Early Optic Nerve Research Articles

Serotonin neuromodulation directs optic nerve regeneration.

Optic nerve (ON) regeneration in mammalian systems is limited by an overshadowing dominance of inhibitory factors. This has severely hampered the identification of pro-regenerative pathways. Here, we take advantage of the regenerative capacity of larval zebrafish to identify pathways that promote ON regeneration. From a small molecule screen, we identified modulators of serotonin (5-HT) signaling that inhibit ON regeneration. We find several serotonin type-1 receptor genes are expressed in RGC neurons during regeneration and that inhibiting 5-HT1 receptors or components of the 5-HT pathway selectively impedes ON regeneration. We show that 5-HT1 receptor signaling is dispensable during ON development yet is critical for regenerating axons to emerge from the injury site. Blocking 5-HT receptors once ON axons have crossed the chiasm does not inhibit regeneration, suggesting a selective role for 5-HT receptor signaling early during ON regeneration. Finally, we show that agonist-mediated activation of 5-HT1 receptors leads to enhanced and ectopic axonal regrowth. Combined, our results provide evidence for mechanisms through which serotonin-dependent neuromodulation directs ON regeneration in vivo.

The Effect of Hydrocephalus on the Optic Nerve in the Presence of Intracranial Mass

The measurement of optic nerve sheath diameter is a noninvasive, practical, and economical method used to identify increased intracranial pressure. The purpose of this study is to detect the preoperative and postoperative changes in optic nerve sheath diameter in patients with intracranial mass, to correlate these changes with optic nerve diameter variations, and to evaluate the impact of hydrocephalus on these alterations. This study was conducted with patients who presented to our clinic with complaints of intracranial mass, were decided for surgery, and underwent surgical procedures. The optic nerve and optic nerve sheath diameter measurement values were different preoperatively and postoperatively, with a significant decrease in the optic nerve sheath diameter in all groups in postoperative measurements, while the optic nerve diameter significantly increased. Although there was no significant difference between the effects of hydrocephalus and intracranial mass-related increase in intracranial pressure on the optic nerve and optic nerve sheath, it was observed that hydrocephalus increased intracranial pressure when considering the Evans ratio. It has been determined that as ventricular dilatation increases, so does intracranial pressure, which leads to an increase in the diameter of the optic nerve sheath, resulting in papilledema and thinning of the optic nerve. These findings indicate the importance of early cerebrospinal fluid diversion and monitoring optic nerve sheath diameter in the management.

Visual conservation treatment dilemmas in neuroblastoma with bilateral blindness

ObjectiveTo investigate the clinical features, treatment strategies, and prognosis of neuroblastoma with bilateral blindness.MethodsThe clinical data of five patients with bilateral blindness neuroblastoma admitted to Beijing Children’s Hospital from April 2018 to September 2020 were retrospectively collected to summarize their clinical characteristics.ResultsAll patients were female and the median age at presentation was 25 (23, 41) months. The median intervention time from the onset of symptoms of bilateral blindness to the start of treatment was 10 (10, 12) days. All five cases were staged as stage M and grouped as high risk. Four cases were MYCN gene amplification and one case was MYCN acquisition. Five children were treated according to a high-risk neuroblastoma treatment protocol. Four children did not recover their vision after treatment, and one case improved to have light perception. All patients were effectively followed up for a median of 20 (12, 31) months, with three deaths, one tumor-free survival, and one recurrent tumor-bearing survival.ConclusionNeuroblastoma with bilateral blindness is rare in the clinic, mostly in children of young age, and is often associated with MYCN amplification and multiple metastases. Early hormone shock therapy and optic nerve decompression are beneficial for preserving the child’s vision. A joint multi-disciplinary treatment may help in the formulation of treatment decisions. Achieving a balance between good visual preservation and survival within the short optic nerve neurotherapeutic window is extremely challenging.

Optic nerve sheath fenestration: A second lease at sight.

