo or psoriasis is significantly impaired compared with normal population, independent of age and sex.     

Protein kinase C expression in the rabbit retina after laser photocoagulation

Background Laser photocoagulation is a well-established treatment for diabetic retinopathy but the mechanism behind its effectiveness has not been elucidated. The protein kinase C (PKC) family is a group of enzymes which has been the subject of extensive interest in clinically related research since the advent of its role in the pathogenesis of diabetic retinopathy. With this study we wanted to explore whether PKC expression is altered in the retina after laser photocoagulation.Methods Normal rabbit eyes were treated with laser photocoagulation of varying intensity and examined after 30 min to 7 weeks. Treated and untreated regions of the retina were investigated histologically with the MC5 monoclonal antibody against PKC. Labeling for glial fibrillary acidic protein (GFAP), as well as hematoxylin and eosin (H&E) staining was also performed to assess the laser-induced trauma.Results In the normal retina, the MC5 antibody labeled rod bipolar cells and photoreceptor outer segments corresponding to PKC alpha. A translocated PKC expression with labeling concentrated in the rod bipolar terminals was seen in specimens examined 30 min after laser treatment, and after 1 week, no expression was seen in any part of the retina. After 2 weeks, PKC expression again indicated a translocated labeling pattern. After 5 weeks, labeling was found only in rod bipolar terminals in the peripheral retina. When comparing high- and low-intensity laser treatment 7 weeks postoperatively, no labeling was found in the high intensity-treated retinas, whereas low intensity-treated eyes displayed a near-normal labeling pattern. H&E staining revealed focal neuroretinal edema immediately after laser treatment, also in untreated areas. At later stages, destruction of the outer nuclear layer and migration of pigment epithelial cells in laser-lesioned areas was seen. GFAP-labeled Müller cells were seen 1 week postoperatively in the entire retina. Labeling after this time decreased, but was still present in laser spots after 5 and 7 weeks.Conclusions Laser photocoagulation alters the expression of PKC in the entire normal rabbit retina. The response follows a temporal pattern and is also related to laser intensity. These findings may help to explain the high efficacy of laser treatment in diabetic retinopathy.The authors have no financial relationship with any supporting organization.The authors have full control of all primary data and agree to allow Graefes Archive for Clinical and Experimental Ophthalmology to review their data if requested.     

Investigating retinal toxicity of tempol in a model of isolated and perfused bovine retina

Purpose

Tempol (4-hydroxy-2,2,6,6-tetramethylpiperidinyl-1-oxyl) is a membrane-permeable superoxide dismutase and potentially neuroprotective substance. This study evaluates the retinal tolerance of 0.5 mM, 1 mM, 2 mM, and 5 mM tempol measured by the electroretinogram (ERG) of an isolated and perfused retina whole mount.

Methods

For functionality testing, bovine retinas were prepared and perfused with an oxygen-saturated standard solution, and the ERG was recorded until stable b-wave amplitudes were reached. Tempol concentrations of 0.5 mM, 1 mM, 2 mM, and 5 mM were tested for 45 minutes. To investigate the effects on photoreceptor function, 1 mM aspartate was added to suppress the b-wave and obtain isolated a-waves. ERG amplitudes were monitored for 100 minutes.

Results

While no toxic effects for concentrations of 0.5 mM and 1 mM tempol could be detected, concentrations of 2 mM tempol and higher caused statistically significant negative effects on the b-wave amplitude (?38 %, p?=?0.02 for 2 mM; ?54 %, p?= 0.02 for 5 mM). The a-wave amplitude remained stable even at higher concentrations.

Conclusions

Although the photoreceptors seem to have a tolerance to high concentrations of tempol, higher intravitreal concentrations than 1 mM should be considered critical.     

Retinal function after vitrectomy

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.     

Full-field electroretinography under general anesthesia in retinoblastoma

Purpose

To investigate the electrical responses of the retina in retinoblastoma (RB), by recording full-field electroretinography (ERG) under general anesthesia.

