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Eric J Sigler - One of the best experts on this subject based on the ideXlab platform.
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microcysts in the inner Nuclear Layer a nonspecific sd oct sign of cystoid macular edema
Investigative Ophthalmology & Visual Science, 2014Co-Authors: Eric J SiglerAbstract:Citation: Sigler EJ. Microcysts in the inner Nuclear Layer, a non-specific SDOCT sign of cystoid macular edema. Invest Ophthalmol Vis Sci. 2014;55:3282–3284. DOI:10.1167/ iovs.14-14056 Recently, many authors have propagated the notion that a specific form of ‘‘microcystic macular edema’’ occurs in patients with optic neuritis and optic atrophy of various etiology and is due to retrograde synaptic degeneration. The finding is isolated to the inner Nuclear Layer on spectral-domain optical coherence tomography in most cases, and is present in a parafoveal, circumferential, and central macular distribution. This perspective critically reviews the evidence and suggests that inner Nuclear Layer cystoid changes are an early and nonspecific indicator of typical cystoid macular edema of any cause, and that the finding is likely not a distinct entity.
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delayed onset inner Nuclear Layer cystic changes following internal limiting membrane removal for epimacular membrane
Graefes Archive for Clinical and Experimental Ophthalmology, 2013Co-Authors: Eric J Sigler, John C Randolph, Steve CharlesAbstract:Purpose To report the rare occurrence of new inner Nuclear Layer cystic spaces occurring in eyes treated with pars plana vitrectomy (PPV) and internal limiting membrane (ILM) removal for idiopathic epimacular membrane (EMM).
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delayed onset inner Nuclear Layer cystic changes following internal limiting membrane removal for epimacular membrane
Graefes Archive for Clinical and Experimental Ophthalmology, 2013Co-Authors: Eric J Sigler, John C Randolph, Steve CharlesAbstract:To report the rare occurrence of new inner Nuclear Layer cystic spaces occurring in eyes treated with pars plana vitrectomy (PPV) and internal limiting membrane (ILM) removal for idiopathic epimacular membrane (EMM). Consecutive patients with EMM without preoperative retinal cystic changes undergoing PPV with ILM peeling were retrospectively evaluated. Patients developing a characteristic inner Nuclear Layer cystic change were analyzed. Inner Nuclear Layer cystic changes appeared in eight of 768 (1.04 %) eyes at a mean postoperative time period of 3.2 ± 0.89 months. No leakage or pooling was demonstrated on postoperative fluorescein angiography. Morphologic characteristics included vertically elongated hyporeflectant spaces within the inner Nuclear Layer on spectral domain optical coherence tomography (SD-OCT). A minority of patients undergoing PPV with ILM peeling develop new, delayed onset, postoperative inner Nuclear Layer cystic spaces with a characteristic SD-OCT appearance and no evidence of angiographic leakage.
Patrik Schatz - One of the best experts on this subject based on the ideXlab platform.
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congenital stationary night blindness with hypoplastic discs negative electroretinogram and thinning of the inner Nuclear Layer
Graefes Archive for Clinical and Experimental Ophthalmology, 2016Co-Authors: Abdulaziz Al Oreany, Abdulaziz Al Hadlaq, Patrik SchatzAbstract:Purpose To describe congenital stationary night blindness (CSNB) with negative electroretinogram, hypoplastic discs, nystagmus and thinning of the inner Nuclear Layer (INL).