To study the safety and efficacy of optic nerve sheath fenestration surgery in patients with optic disc edema due to different etiologies. Records of 18 eyes of 15 patients who underwent optic nerve sheath fenestration for vision threatening optic disc edema were reviewed retrospectively, and results were analyzed. Improvement of visual acuity was the main measure of outcome. Improved visual fields, resolution of optic disc edema, diplopia, and headache were other benefits that were observed. Fifteen patients between 13 and 54 years of age were included in the study. Three patients underwent successive bilateral surgery. Idiopathic intracranial hypertension was the most common cause for optic disc edema and was found in 80% of the patients. Mean preoperative logMAR acuity was -1.9789 ± 1.46270, which improved to -0.9022 ± 1.23181 (p < 0.005) in the operated eye, and mean logMAR acuity of contralateral eye improved from -1.3378 ± 1.50107 to -1.0667 ± 1.33813 (p < 0.05). Early optic nerve sheath fenestration is an effective modality for treating optic disc edema due to a wide myriad of causes and helps resolve the associated symptoms.

Optical coherence tomography detects early optic nerve damage before visual field defect in patients with pituitary tumors.

Optical coherence tomography (OCT) is a useful tool for predicting visual recovery after the removal of pituitary tumors. However, the utility of OCT in patients with pituitary tumors and a normal visual field is unclear. We aimed to analyze OCT features in pituitary tumors without visual field defects. Pituitary tumors without visual field defects were selected. A total of 138 eyes from 69 patients, assessed by the Humphrey visual field test and OCT, were enrolled in this study. Using preoperative coronal sections of MR images, patients were divided into chiasmal compression (CC) and non-chiasmal compression (non-CC) groups, and OCT characteristics were examined. The CC and non-CC groups consisted of 40 and 29 patients, respectively. There were no differences in age, sex, tumor type, or degree of visual field testing, but the tumor size was different between the two groups. On OCT, macular thickness ganglion cell complex (mGCC) was significantly thinner in the CC group than that in the non-CC group (112.5 vs 117.4 um, P < 0.05). Based on a database of healthy participants, 24% and 2% of eyes in the CC and non-CC groups had abnormal mGCC thickness (P < 0.01), respectively. In a sub-analysis of the CC group, patients with an abnormal mGCC thickness were older than a normal one (58.2 vs 41.1years, p < 0.01). OCT can detect early optic nerve damage due to optic CC by pituitary tumors, even in normal visual fields. The degree of mGCC thinning may provide an appropriate surgical timing for pituitary tumors that compress the optic chiasm.

Ultrastructural characteristics of oligodendrocyte precursor cells in the early postnatal mouse optic nerve observed by serial block-face scanning electron microscopy.

Oligodendrocyte precursor cells (OPC) arise from restricted regions of the central nervous system (CNS) and differentiate into myelin-forming cells after migration, but their ultrastructural characteristics have not been fully elucidated. This study examined the three-dimensional ultrastructure of OPCs in comparison with other glial cells in the early postnatal optic nerve by serial block-face scanning electron microscopy. We examined 70 putative OPCs (pOPC) that were distinct from other glial cells according to established morphological criteria. The pOPCs were unipolar in shape with relatively few processes, and their Golgi apparatus were localized in the perinuclear region with a single cisterna. Astrocytes abundant in the optic nerve were distinct from pOPCs and had a greater number of processes and more complicated Golgi apparatus morphology. All pOPCs and astrocytes contained a pair of centrioles (basal bodies). Among them, 45% of pOPCs extended a short cilium, and 20% of pOPCs had centrioles accompanied by vesicles, whereas all astrocytes with basal bodies had cilia with invaginated ciliary pockets. These results suggest that the fine structures of pOPCs during the developing and immature stages may account for their distinct behavior. Additionally, the vesicular transport of the centrioles, along with a short cilium length, suggests active ciliogenesis in pOPCs.

Astrocyte heterogeneity within white matter tracts and a unique subpopulation of optic nerve head astrocytes.