Methods

The ERG was recorded using Ephios hand-held portable ERG system, according to International Standards for Clinical Electrophysiology of Vision. Forty-eight eyes of 43 cases and 33 eyes of 33 controls were enrolled. The cases were classified based on international intraocular retinoblastoma classification (IIRC). Forty-eight eyes of cases were divided into 30 cases with active RB and 18 cases with regressed RB.

Results

The amplitudes of a- and b-waves were decreased as compared to controls in all subgroups. The implicit times of all RB patients from group A to C differed statistically from controls (p value < 0.05) except for single-flash rod response. The ERG waveforms in group E eyes were non-recordable. The comparison of ERG parameters between active and regressed groups (IIRC groups A and B) was statistically insignificant. Single case follow-up of unilateral RB after systemic chemotherapy showed improvement in amplitudes compared to baseline parameters.

Conclusions

Reduced amplitudes and delayed implicit times were noted in advanced disease. The ERG of RB cases did not follow any specific pattern of waveform. ERG appears to be a dynamic parameter to observe changes following treatment for RB. Although ERG is not a diagnostic test for RB, it can be used as a complementary test to assess the residual retinal function in RB eyes.     

Multifocal electroretinogram contributes to differentiation of various clinical pictures within a family with Bardet–Biedl syndrome

Purpose

To demonstrate the use of the multifocal electroretinogram (mfERG) in addition to the full-field electroretinogram (ERG) in defining varying clinical pictures in children within a family with Bardet–Biedl syndrome (BBS).

Methods

All members from a family generation underwent a detailed history and examination before proceeding to a detailed ERG in accordance with the International Society of Clinical Electrophysiology for Vision protocol and a rapid, low-resolution mfERG. Of the sibling pair, the 13-year-old boy showed reduced vision and atypical maculopathy and the 10-year-old sister showed normal vision and atrophic maculopathy. Parents had normal ocular examination.

Results

The male sibling had reduced rod and cone full-field ERG responses with a relatively spared central response from the mfERG suggesting central macular sparing. In contrast, for the female sibling, the ERG was normal for the cone pathway although reduced for rod pathway, with mfERG showing central involvement. The mother had rod responses at the lower end of normal range, a normal cone pathway, and a normal mfERG. The father showed a normal ERG and mfERG.

Conclusion

The mfERG is a useful adjunct to full-field ERG in the paediatric population and in family studies.     

Delayed presentation of melanoma-associated retinopathy and subsequent resolution with cytoreduction surgery

Background

To present a case of melanoma-associated retinopathy (MAR) which manifested 26 months prior to a formal diagnosis of melanoma.

Methods

Case report.

Results

A 72-year-old female presented with bilateral continuous photopsia consistent with MAR of 7-months duration. At this point, visual function appeared normal with the exception of mildly impaired colour vision (10/17 Ishihara plates). The flash electroretinographic (ERG) revealed extinguished rod responses, a normal a-wave and reduced b-wave (electronegative ERG) on the maximal combined response, absent oscillatory potentials and broadened a-wave trough on the cone response. Multifocal ERG (mfERG) responses were delayed and demonstrated atypical morphology. Nineteen months after the initial presentation, her visual symptoms had progressed significantly with constant debilitating photopsia in combination with 13 kg weight loss. Biopsy of a now evident left axillary mass demonstrated a metastatic high-grade malignant melanoma. No primary was detected, and an axillary lymph node clearance was undertaken. Subsequently, visual symptoms resolved with corresponding improvement in the ERG over the next 18 months. Rod responses recovered such that the amplitude was at the lower limit of normal and the mfERG response delay lessened. Unfortunately, the melanoma recurred and the patient passed away 6 months later. Visual symptoms did not recur.

Conclusion

We present a case which demonstrates MAR may precede the formal diagnosis of melanoma by up to 26 months. The potential for improvement in the rod visual function persists over a period of years with normalisation of an electronegative waveform. In this case, cytoreductive surgery resulted in complete resolution of the MAR, which did not return even with a recurrence of the tumour.     