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congenital stationary night blindness with hypoplastic discs negative electroretinogram and thinning of the inner Nuclear Layer
Graefes Archive for Clinical and Experimental Ophthalmology, 2016Co-Authors: Abdulaziz Al Oreany, Abdulaziz Al Hadlaq, Patrik SchatzAbstract:Purpose: To describe congenital stationary night blindness (CSNB) with negative electroretinogram, hypoplastic discs, nystagmus and thinning of the inner Nuclear Layer (INL). Methods: Retinal structure was analyzed qualitatively with spectral domain optical coherence tomography and wide field imaging. Retinal function was evaluated with full-field electroretinography (ffERG). Molecular genetic testing included next-generation sequencing (NGS) of the known genes involved in CSNB. Results: Patients presented with CSNB presented with nystagmus, high myopia, hypoplastic discs and negative ffERG with no measurable rod response. The retinas appeared normal and automated segmentation of retinal Layers demonstrated a relative reduction of thickness of the INL. There was no significant change in the ffERG after prolonged 2 hour dark adaptation compared to standard 30 minute dark adaptation. Affected family members harboured the homozygous 1-bp deletion c.2394delC in exon 18 of the TRPM1 gene, whereas their unaffected parents were heterozygous carriers. Conclusions: This data expands the genotype and phenotype spectrum of CSNB. The lack of improvement of rod responses after prolonged dark adaptation, together with thinning of the INL, is compatible with postreceptoral transmission dysfunction in the bipolar cells. Such knowledge may prove useful in future development of treatment for outer retinal dystrophies, using opsin genes to restore light responses in survivor neurons in the inner retina. (Less)
Jeffrey M. Gelfand - One of the best experts on this subject based on the ideXlab platform.
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reply microcysts in the inner Nuclear Layer from optic atrophy are caused by retrograde trans synaptic degeneration combined with vitreous traction on the retinal surface
Brain, 2013Co-Authors: Ari J. Green, Daniel M. Schwartz, Jeffrey M. GelfandAbstract:ARTICLE Sir, We appreciate the thoughtful commentary from Drs Lujan and Horton (2013) based on their patient with Kjer’s dominant optic atrophy with retinal inner Nuclear Layer microcysts. Their case highlights an emerging understanding that inner Nuclear microcysts are not specific to multiple sclerosis. We—and others—have now identified them in neuromyelitis optica (Gelfand et al. , 2013; Sotirchos et al. , 2013) and they have been reported to occur in neurofibromatosis 1-associated optic nerve glioma (Abegg et al. , 2012), dominant optic atrophy and Leber’s hereditary optic neuropathy (Barboni et al. , 2013). Anecdotally, we have also heard of individual cases in patients with nerve atrophy following papilledema and traumatic optic neuropathy. However—other than for neuromyelitis optica—the frequency with which the microcysts occur in non-multiple sclerosis optic neuropathy remains unclear, and we are not aware of any cases of retinal inner Nuclear Layer microcysts that have been described in the most common optic neuropathy, glaucoma. Lujan and Horton (2013) assert that these inner Nuclear Layer microcystic changes are related to trans-synaptic cell loss based on pathological reports that highlight inner Nuclear Layer cell loss in multiple sclerosis (Gills and Wadsworth, 1967; Green et al. , 2010) and after other forms of optic nerve injury (Van …
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microcystic inner Nuclear Layer abnormalities and neuromyelitis optica
JAMA Neurology, 2013Co-Authors: Jeffrey M. Gelfand, Rachel Nolan, Sam Arnow, Bruce A C Cree, Ari J. GreenAbstract:Importance Microcystic abnormalities involving the inner Nuclear Layer of the retina occurs in a subset of patients with multiple sclerosis, most commonly in eyes previously affected by symptomatic optic neuritis. Acute optic neuritis is a cardinal manifestation of neuromyelitis optica (NMO). To our knowledge, microcystic inner Nuclear Layer abnormalities have not been investigated in NMO. Objective To establish whether microcystic inner Nuclear Layer abnormalities occur in NMO. Design Observational, retrospective study. Setting University of California at San Francisco Multiple Sclerosis Center (academic specialty clinic). Patients Twenty-five consecutive patients with NMO based on 2006 diagnostic criteria or with NMO spectrum disease (defined by seropositivity for anti–aquaporin 4 IgG in the context of a single episode of transverse myelitis or optic neuritis). Exposure Spectral-domain optical coherence tomography. Main Outcomes and Measures Identification of microcystic inner Nuclear Layer pathology on spectral-domain optical coherence tomography. Multivariable linear regression was used to examine associations between microcystic changes and measures of retinal structure and function. The hypothesis was generated prior to the data being reviewed and analyzed. Results Microcystic changes were identified in 5 of 25 patients with NMO (20%) and 7 of 48 total eyes, including 7 of 29 eyes (24%) previously affected by optic neuritis. Microcystic changes occurred exclusively in eyes with a history of acute symptomatic optic neuritis (100% of eyes with microcystic changes had experienced prior optic neuritis compared with 71% of NMO eyes without microcystic abnormalities). There were no significant differences between patients with NMO with and without microcystic changes in terms of age, sex, and aquaporin 4–IgG antibody status. The mean age in this cohort was 44 years (range, 13-81 years); 84% were women; 80% were aquaporin 4–IgG seropositive; and the median Expanded Disability Status Scale score was 4.0 (interquartile range, 3.0-6.5). Conclusions and Relevance Microcystic inner Nuclear Layer pathology occurs in a proportion of patients with NMO in eyes previously affected by acute optic neuritis. Additional research is needed to understand the cause of this retinal pathology and determine whether it contributes to persistent visual disability in patients with NMO following optic neuritis.