Much of what we know about astrocyte form and function is derived from the study of gray matter protoplasmic astrocytes, whereas white matter fibrous astrocytes remain relatively unexplored. Here, we used the ribotag approach to isolate ribosome-associated mRNA and investigated the transcriptome of uninjured fibrous astrocytes from three regions: unmyelinated optic nerve head, myelinated optic nerve proper, and corpus callosum. Astrocytes from each region were transcriptionally distinct and we identified region-specific astrocyte genes and pathways. Energy metabolism, particularly oxidative phosphorylation and mitochondrial protein translation emerged as key differentiators of astrocyte populations. Optic nerve astrocytes expressed higher levels of neuroinflammatory pathways than corpus callosum astrocytes and we further identified CARTPT as a new marker of optic nerve head astrocytes. These previously uncharacterized transcriptional profiles of white matter astrocyte types reveal their functional diversity and a greater heterogeneity than previously appreciated.

The effect of postmenopausal hormonal drop on optic nerve head and peripapillary perfusion using optical coherence tomography angiography (OCTA)

We studied the effect of menopause with subsequent estrogen drop on optic nerve head structure and peripapillary vasculature. This cross-sectional analytic study was carried out on 100 eyes of 100 patients; patients were divided into a premenopausal group (50 eyes) and a postmenopausal group (50 eyes). Optical coherence tomography was done to evaluate retinal nerve fiber layer thickness (RNFLT) and optical coherence tomography angiography (OCTA) to assess the peripapillary capillary vessel density. RNFLT as well as the peripapillary vessel density (VD) were significantly lower in the postmenopausal group (P value < 0.001) with increasing age, hormonal drop, and higher intraocular pressure (IOP), specifically in the inferior quadrant. However, the negative correlation between IOP and VD (r = − 0.541) was stronger than its negative correlation with RNFLT (r = − 0.318). Postmenopausal hormonal changes lead to a significant rise in IOP-although still not glaucomatous- and a decrease in the RNFLT and perfusion of the optic nerve. This confirms the relation between hormonal drop and glaucoma in postmenopausal women. Changes in peripapillary vascular density were more evident than RNFL in correlation with IOP and age changes. So, OCTA can be used to detect early optic nerve affection.

Pigmented Lamina Cribrosa

An 11-year-old Asian-American boy presented for evaluation of optic nerve pigmentation. Vision was 20/20 in each eye without correction. Color fundus photography of the right eye revealed a cup-to-disc ratio of 0.6, and a pigmented cribriform structure in the optic cup. We believe this represents melanocytic pigmentation of the scleral fibers of the lamina cribrosa. It could also be a very early optic nerve melanocytoma. However, melanocytoma has been described as a pigmented lesion that obscures part or all of the optic disc.

Functional and Morphological Changes in the Visual Pathway in Patients with Graves' Orbitopathy.

Background: The aim of the study was to perform a functional and structural evaluation of the anterior visual pathway in patients with Graves’ Orbitopathy (GO) using electrophysiological tests and OCT, as well as to identify potential parameters that could be useful in detecting early optic nerve damage. Methods: 47 GO patients were enrolled in the study and divided into three groups, depending on their disease severity: Group 1 with mild GO, Group 2 with moderate-to-severe GO, and Group 3 with dysthyroid optic neuropathy (DON). Pattern visual evoked potential (PVEP), flash visual evoked potential (fVEP), pattern electroretinogram (pERG), and optical coherence tomography (OCT) findings were compared between the groups. Results: In the DON Group (Group 3), N75, P100, and P2 latencies were significantly extended, whereas P100, P50, and N95 amplitudes were significantly reduced as compared to the non-DON group (Groups 1 and 2). Group 3 also had significantly thinner peripapillary retinal nerve fiber layer (RNFL) and macular ganglion cell complex (GCC). In Group 2, as compared to Group 1, P100 amplitudes were significantly reduced for all check sizes, while P100 latency was elongated for the check size of 0.9°. Group 2 also had a significantly thinner average GCC and GCC in the superior quadrant. Conclusions: Electrophysiological examinations may be of use in diagnosis of DON. OCT findings and electrophysiological responses vary in patients with different GO severity. Including regular electrophysiological evaluation and OCT in the examination of patients with GO could be of benefit. However, more research is needed to establish the true significance of pVEP, fVEP, pERG, and OCT in monitoring patients with GO.

Super-Resolution Ultrasound Localization Microscopy for Visualization of the Ocular Blood Flow.