Retinal function after scleral buckling for recent onset rhegmatogenous retinal detachment: assessment with electroretinography and optical coherence tomography

PURPOSE: To investigate central and peripheral retinal function after scleral buckling surgery for recent onset rhegmatogenous retinal detachment (RD). METHODS: Fifteen phakic patients with rhegmatogenous RD for <1 week underwent scleral buckling surgery. Clinical investigation, optical coherence tomography (OCT), full-field electroretinography (ERG), and multifocal ERG (mfERG) with fundus illumination were performed preoperatively and 6 months postoperatively. RESULTS: Anatomical success was achieved in 14 patients. mfERG amplitudes were reduced preoperatively in detached retina, with significant improvement at follow-up (P = 0.002). Foveal amplitudes improved significantly (P = 0.027). There was no significant difference in postoperative mfERG amplitudes between areas that had been preoperatively detached or attached (P = 0.739). In the subgroup of eight patients in whom the detachment engaged the fovea preoperatively, rod function improved significantly as assessed with full-field ERG (P = 0.008). In these patients, the extent of detachment ranged between 4 clock hours and 6 clock hours, as compared with 2 clock hours and 5 clock hours in the remaining patients. OCT showed subretinal foveal fluid in four patients at follow-up. CONCLUSIONS: In recent onset rhegmatogenous RD, total rod and localized central retinal dysfunction in detached retina can improve significantly after reattachment. mfERG and OCT are suitable tools for further studies of functional outcomes in RD.     

ERG findings in patients using hydroxychloroquine

PURPOSE: To examine the electroretinographic findings derived by full-field electroretinography (ERG) and multifocal electroretinography (mfERG) in patients taking HCQ for different periods of time, including those without clinically visible evidence of toxicity. METHODS: The records for 26 consecutive patients (51 eyes, age 19-79) who had used HCQ for 1-30 years and who had an ERG and/or mfERG were reviewed. RESULTS: The patients fell into 5 groups, according to the results from their electrophysiological and clinical evaluation: no abnormalities (n = 13), cone b-wave delay (n = 2), decreased ERG amplitude (n = 6), borderline toxicity (n = 2) and toxicity (bull's-eye maculopathy; n = 3). Oscillatory potential (OP) amplitudes were decreased in many of the patients, even in the absence of any other clinical or ERG abnormality. MfERGs were normal in these patients without other abnormalities, but showed a bull's eye pattern of damage in a patient with clear toxicity. The full-field ERG parameters (including OPs) showed little relationship to the duration of HCQ usage or the cumulative dose. CONCLUSIONS: A substantial subgroup of patients showed varying degrees of diffuse ERG abnormality despite having no clinical signs (visual or fundus changes) of toxicity, and despite a range of cumulative doses. The mfERG showed toxicity, but its sensitivity in detecting early change remains to be determined. Further work is needed in order to separate pharmacological actions of the drug, retinopathy from the systemic disease, and potential toxic effects.     

Spatial differences in corneal electroretinogram potentials measured in rat with a contact lens electrode array

Purpose

It has been known for several decades that the magnitude of the corneal electroretinogram (ERG) varies with position on the eye surface, especially in the presence of focal or asymmetric stimuli or retinal lesions. However, this phenomenon has not been well-characterized using simultaneous measurements at multiple locations on the cornea. This work provides the first characterization of spatial differences in the ERG across the rat cornea.

Methods

A contact lens electrode array was employed to record ERG potentials at 25 corneal locations simultaneously following brief full-field flash stimuli in normally sighted Long-Evans rats. These multi-electrode electroretinogram (meERG) responses were analyzed for spatial differences in a-wave and b-wave amplitudes and implicit times.

Results

Spatially distinct ERG potentials could be recorded reliably. Comparing relative amplitudes across the corneal locations suggested a slight non-uniform distribution when using full-field, near-saturating stimuli. Amplitudes of a- and b-waves were approximately 3 % lower in the inferior quadrant than in the superior quadrant of the cornea.

Conclusions

The present results comprise the start of the first normative meERG database for rat eyes and provide a basis for comparison of results from eyes with functional deficit. Robust measures of spatial differences in corneal potentials will also support optimization and validation of computational source models of the ERG. To fully utilize the information contained in the meERG data, a detailed understanding of the roles of the many determinants of local corneal potentials will eventually be required.     