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microcystic macular oedema thickness of the inner Nuclear Layer of the retina and disease characteristics in multiple sclerosis a retrospective study
Lancet Neurology, 2012Co-Authors: Shiv Saidha, Elias S Sotirchos, Michaela A Seigo, Yasir J. Sepah, John N Ratchford, Jiwon Oh, Ciprian M. Crainiceanu, Jeffrey M. Gelfand, Mohamed Ibrahim, Scott D NewsomeAbstract:Summary Background Microcystic macular oedema (MMO) of the retinal inner Nuclear Layer (INL) has been identified in patients with multiple sclerosis (MS) by use of optical coherence tomography (OCT). We aimed to determine whether MMO of the INL, and increased thickness of the INL are associated with disease activity or disability progression. Methods This retrospective study was done at the Johns Hopkins Hospital (Baltimore, MD, USA), between September, 2008, and March, 2012. Patients with MS and healthy controls underwent serial OCT scans and clinical assessments including visual function. OCT scanning, including automated intraretinal Layer segmentation, yielded thicknesses of the retinal nerve fibre Layer, the ganglion cell Layer plus inner plexiform Layer, the INL plus outer plexiform Layer (the combined thickness of these Layers was used as a surrogate measure of INL thickness), and the outer Nuclear Layer. Patients with MS also underwent annual brain MRI scans. Disability scores were compared with the Wilcoxon rank-sum test. Mixed-effects linear regression was used to compare OCT measures and letter-acuity scores. Logistic regression was used to examine the relations of baseline OCT thicknesses with clinical and radiological parameters. Findings 164 patients with MS and 60 healthy controls were assessed. Mean follow-up was 25·8 months (SD 9·1) for patients with MS and 22·4 months (11·4) for healthy controls. Ten (6%) patients with MS had MMO during at least one study visit; MMO was visible at baseline in four of these patients. Healthy controls did not have MMO. Patients with MS and MMO had higher baseline MS severity scores (median 5·93 [range 2·44–8·91]) than those who did not have MMO at any time during the study (151 patients; 3·81 [0·13–9·47]; p=0·032), although expanded disability status scale (EDSS) scores were not significantly different (5·2 [1·0–6·5] for patients with MS and MMO vs 2·5 [0·0–8·0] for those without MMO; p=0·097). The eyes of patients with MS and MMO (12 eyes) versus those without MMO (302 eyes) had lower letter-acuity scores (100% contrast, p=0·017; 2·5% contrast, p=0·031; 1·25% contrast, p=0·014), and increased INL thicknesses (p=0·003) at baseline. Increased baseline INL thickness in patients with MS was associated with the development of contrast-enhancing lesions (p=0·007), new T2 lesions (p=0·015), EDSS progression (p=0·034), and relapses in patients with relapsing-remitting MS (p=0·008) during the study. MMO was not associated with disease activity during follow-up. Interpretation Increased INL thickness on OCT is associated with disease activity in MS. If this finding is confirmed, INL thickness could be a useful predictor of disease progression in patients with MS. Funding National Multiple Sclerosis Society, National Eye Institute, Braxton Debbie Angela Dillon and Skip Donor Advisor Fund.
Abdulaziz Al Oreany - One of the best experts on this subject based on the ideXlab platform.