The ocular vascular system plays an important role in preserving the visual function. Alterations in either anatomy or hemodynamics of the eye may have adverse effects on vision. Thus, an imaging approach that can monitor alterations of ocular blood flow of the deep eye vasculature ranging from capillary-level vessels to large supporting vessels would be advantageous for detection of early stage retinal and optic nerve diseases. We propose a super-resolution ultrasound localization microscopy (ULM) technique that can assess both the microvessel and flow velocity of the deep eye with high resolution. Ultrafast plane wave imaging was acquired using an L22-14v linear array on a high frequency Verasonics Vantage system. A robust microbubble localization and tracking technique was applied to reconstruct ULM images. The experiment was first performed on pre-designed flow phantoms in vitro and then tested on a New Zealand white rabbit eye in vivo calibrated to various intraocular pressures (IOP) - 10 mmHg, 30 mmHg and 50 mmHg. We demonstrated that retinal/choroidal vessels, central retinal artery, posterior ciliary artery, and vortex vein were all visible at high resolution. In addition, reduction of vascular density and flow velocity were observed with elevated IOPs. These results indicate that super-resolution ULM is able to image the deep ocular tissue while maintaining high resolution that is comparable with optical coherence tomography angiography. Capability to detect subtle changes of blood flow may be clinically important in detecting and monitoring eye diseases such as glaucoma.

Early corneal and optic nerve changes in a paediatric population affected by obstructive sleep apnea syndrome.

The relation between OSAS and eye diseases is well known in adults, while very few and contradictory data can be found regarding paediatric ages. The aim of this study is to explore the early corneal, macular and optic nerve changes in paediatric patients with OSAS. Prospective study that enrolled children aged ≥ 4years referred to the Paediatric Pneumology Clinic in Verona for suspected obstructive sleep apnoea syndrome (OSAS) and investigated with the overnight respiratory polygraphy. Patients with apnoea-hypopnea index (AHI) > 1 were classified as OSAS, while those with AHI < 1 were classified non-OSAS. All patients underwent comprehensive eye examination including slit lamp, refraction, intraocular pression (Goldman applanation tonometry), corneal tomography (corneal astigmatism, corneal keratometry at the apex, surface asymmetry index, central corneal thickness and thinnest corneal thickness) and optical coherence tomography (central macular thickness, macular volume and retinal nerve fibre layer). Seventy-two children were enrolled in the study. The overall prevalence of OSAS was 48.6%. Statistically significant differences were found between OSAS and non-OSAS group for corneal asymmetry (0.9 ± 0.5 and 0.6 ± 0.3, respectively; p = 0.02), thinnest corneal thickness (551.8 ± 33.9 and 563.7 ± 32.5; p = 0.04), average retinal nerve fibre layer (102.8 ± 10.5µm and 98.1 ± 12.3µm; p = 0.012) and in nasal quadrant (76.2 ± 15.4µm and 66.5 ± 12.6µm; p = 0.0002). A comprehensive eye examination with corneal and optic nerve imaging showed early corneal and optic nerve changes in children newly diagnosed with OSAS. These could be prelude of the known ocular manifestations associated with OSAS in adult patients.

Pathogenic NR2F1 variants cause a developmental ocular phenotype recapitulated in a mutant mouse model.