Recovery of function following regeneration of the damaged retina in the adult newt, <Emphasis Type="Italic">Notophthalmus viridescens</Emphasis>

The red-spotted newt, Notophthalmus viridescens, is one of few adult vertebrate organisms that has retained the remarkable ability to regenerate a complete retina following injury or removal. The aim of this study was to develop a non-invasive method to monitor recovery of components within the retinal circuitry, in vivo, following surgical removal (retinectomy) of the adult newt retina. A novel and reproducible protocol was established for full-field electroretinography in the intact newt retina. Electroretinograms (ERGs) were measured at the corneal surface. The effects of dilation and external body temperature on the ERG amplitudes were measured as well as the reproducibility in recording ERGs in the same animal over time. Retinectomies were conducted on 15 newts, and the a- and b-wave amplitudes were measured prior to retinectomy and at various timepoints after retinectomy. Surgical removal of the retina resulted in an initial loss of ERG a- and b-waves, representing loss of photoreceptor cells and cells of the inner nuclear layer. The ERG amplitudes recovered to baseline levels by 15 weeks post-retinectomy, indicative of subsequent restoration of retinal function after regeneration.     

The 52nd ISCEV International Symposium Abstract Issue

Purpose

We have monitored retinal function in patients treated for retinoblastoma (primarily, but not exclusively by intra-arterial chemotherapy infusion) by electroretinography (ERG) recordings for the past 7 years. We here present data from 599 ERG studies of 108 patients, in which a complete ERG protocol including both photopic and scotopic recordings was performed, in justification of our frequent practice of reporting primarily 30-Hz photopic flicker amplitude data.

Methods

Patients referred for treatment of retinoblastoma underwent ERG recordings during examination under anesthesia whenever possible: at baseline and following most treatment sessions. Correlations were calculated for the complete datasets between the four primary amplitude response parameters: photopic single flash b-wave, photopic 30-Hz flicker peak-to-trough, scotopic rod-isolating b-wave, and scotopic maximal flash b-wave.

Results

Using our adaptation of the International Society for Clinical Electrophysiology of Vision-recommended standard ERG protocol, ERG responses of eyes of patients with untreated retinoblastoma or following traditional or intra-arterial treatment for retinoblastoma show very high correlations between 30-Hz flicker amplitude responses and three other standard photopic and scotopic ERG response amplitudes. Reductions in ERG amplitudes seen in these eyes following treatment show no significant difference between retinal dysfunction estimated using rod- or cone-dominated responses.

Conclusion

These observations support the use of photopic response amplitudes (especially in response to 30-Hz flicker) as the primary ERG outcome measure in studies of treated and untreated eyes with retinoblastoma when more complete ERG protocols may be impractical.     

Retinal dysfunction in a presymptomatic patient with Huntington’s disease

Purpose

Huntington’s disease (HD) is an autosomal dominant, neurodegenerative disorder characterized by progressive motor dysfunction, cognitive decline, and psychiatric disturbances. Studies have shown retinal abnormalities in patients and mouse models with HD; however, to our knowledge, no prior research papers evaluated retinal structure and function in a presymptomatic patient with HD. The aim of this report is to present a case of retinal dysfunction in a presymptomatic patient with HD.

Methods

We investigated retinal structure and function in a 25-year-old male who tested positive for the gene that causes HD, but did not have any symptoms normally associated with HD. Vision and ocular testing included a comprehensive dilated ophthalmic examination, 24-2 full-threshold Humphrey visual field, spectral-domain optical coherence tomography (SD-OCT), fundus photography, full-field electroretinogram (ERG), and multifocal electroretinogram (mfERG).

Results

Visual electrophysiology testing showed rod and cone functional anomalies in both eyes. Full-field ERG amplitudes were subnormal in both eyes for the dark-adapted (DA) 0.01 ERG, DA 3 ERG, DA 3 oscillatory potentials (OPs), DA 10 ERG, light-adapted (LA) 3 ERG, and LA 30 Hz flicker, but peak times for the six standard ERG responses were not significantly different from normals. mfERGs revealed functional anomalies of the central retina with attenuated P1 amplitudes for five of the six concentric rings in the right eye and all six rings in the left eye. mfERG P1 peak times were normal at all eccentricities. Dilated fundus examination, SD-OCT, and fundus photography were unremarkable in both eyes. The visual field was normal in the right eye, but there was a mild paracentral field defect in the left eye.

