Pineal germinomas can be very complex in terms of presentation, diagnosis, and management. This review attempts to simplify this complexity in an organized manner, addressing the anatomic relationships that provide the b...Pineal germinomas can be very complex in terms of presentation, diagnosis, and management. This review attempts to simplify this complexity in an organized manner, addressing the anatomic relationships that provide the basis for the uniqueness of pineal germinoma. Ocular findings and signs and symptoms of elevated intracranial pressure are the keys to suspecting the diagnosis and obtaining the necessary imaging and cerebrospinal fluid studies. Other symptoms can suggest spread beyond the pineal region. Surgery may only be needed to obtain tissue for a definitive diagnosis, as germinoma is highly responsive to chemotherapy and focused radiation therapy. Hydrocephalus, usually related to tumor obstruction of the cerebral aqueduct, may also need to be addressed. Outcome for pineal germinoma is usually excellent, but relapse can occur and may require additional intervention. These issues are detailed in this review.
Glaucoma is one of the main causes of irreversible blindness in the world. The most common form, primary open-angle glaucoma, is an optic neuropathy that is characterized by a progressive loss of retinal ganglion cells a...Glaucoma is one of the main causes of irreversible blindness in the world. The most common form, primary open-angle glaucoma, is an optic neuropathy that is characterized by a progressive loss of retinal ganglion cells and their axons, leading to structural changes in the optic nerve head and associated visual field defects. Elevated intraocular pressure remains the most important modifiable risk factor for primary open-angle glaucoma. However, a significant proportion of patients develop glaucomatous damage in the absence of increased intraocular pressure, a condition known as normal-tension glaucoma (NTG). The pathophysiology underlying NTG remains unclear. Several studies have revealed that vascular and cerebrospinal fluid (CSF) factors may play significant roles in the development of NTG. Vascular failure caused by functional or structural abnormalities, and compartmentation of the optic nerve subarachnoid space with disturbed CSF dynamics have been shown to be associated with NTG. In the present article, based on the concept of the glymphatic system and observations in patients with NTG, we hypothesize that failure of fluid transport via the glymphatic pathway in the optic nerve may be involved in the pathogenesis of some if not many cases of NTG. According to this hypothesis, vascular and CSF factors may share reduced glymphatic transport and perivascular waste clearance in the optic nerve as a final common pathway leading to the development of NTG. In addition, we speculate that some cases of NTG may reflect glymphatic dysfunction in natural brain aging and central nervous system diseases, such as Alzheimer's disease. Clearly, further studies are needed to gain additional insight into the relative contribution of these factors and conditions to reduced glymphatic transport in the optic nerve.
Barrett-Young A, Abraham WC, Cheung CY
… +13 more, Gale J, Hogan S, Ireland D, Keenan R, Knodt AR, Melzer TR, Moffitt TE, Ramrakha S, Tham YC, Wilson GA, Wong TY, Hariri AR, Poulton R
PURPOSE: The retina has potential as a biomarker of brain health and Alzheimer's disease (AD) because it is the only part of the central nervous system which can be easily imaged and has advantages over brain imaging tec...PURPOSE: The retina has potential as a biomarker of brain health and Alzheimer's disease (AD) because it is the only part of the central nervous system which can be easily imaged and has advantages over brain imaging technologies. Few studies have compared retinal and brain measurements in a middle-aged sample. The objective of our study was to investigate whether retinal neuronal measurements were associated with structural brain measurements in a middle-aged population-based cohort. PARTICIPANTS AND METHODS: Participants were members of the Dunedin Multidisciplinary Health and Development Study (n=1037; a longitudinal cohort followed from birth and at ages 3, 5, 7, 9, 11, 13, 15, 18, 21, 26, 32, 38, and most recently at age 45, when 94% of the living Study members participated). Retinal nerve fibre layer (RNFL) and ganglion cell-inner plexiform layer (GC-IPL) thickness were measured by optical coherence tomography (OCT). Brain age gap estimate (brainAGE), cortical surface area, cortical thickness, subcortical grey matter volumes, white matter hyperintensities, were measured by magnetic resonance imaging (MRI). RESULTS: Participants with both MRI and OCT data were included in the analysis (RNFL n=828, female n=413 [49.9%], male n=415 [50.1%]; GC-IPL n=825, female n=413 [50.1%], male n=412 [49.9%]). Thinner retinal neuronal layers were associated with older brain age, smaller cortical surface area, thinner average cortex, smaller subcortical grey matter volumes, and increased volume of white matter hyperintensities. CONCLUSION: These findings provide evidence that the retinal neuronal layers reflect differences in midlife structural brain integrity consistent with increased risk for later AD, supporting the proposition that the retina may be an early biomarker of brain health.