Pathogenic NR2F1 variants cause a rare autosomal dominant neurodevelopmental disorder referred to as the Bosch–Boonstra–Schaaf Optic Atrophy Syndrome. Although visual loss is a prominent feature seen in affected individuals, the molecular and cellular mechanisms contributing to visual impairment are still poorly characterized. We conducted a deep phenotyping study on a cohort of 22 individuals carrying pathogenic NR2F1 variants to document the neurodevelopmental and ophthalmological manifestations, in particular the structural and functional changes within the retina and the optic nerve, which have not been detailed previously. The visual impairment became apparent in early childhood with small and/or tilted hypoplastic optic nerves observed in 10 cases. High-resolution optical coherence tomography imaging confirmed significant loss of retinal ganglion cells with thinning of the ganglion cell layer, consistent with electrophysiological evidence of retinal ganglion cells dysfunction. Interestingly, for those individuals with available longitudinal ophthalmological data, there was no significant deterioration in visual function during the period of follow-up. Diffusion tensor imaging tractography studies showed defective connections and disorganization of the extracortical visual pathways. To further investigate how pathogenic NR2F1 variants impact on retinal and optic nerve development, we took advantage of an Nr2f1 mutant mouse disease model. Abnormal retinogenesis in early stages of development was observed in Nr2f1 mutant mice with decreased retinal ganglion cell density and disruption of retinal ganglion cell axonal guidance from the neural retina into the optic stalk, accounting for the development of optic nerve hypoplasia. The mutant mice showed significantly reduced visual acuity based on electrophysiological parameters with marked conduction delay and decreased amplitude of the recordings in the superficial layers of the visual cortex. The clinical observations in our study cohort, supported by the mouse data, suggest an early neurodevelopmental origin for the retinal and optic nerve head defects caused by NR2F1 pathogenic variants, resulting in congenital vision loss that seems to be non-progressive. We propose NR2F1 as a major gene that orchestrates early retinal and optic nerve head development, playing a key role in the maturation of the visual system.

Approaches to Potentiated Neuroprotective Treatment in the Rodent Model of Ischemic Optic Neuropathy.

Nonarteritic anterior ischemic optic neuropathy (NAION) commonly causes sudden optic nerve (ON)-related vision loss. The rodent NAION model (rAION) closely resembles NAION in presentation and physiological responses. We identified early rAION-associated optic nerve head (ONH) inflammatory gene expression responses and the anti-inflammatory prostaglandin PGJ2’s effects on those responses. We hypothesized that blocking pro-inflammatory prostaglandin (PGE2) production by inhibiting monoacylglycerol lipase or cyclooxygenase activity and co-administering PGJ2 would potentiate RGC survival following ischemic neuropathy. Deep sequencing was performed on vehicle- and PGJ2-treated ONHs 3d post-rAION induction. Results were compared against responses from a retinal ischemia model. Animals were treated with PGJ2 and MAGL inhibitor KML29, or PGJ2 + COX inhibitor meloxicam. RGC survival was quantified by stereology. Tissue PG levels were quantified by ELISA. Gene expression was confirmed by qPCR. PGJ2 treatment nonselectively reduced inflammatory gene expression post-rAION. KML29 did not reduce PGE2 1d post-induction and KML29 alone increased RGC loss after rAION. Combined treatments did not improve ONH edema and RGC survival better than reported with PGJ2 alone. KML29′s failure to suppress PGE2 ocular synthesis, despite its purported effects in other CNS tissues may result from alternative PG synthesis pathways. Neither KML29 nor meloxicam treatment significantly improved RGC survival compared with vehicle. While exogenous PGJ2 has been shown to be neuroprotective, treatments combining PGJ2 with these PG synthesis inhibitors do not enhance PGJ2’s neuroprotection.

Early effects of viper envenomation on retina and optic nerve blood flow: An optical coherence tomography angiography study

In this study, the early retinal and optic nerve blood flows of patients exposed to Viper bite were evaluated with non-invasive optical coherence tomography angiography (OCTA) and compared with healthy controls. The retinal and optic disc OCTA data of 31 victims of viper bite (group S) without systemic envenomation clinical symptoms and 31 healthy controls (group C) were compared. Only patients with early signs of envenomation were included in the study. Optical coherence tomography angiographies were performed with RTVue XR Avanti with AngioVue software. Vascular densities in the whole image, foveal, parafoveal regions at the superficial and the deep capillary plexus segments were acquired and statistically analyzed. The flow area parameters were measured in the superficial retinal capillary plexus, deep retinal capillary plexus, outer retinal capillary plexus, and choriocapillaris layers of the macula in 1-mm and 3-mm diameter areas. The peripapillary flow areas were measured for the optic nerve head, vitreous, radial peripapillary capillary (RPC), and choroid in a 4.50-mm diameter area. Foveal and parafoveal thicknesses were also measured and compared. The average hospital admission time of the patients in group S was 1.24 ± 0.75 (0.50–3.00) hours. Age (p = 0.103) and gender (p = 0.714) were similar in both groups. Superficial (p = 0.010), deep flow areas (p = 0.034), and superficial parafoveal vascular density (p = 0.001) were significantly reduced in group S compared to group C. The outer retinal flow area (p < 0.001) increased significantly in group S. Nerve head flow area (p = 0.035), one of the optic disc flow areas, was found to be decreased in group S. Notably, foveal (p < 0.001) and parafoveal (p = 0.003) thicknesses and superficial (p = 0.001) and deep (p < 0.001) foveal vascular densities were greater in group S. Compared to group C, the superficial (p = 0.009) and deep (p = 0.009) foveal flow areas in the central foveal area with a diameter of 1 mm increased significantly in group S. Viper venom may cause blood flow changes in the retina and optic disc and an increase in retinal thickness in the early period although there are no signs of systemic envenomation.