Conclusions

Our results illustrate that the ERG and mfERG detected early retinal dysfunction in a presymptomatic patient with HD consistent with electroretinogram findings in animal models of HD. However, our report was limited to one patient and additional studies are needed to verify whether the ERG and/or mfERG can uncover neural dysfunction before motor, behavioral, and cognitive abnormalities are discernible in patients with HD.

     

Retinal function in eyes with proliferative diabetic retinopathy treated with intravitreal ranibizumab and multispot laser panretinal photocoagulation

Purpose

To compare retinal function changes in eyes with proliferative diabetic retinopathy (PDR) after intravitreal ranibizumab (IVR), combined or not with conventional (ETDRS) or multispot laser panretinal (PASCAL) photocoagulation (PRP).

Methods

This study included laser-naive PDR patients that required PRP. Eyes were randomly and prospectively assigned to receive IVR or IVR combined with PASCAL or EDTRS. PRP was performed at baseline in 1 (PASCAL) or 2 (ETDRS) sessions. In eyes with macular edema, macular short pulse grid laser was associated with IVR at baseline and IVR was repeated monthly or quarterly if neovascularization was detected on angiography. Comprehensive ophthalmological evaluations, including SD-OCT, were performed at baseline and every 4 weeks after treatment. Full-field electroretinography (ERG: extended ISCEV standard) was performed at baseline and at 12, 24 and 48 weeks.

Results

IVR?=?13, PASCAL?=?15 and ETDRS?=?15 eyes finished 48-week follow-up. There was a statistically significant BCVA improvement of 0.1–0.3 logMAR in all groups, and fluorescein angiography leakage area (FLA) reduced in 56%, 73%, and 73% from baseline for ETDRS, IVR and PASCAL, respectively, up to 48 weeks without significant differences between groups (p?>?0.05). A significant a- and b-wave amplitudes reduction was observed for dark- and light-adapted ERG for ETDRS and PASCAL, but only minor dark-adapted b-wave reduction was found for IVR, up to 48 weeks. As an example, at week 48, combined response b-wave amplitude reduced in 181.5?±?31.4 µV, 128.0?±?27.9 µV and 82.4?±?15.2 µV for ETDRS, PASCAL and IVR (p?<?0.05 each group), respectively. No significant difference was observed between ETDRS and PASCAL for any ERG parameter.

Conclusions

IVR combined with single or multiple spot PRP causes similar retinal function impairment during 48 weeks of observation, while IVR alone seems to be similarly effective controlling FLA without changing retinal function.

     

Electroretinographic assessment of retinal function during acute exposure to normobaric hypoxia

Background

The current study aimed to investigate retinal function during exposure to normobaric hypoxia.

Methods

Standard Ganzfeld ERG equipment (Diagnosys LLC, Cambridge, UK) using an extended ISCEV protocol was applied to explore intensity–response relationship in dark- and light- adapted conditions in 13 healthy volunteers (mean age 25?±?3 years). Baseline examinations were performed under atmospheric air conditions at 341 meters above sea level (F_IO₂ of 21 %), and were compared to hypoxia (F_IO₂ of 13.2 %) by breathing a nitrogen-enriched gas mixture for 45 min. All subjects were monitored using infrared oximetry and blood gas analysis.

Results

The levels of PaCO₂ changed from 38.4?±?2.7 mmHg to 36.4?±?3.0 mmHg, PaO₂ from 95.5?±?1.9 mmHg to 83.7?±?4.6 mmHg, and SpO₂ from 100?±?0 % to 87?±?4 %, from baseline to hypoxia respectively. A significant decrease (p?<?0.05) was found for saturation amplitude of the dark-adapted b-wave intensity–response function (Vmax), dark-adapted a- and b-wave amplitudes of combined rod and cone responses (3 and 10 cd.s/m²), light-adapted b-wave amplitudes of single flash (3 and 10 cd.s/m²), and flicker responses (5–45 Hz) during hypoxia compared to baseline, without changes in implicit times. The a-wave slope of combined rod and cone responses (3 and 10 cd.s/m²) and the oscillatory potentials were significantly lower during hypoxia (p?<?0.05). A isolated light-adapted ON response (250 ms flash) showed a reduction of amplitudes at hypoxia (p?<?0.05), but no changes were observed for the OFF response.