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congenital stationary night blindness with hypoplastic discs negative electroretinogram and thinning of the inner Nuclear Layer
Graefes Archive for Clinical and Experimental Ophthalmology, 2016Co-Authors: Abdulaziz Al Oreany, Abdulaziz Al Hadlaq, Patrik SchatzAbstract:Purpose To describe congenital stationary night blindness (CSNB) with negative electroretinogram, hypoplastic discs, nystagmus and thinning of the inner Nuclear Layer (INL).
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congenital stationary night blindness with hypoplastic discs negative electroretinogram and thinning of the inner Nuclear Layer
Graefes Archive for Clinical and Experimental Ophthalmology, 2016Co-Authors: Abdulaziz Al Oreany, Abdulaziz Al Hadlaq, Patrik SchatzAbstract:Purpose: To describe congenital stationary night blindness (CSNB) with negative electroretinogram, hypoplastic discs, nystagmus and thinning of the inner Nuclear Layer (INL). Methods: Retinal structure was analyzed qualitatively with spectral domain optical coherence tomography and wide field imaging. Retinal function was evaluated with full-field electroretinography (ffERG). Molecular genetic testing included next-generation sequencing (NGS) of the known genes involved in CSNB. Results: Patients presented with CSNB presented with nystagmus, high myopia, hypoplastic discs and negative ffERG with no measurable rod response. The retinas appeared normal and automated segmentation of retinal Layers demonstrated a relative reduction of thickness of the INL. There was no significant change in the ffERG after prolonged 2 hour dark adaptation compared to standard 30 minute dark adaptation. Affected family members harboured the homozygous 1-bp deletion c.2394delC in exon 18 of the TRPM1 gene, whereas their unaffected parents were heterozygous carriers. Conclusions: This data expands the genotype and phenotype spectrum of CSNB. The lack of improvement of rod responses after prolonged dark adaptation, together with thinning of the INL, is compatible with postreceptoral transmission dysfunction in the bipolar cells. Such knowledge may prove useful in future development of treatment for outer retinal dystrophies, using opsin genes to restore light responses in survivor neurons in the inner retina. (Less)
Jonathan C Horton - One of the best experts on this subject based on the ideXlab platform.
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microcysts in the inner Nuclear Layer from optic atrophy are caused by retrograde trans synaptic degeneration combined with vitreous traction on the retinal surface
Brain, 2013Co-Authors: Brandon J Lujan, Jonathan C HortonAbstract:ARTICLE Sir, it has been reported that microcysts from macular oedema occur in 4.7% of patients with multiple sclerosis (Gelfand et al. , 2012). It is now evident that these microcysts do not represent oedema, nor is their occurrence a specific feature of demyelinating disease, because they have been identified in patients with Leber’s hereditary optic neuropathy, Kjer’s dominant optic atrophy, and optic glioma (Abegg et al. , 2012; Barboni et al. , 2013). However, their precise aetiology has remained a mystery. As pointed out by Abegg et al. (2012), the classical ocular pathology literature provides a clue to the formation of microcysts. Van Buren (1963) described retrograde trans-synaptic degeneration of the inner Nuclear Layer in histological sections of the retina after lesions of the optic nerve or chiasm, causing ‘cystic degeneration’ that resembles the ‘microcysts’ now being imaged with optical coherence tomography. Gills and Wadsworth (1967) found a 15–60% reduction in cell population in the inner Nuclear Layer in nine patients with longstanding blindness from compressive or traumatic lesions of the optic nerve. In at least three cases, microcycsts were present in the inner Nuclear Layer (Fig. 1). In two patients with more recent blindness, cell loss was not observed. Presumably, sufficient time had not elapsed for trans-synaptic degeneration to occur. Figure 1 ( A ) Histological section of the retina showing cysts in the inner Nuclear Layer following retrograde trans-synaptic degeneration from a tumour of the optic nerve. Reproduced from Gills and Wadsworth (1967) with permission from James P. Gills. ( B ) Image obtained by optical coherence tomography from a patient with optic atrophy, showing cysts in the inner Nuclear Layer. IPL = inner plexiform Layer; INL = inner Nuclear Layer; OPL = outer plexiform Layer; HFL = Henle fibre Layer; ONL = outer Nuclear Layer. Gills and Wadsworth (1967) also …