Longitudinal assessment of optic nerve head changes using optical coherence tomography in a primate microbead model of ocular hypertension

In humans, the longitudinal characterisation of early optic nerve head (ONH) damage in ocular hypertension (OHT) is difficult as patients with glaucoma usually have structural ONH damage at the time of diagnosis. Previous studies assessed glaucomatous ONH cupping by measuring the anterior lamina cribrosa depth (LCD) and minimal rim width (MRW) using optical coherence tomography (OCT). In this study, we induced OHT by repeated intracameral microbead injections in 16 cynomolgus primates (10 unilateral; 6 bilateral) and assessed the structural changes of the ONH longitudinally to observe early changes. Elevated intraocular pressure (IOP) in OHT eyes was maintained for 7 months and serial OCT measurements were performed during this period. The mean IOP was significantly elevated in OHT eyes when compared to baseline and compared to the control eyes. Thinner MRW and deeper LCD values from baseline were observed in OHT eyes with the greatest changes seen between month 1 and month 2 of OHT. Both the mean and maximum IOP values were significant predictors of MRW and LCD changes, although the maximum IOP was a slightly better predictor. We believe that this model could be useful to study IOP-induced early ONH structural damage which is important for understanding glaucoma pathogenesis.

Altered Energy Metabolism During Early Optic Nerve Crush Injury: Implications of Warburg-Like Aerobic Glycolysis in Facilitating Retinal Ganglion Cell Survival

Neurons, especially axons, are metabolically demanding and energetically vulnerable during injury. However, the exact energy budget alterations that occur early after axon injury and the effects of these changes on neuronal survival remain unknown. Using a classic mouse model of optic nerve-crush injury, we found that traumatized optic nerves and retinas harbor the potential to mobilize two primary energetic machineries, glycolysis and oxidative phosphorylation, to satisfy the robustly increased adenosine triphosphate (ATP) demand. Further exploration of metabolic activation showed that mitochondrial oxidative phosphorylation was amplified over other pathways, which may lead to decreased retinal ganglion cell (RGC) survival despite its supplement to ATP production. Gene set enrichment analysis of a microarray (GSE32309) identified significant activation of oxidative phosphorylation in injured retinas from wild-type mice compared to those from mice with deletion of phosphatase and tensin homolog (PTEN), while PTEN-/- mice had more robust RGC survival. Therefore, we speculated that the oxidation-favoring metabolic pattern after optic nerve-crush injury could be adverse for RGC survival. After redirecting metabolic flux toward glycolysis (magnifying the Warburg effect) using the drug meclizine, we successfully increased RGC survival. Thus, we provide novel insights into a potential bioenergetics-based strategy for neuroprotection.

Correlation Between Optic Disc Peripapillary Capillary Network and Papilledema Grading in Patients With Idiopathic Intracranial Hypertension: A Study of Optical Coherence Tomography Angiography.