Conclusions

The results show significant impairment of retinal function during simulated normobaric short-term hypoxia affecting specific retinal cells of rod and cone pathways.     

Psychophysically determined full-field stimulus thresholds (FST) in retinitis pigmentosa: relationships with electroretinography and visual field outcomes

Purpose

The purpose of this study is to describe the relationships between full-field stimulus threshold (FST), electroretinography (ERG), and visual field (VF) outcomes in retinitis pigmentosa (RP).

Methods

Data from 47 patients with RP (n = 94 eyes) were evaluated. Patients were submitted to comprehensive ophthalmological examination including measurement of best-corrected visual acuity (BCVA), 30-2 threshold static VF, and microperimetry. Full-field ERG (ISCEV standard) was recorded, and achromatic FST was measured using a Diagnosys Espion system with the ColorDome? LED full-field stimulator (Diagnosys LLC, Lowell, MA, USA).

Results

BCVA mean ± SD was 0.31 ± 0.03 logMAR, and FST mean ± SD was ?18.45 ± 9.53 dB. No significant correlation was found between BCVA and FST. In contrast, statistically significant correlations were found between FST and static 30-2 VF mean deviation (r = ?0.389; P < 0.01), microperimetry mean threshold (r = ?0.607; P < 0.01). Dark and light-adapted ERGs were detectable in 28 and 48 eyes, respectively. Nevertheless, considering only the eyes with recordable ERG responses, moderate correlations were found between combined dark-adapted a-wave amplitude (r = ?0.560; P < 0.01), b-wave amplitude (r = ?0.643; P < 0.001), 30-Hz flicker response (r = ?0.501; P < 0.01), and FST, and high correlation with FST for cone b-wave amplitude (r = ?0.715; P < 0.01).

Conclusions

FST could be successfully determined in RP patients with a wide range of vision loss. FST results showed stronger correlations with full-field ERG amplitude than with sensitivity measured with visual field tests. FST is as an alternative to VF or ERG for assessment of retinal function in patients unable to do visual fields or with non-detectable ERGs.     

Decreased retinal–choroidal blood flow in retinitis pigmentosa as measured by MRI

Purpose

To evaluate retinal and choroidal blood flow (BF) using high-resolution magnetic resonance imaging (MRI) as well as visual function measured by the electroretinogram (ERG) in patients with retinitis pigmentosa (RP).

Methods

MRI studies were performed in 6 RP patients (29–67 years) and 5 healthy volunteers (29–64 years) on a 3-Tesla scanner with a custom-made surface coil. Quantitative BF was measured using the pseudo-continuous arterial spin-labeling technique at 0.5 × 0.8 × 6.0 mm. Full-field ERGs of all patients were recorded. Amplitudes and implicit times of standard ERGs were analyzed.

Results

Basal BF in the posterior retinal-choroid was 142 ± 16 ml/100ml/min (or 1.14 ± 0.13 μl/mm²/min) in the control group and was 70 ±19 ml/100ml/min (or 0.56 ± 0.15 μl/mm²/min) in the RP group. Retinal–choroidal BF was significantly reduced by 52 ± 8 % in RP patients compared to controls (P<0.05). ERG a- and b-wave amplitudes of RP patients were reduced, and b-wave implicit times were delayed. There were statistically significant correlations between a-wave amplitude and BF value (r=0.9, P<0.05) but not between b-wave amplitude and BF value (r =0.7, P=0.2).

Conclusions

This study demonstrates a novel non-invasive MRI approach to measure quantitative retinal and choroidal BF in RP patients. We found that retinal–choroidal BF was markedly reduced and significantly correlated with reduced amplitudes of the a-wave of the standard combined ERG.     

