Novel biallelic splice-site BBS1 variants in Bardet –Biedle syndrome: a case report of the first Japanese patient
ConclusionsThis is the first report of a BBS patient with biallelic splice-siteBBS1 variants in the Japanese population. Disparity between funduscopic and ERG findings may be a feature ofBBS1-associated rod-cone dystrophy. (Source: Documenta Ophthalmologica)
Source: Documenta Ophthalmologica - January 28, 2020 Category: Opthalmology Source Type: research
ERG shrinks by 10% when reducing dark adaptation time to 10 min, but only for weak flashes
ConclusionReducing dark adaptation time from 20 to 10 min in normal participants has no effect on the ISCEV DA 3 and DA 10 ERG. The reduction in DA 0.01 ERGs to 87 ± 2% agrees with Hamilton and Graham (Doc Ophthalmol 133:11–19, 2016.https://doi.org/10.1007/s10633-016-9554-x) who found 90 ± 2% and with Asakawa et al. (Doc Ophthalmol 139:33–44, 2019.https://doi.org/10.1007/s10633-019-09693-8) who found 83%. Pending verification in pathophysiological states, the current results suggest that one might be able to correct for the 10% amplitude loss when gaining 10 min through shortened DA. (Source: Documenta Ophthalmologica)
Source: Documenta Ophthalmologica - January 28, 2020 Category: Opthalmology Source Type: research
Evaluation of retinal fiber thickness and visual pathways with optic coherence tomography and pattern visual evoked potential in different clinical stages of obstructive sleep apnea syndrome
ConclusionsIn mild and severe stages of the disease, edema and inflammation were evident and VEP PT and amplitudes were affected in both groups. Furthermore, thinning in RNFL in the severe stage of the disease might be associated with higher atrophy levels and prolonged exposure to hypoxia. (Source: Documenta Ophthalmologica)
Source: Documenta Ophthalmologica - January 23, 2020 Category: Opthalmology Source Type: research
Analysis of retinal structure and function in cone dystrophy with supernormal rod response
ConclusionThis is the largest cohort of CDSRR from a single ethnic background. Rod dysfunction and reduced 30-Hz flicker response were demonstrated in all patients. In contrast to previous descriptions in the literature, a supernormal combined dark-adapted rod-cone ERG was present in the majority of the patients at standard stimulus intensity. Considering the consistent genotype and the demonstration of likely pathogenic genetic variants in all the patients, we argue that the combination of delayed rod b-wave and subnormal flicker response strongly suggests the diagnosis of CDSRR. (Source: Documenta Ophthalmologica)
Source: Documenta Ophthalmologica - January 19, 2020 Category: Opthalmology Source Type: research
Acknowledgement to scientific referees 2019
(Source: Documenta Ophthalmologica)
Source: Documenta Ophthalmologica - January 12, 2020 Category: Opthalmology Source Type: research
Electrophysiological and SD-OCT findings in patients receiving chloroquine therapy in relation to cumulative dosage and duration of treatment
ConclusionsThis study shows a strong association between mfERG ring values and the corresponding OCT thickness measures; however, mfERG may enhance early detection of functional changes in patients treated with chloroquine, especially in ambiguous cases. At low chloroquine cumulative dosages, different subjects might have different susceptibilities to the drug. (Source: Documenta Ophthalmologica)
Source: Documenta Ophthalmologica - January 10, 2020 Category: Opthalmology Source Type: research
Late presentation of RPE65 retinopathy in three siblings
ConclusionsRPE65 retinopathy seems to be relatively common on the Arabian peninsula, and in addition it may be underdiagnosed. To the best of our knowledge, this is the first detailed presentation, including multimodal retinal imaging and electrophysiological assessment, of such patients from this region. Patients with late presentation ofRPE65 retinopathy may be eligible for gene therapy, in terms of remaining retinal function and structural preservation. The therapeutic window of such therapy remains to be determined. (Source: Documenta Ophthalmologica)
Source: Documenta Ophthalmologica - January 9, 2020 Category: Opthalmology Source Type: research
Multifocal electroretinography in patients with macular telangiectasia type 2
ConclusionmfERG reflects localised inner retinal dysfunction in MacTel type 2. Correlation with BCVA reinforces that mfERG should be used to objectively assess the macular function in these patients. (Source: Documenta Ophthalmologica)
Source: Documenta Ophthalmologica - January 6, 2020 Category: Opthalmology Source Type: research
A novel method for electroretinogram assessment in patients with central retinal vein occlusion
ConclusionThe proposed features have made it possible to distinguish between healthy and CRVO eyes. This method could be helpful in some cases with no definite diagnosis or to estimate the severity of CRVO. (Source: Documenta Ophthalmologica)
Source: Documenta Ophthalmologica - January 6, 2020 Category: Opthalmology Source Type: research
ISCEV extended protocol for derivation and analysis of the strong flash rod-isolated ERG a-wave
AbstractThe International Society for the Clinical Electrophysiology of Vision (ISCEV) standard for full-field electroretinography (ERG) describes a minimum set of tests, but encourages the use of additional protocols for clinical ERG testing. This extended protocol describes recording methods and derivations that will allow analysis of rod-driven components of the dark-adapted (DA) strong flash ERG a-wave, more closely related to rod phototransduction than ISCEV standard DA ERGs. The method involves recording ERGs to a flash strength equivalent to 30 cd s m2 under conditions of dark adaptation and additionally to the ...