The continued increase in idiopathic intracranial hypertension (IIH) prevalence has many implications for societal health care. Its potential vision-threatening consequences make ophthalmologists key players in its diagnosis and management. Newer technology such as optical coherence tomography angiography (OCT-A) enables evaluation of the branching complexity of the peripapillary capillary plexus, a region where accurate imaging via fluorescein angiography was previously limited. A cross-sectional, observational study of 23 (46 eyes) consecutive patients with IIH. Peripapillary total vasculature was recorded using commercial OCT-A en face vessel density mapping. In addition, OCT-A blood flow slab was compared with papilledema grading. OCT-A images were analyzed using a customized image analysis protocol using ImageJ software (v1.51w) and Photoshop software (Adobe Systems, CA). SPSS software version 25 was used for statistical analysis (SPSS Inc, IBM, Chicago, IL). Skeletonized vessel density peripapillary capillary plexus was significantly associated with Frisen papilledema grades, OCT retinal nerve fiber layer (RNFL), and macular ganglion cell layer (GCL) thickness with a P < 0.001, P = 0.022, and P = 0.006, respectively. Every point increase in grade was correlated with a decrease of 9.1 pixels/mm2 in vessel density (R = 0.512, β = -0.115 ± 0.029; P < 0.001). Increased papilledema was correlated with an increased retinal blood flow percentage (R = 0.300, β = 2.114 ± 1.013; P < 0.05) and decreased choroidal blood flow (CBF) percentage (R = 0.300, β = 2.114 ± 1.013; P < 0.05). Every point increase in grade was correlated with a decrease in CBF by 47.4%, as calculated using a linear best-fit line inclusive for all of the data points. OCT-A allows for effective visualization and quantification of the peripapillary retinal vasculature. Our results demonstrate a correlation between skeletonized peripapillary density and papilledema grading, OCT RNFL thickness, and GCL thickness. In addition, we show a significant negative correlation between CBF and papilledema grading. These changes provide key findings regarding the pathophysiology of optic neuropathy in papilledema and highlight the potential of OCT-A as a diagnostic tool for papilledema and a clinical marker for detecting early optic nerve damage.

Intracranial hypertension, nephritis and serositis complicating unexpected pregnancy in a lupus patient

Background: Systemic lupus erythematosus (SLE) flare in pregnancy is challenging. The simultaneous occurrence of intracranial hypertension (IH) is unusual and raises the possibility of being a presentation of neuropsychiatric SLE. Aim of the work: We here discuss the presentation and management of a complicated case of lupus associated with IH, lupus nephritis (LN) and serositis in unplanned pregnancy. Case presentation: A 26-year old SLE patient presented 10 weeks pregnant with marked pleural effusion, hypertension, grade V papilledema, impaired renal functions, proteinuria (2.2 g/24 h), high anti-double stranded deoxyribonucleic acid and consumed complement (C3). Renal biopsy revealed LN (class IV and V). MRI brain demonstrated features consistent with IH and perimetry revealed early optic nerve affection. Ultrasound-guided pleurocentesis and relieving lumbar puncture were performed. Pulse methylprednisolone and cyclosporine A (CsA) (2 mg/kg/day) were provided. Acetazolamide was added to manage the IH and meticulous control of BP was achieved. In 2 weeks renal functions, proteinuria and papilloedema improved. The patient gave birth via vaginal delivery at full term to a normal healthy boy. On follow-up after delivery, proteinuria ranged from 100 to 300 mg/24 h with normal renal functions and fundus. The patient was maintained on CsA, hydroxychloroquine and tapering doses of steroids with no relapses. Conclusion: Pregnant SLE cases presenting with IH, LN and serositis are challenging concerning the management of SLE activity. IH may be a sign of lupus activity and its resolution coincides with the control of the flare. Lower dose CsA is potentially effective in treating LN in pregnancy with no reported side effects.

Поражения органа зрения при сифилисе

The paper is focused on diagnosing eye structure lesions in patients with syphilis. Two clinical cases are described: a case of syphilitic uveitis in early syphilis and optic nerve atrophy in late syphilis. The main purpose is to increase the awareness of ophthalmologists about specific eye diseases in syphilis and the need for early detection of such pathologies. For citation: Bokhonovich D.V., Loseva O.K., Ryabtseva A.A., Kovrizhkina A.A., Yudakova V.M., Chernysheva N.V. Eye lesions in syphilis. Russian ophthalmological journal. 2018; 11 (2): 46-53. doi: 10.21516/2072-0076-2018-11-2-46-53 (In Russian).