Establishing baseline rod electroretinogram values in achromatopsia and cone dystrophy

Purpose

To establish the normal range of values for rod-isolated b-wave amplitudes in achromatopsia and cone dystrophies.

Methods

We reviewed charts of 112 patients with various types of cone dystrophy, and compared their standardized electroretinographic rod b-wave amplitudes with age-matched normal controls. Twenty-six patients had known mutations in achromatopsia and cone dystrophy genes, while 53 were characterized by their inheritance pattern since they had yet to have their gene identified. Visual acuity information and scotomata were documented.

Results

We found that patients with achromatopsia and cone dystrophy had rod b-wave amplitudes that were significantly lower than age-matched controls, but found no evidence of rod amplitude progression nor loss of peripheral visual fields in the study group.

Conclusions

We found that cone dystrophy patients of all types had depressed rod-isolated ERGs across the board. If typical diagnostic criteria are used, these patients might be considered to have ??abnormal?? rod-isolated electroretinographic values, and might be called ??cone-rod dystrophy??, even though the waveforms are stable for years. Patients with cone-rod dysfunction patterns on ERG can be better understood by also performing kinetic (Goldmann) visual fields, which will help to distinguish cone dystrophies from progressive cone-rod dystrophies by central scotomata size and progression over time in many forms of cone-rod dystrophy.     

Multifocal electroretinography amplitudes increase after photocoagulation in areas with increased retinal thickness and hard exudates

Purpose: This study aimed to evaluate local response on multifocal electroretinography (mfERG) and to assess retinal thickness with optical coherence tomography (OCT) after focal laser treatment in areas with retinal oedema and exudates in patients with diabetic retinopathy. Methods: Twelve diabetes patients (aged 60 ± 14 years, diabetes duration 16 ± 8 years) treated with focal or grid photocoagulation in areas with retinal oedema and/or exudates underwent mfERG and OCT before and 3 months after treatment. The average thickness (in μm) in any of the nine sectors (defined according to the ETDRS) treated with photocoagulation was measured. Amplitudes and implicit times were analysed in corresponding areas on the mfERG. Results: Mean mfERG amplitudes increased after photocoagulation (21.5 ± 8.0 nV/deg² versus 16.8 ± 6.1 nV/deg²; p = 0.012), whereas no difference was seen in implicit times. Mean OCT values in the treated regions were lower at follow‐up (272 ± 23 μm versus 327 ± 79 μm; p = 0.013). No correlation was seen between changes in mfERG response and changes in OCT values. The decrease in retinal thickness was correlated with the number of laser spots applied (p = 0.002). Conclusions: Focal argon laser treatment is effective in reducing retinal thickness. In addition, treated areas tend to show improved retinal function as demonstrated by increased amplitudes on mfERG.     

Full-field electroretinogram response to increment and decrement stimuli

Purpose

The d-wave is typically elicited after the termination of an increment flash, but a decrement flash provides an alternative, and perhaps more appropriate, stimulus to elicit the d-wave. Here, we investigated the affects of stimulus polarity on the electroretinogram (ERG) response.

Methods

ERG responses elicited to increment and decrement flashes of varying intensity and duration from different background levels were measured from human participants to assess the b-wave and d-wave responses as a function of adaptation level and flash polarity. Response amplitudes were measured using standard metrics for waveform analysis.

Results

The amplitude of the b-wave is larger than the d-wave regardless of flash polarity when using different background levels which maximized the dynamic range of the two waveforms. However, when response amplitudes are measured from a common background, the d-wave elicited with decrement flash was larger than the b-wave elicited by an increment flash. This trend was evident across a range of background levels. The b-wave and d-wave become separate entities when flash duration reaches approximately 50 ms. Rapid-on and rapid-off sawtooth stimuli were also tested against increment and decrement step stimuli that were matched in mean luminance. These two stimulus types produced different amplitude b-wave and d-wave responses, suggesting asymmetric effects of the two stimulus types on the retinal response.

Conclusions

We conclude that the response properties of the b-wave and d-wave are influenced by the duration, polarity and waveform of the stimulus, as well as the background from which the stimuli arise.