Source: Documenta Ophthalmologica - January 3, 2020 Category: Opthalmology Source Type: research
Electroretinographic abnormalities associated with pregabalin: a case report
ConclusionsThis is the first report to indicate that ERG abnormalities might be associated with pregabalin treatment. Our results suggest that pregabalin may affect LTCC function via theα2 –δ1/α2 –δ2 subunits, which leads to defective synaptic transmission from rod/cone photoreceptors to bipolar cells. (Source: Documenta Ophthalmologica)
Source: Documenta Ophthalmologica - January 2, 2020 Category: Opthalmology Source Type: research
A new electrophysiological non-invasive method to assess retinocortical conduction time in the Dark Agouti rat through the simultaneous recording of electroretinogram and visual evoked potential
ConclusionsSimultaneous recording of ERG and VEP has been previously achieved through invasive methods requiring surgery. Here, we present a new non-invasive method, which allowed to obtain peak and retinocortical times that were constant across a long period and had a good repeatability over time. This method will ensure not only a gain in animal welfare, but will also avoid stress and eye or brain lesions which can interfere with experimental variables. (Source: Documenta Ophthalmologica)
Source: Documenta Ophthalmologica - December 11, 2019 Category: Opthalmology Source Type: research
Electroretinograms of eyes with Stickler syndrome
ConclusionsThe reduced or absent full-field ERGs in eyes with Stickler syndrome indicate that the physiology of the entire retina was negatively altered. The greater reduction in the ERGs with increasing age suggests that the physiological alterations of the retina are progressive. (Source: Documenta Ophthalmologica)
Source: Documenta Ophthalmologica - November 27, 2019 Category: Opthalmology Source Type: research
Foveation dynamics in congenital nystagmus IV: vergence
ConclusionsTarget foveation qualities during fixation of targets at various near distances were equivalent to that during fixation of a far target. Stereo discrimination was limited by the foveation quality of the eye with the higher NAFX waveform. Foveation period slopes during vergence tracking demonstrated vergence movements despite the ongoing CN oscillation. Similar to what we found with fixation, pursuit, and the vestibulo-ocular systems, these findings establish that vergence in both static and dynamic viewing conditions functions normally in the presence of the CN oscillation. (Source: Documenta Ophthalmologica)
Source: Documenta Ophthalmologica - November 26, 2019 Category: Opthalmology Source Type: research
ISCEV extended protocol for the S-cone ERG
AbstractThe International Society for Clinical Electrophysiology of Vision (ISCEV) standard for full-field electroretinography (ERG) describes a minimum procedure for testing generalized retinal function but encourages more extensive testing. This extended protocol describes a method of assessing the function of the short-wavelength-sensitive cone (S-cone) retinal pathway, using a short-wavelength flash superimposed on a background that saturates the rods and adapts the L/M-cones to elicit a response, known as the S-cone ERG. Stimulus parameters such as the strength and luminance of the flash and background, respectively, ...
Source: Documenta Ophthalmologica - November 19, 2019 Category: Opthalmology Source Type: research