PURPOSE: To identify ophthalmic findings in Alzheimer's type dementia (ATD) compared to normal subjects. PATIENTS AND METHODS: This comparative descriptive study included participants from the institution's cognitive fit...PURPOSE: To identify ophthalmic findings in Alzheimer's type dementia (ATD) compared to normal subjects. PATIENTS AND METHODS: This comparative descriptive study included participants from the institution's cognitive fitness center. Complete ophthalmic examinations were performed. Optical coherence tomography (OCT) and OCT angiography (OCTA) were used to analyze retinal thickness and vascular density. The Ocular Surface Disease Index (OSDI) score and tear breakup time (TBUT) were used to assess dry eye. The blink rate was counted by a well-trained observer. Cognitive function was evaluated using the Thai Mental State Examination (TMSE) score. Correlation analysis was performed to compare OCT, OCTA parameters, and TMSE. RESULTS: We included 24 ATD patients and 39 normal participants as a control group by age and sex-matched. The prevalence of dry eye using the Asia Dry Eye Society criteria was 15% and 13% in normal and ATD patients, respectively. The differences in OSDI scores, TBUT, and blink rate between the two groups were not statistically significant. The parafoveal and perifoveal macular thickness of the ATD group were significantly lower than that of the control group (p<0.01). All parameters of the vessel density of the ATD group were significantly lower than in the control group, including the whole macular vessel density (p<0.01), optic disc vessel density at the nerve head level (p<0.01), and optic disc vessel density at the radial peripapillary capillary level (p<0.05). After age adjustment, there were no statistically significant differences in all the OCT and OCTA parameters. There was a positive correlation between retinal thickness and vessel density in the macular and optic disc region and TMSE scores. CONCLUSION: Perifoveal and parafoveal retinal thickness might be more sensitive than peripapillary RNFL thickness to detect neurodegenerative changes in patients with ATD. Macular thickness and vessel density reduction were also positively correlated with cognitive decline.
Ocular myasthenia gravis (OMG) is a neuromuscular disease characterized by autoantibody production against post-synaptic proteins in the neuromuscular junction. The pathophysiological auto-immune mechanisms of myasthenia...Ocular myasthenia gravis (OMG) is a neuromuscular disease characterized by autoantibody production against post-synaptic proteins in the neuromuscular junction. The pathophysiological auto-immune mechanisms of myasthenia are diverse, and this is governed primarily by the type of autoantibody production. The diagnosis of OMG relies mainly on clinical assessment, the use of serological antibody assays for acetylcholine receptors (AchR), muscle-specific tyrosine kinase (MusK), and low-density lipoprotein 4 (LPR4). Other autoantibodies against post-synaptic proteins, such as cortactin and agrin, have been detected; however, their diagnostic value and pathogenic effect are not yet clearly defined. Clinical tests such as the ice test and electrophysiologic tests, particularly single-fiber electromyography, have a valuable role in diagnosis. The treatment of OMG is primarily through cholinesterase inhibitors (pyridostigmine), and steroids are frequently required in cases of ophthalmoplegia. Other immunosuppressive therapies include antimetabolites (azathioprine, mycophenolate mofetil, methotrexate) and biological agents such as B-cell depleting agents (Rituximab) and complement inhibitors (eculizumab). Evidence is scarce on the effect of immunosuppressive therapy on altering the natural course of OMG. Clinicians must be vigilant of a myasthenic syndrome in patients using immune-check inhibitors. Reliable and consistent biomarkers are required to assess disease severity and response to therapy to optimize the management of OMG. The purpose of this review is to summarize the current trends and the latest developments in diagnosing and treating OMG.
INTRODUCTION: Previous works on experience-dependent brain plasticity have been limited to the cortical structures, overlooking subcortical visual structures such as the lateral geniculate nucleus (LGN). Animal studies h...INTRODUCTION: Previous works on experience-dependent brain plasticity have been limited to the cortical structures, overlooking subcortical visual structures such as the lateral geniculate nucleus (LGN). Animal studies have shown substantial experience dependent plasticity and using fMRI, human studies have demonstrated similar properties in patients with cataract surgery. However, in neither animal nor human studies LGN has not been directly assessed, mainly due to its small size, tissue heterogeneity, low contrast/noise ratio, and low spatial resolution. METHODS: Utilizing a new algorithm that markedly improves the LGN visibility, LGN was evaluated in a group of low vision patients before and after retinal intervention to reinstate vision and normal sighted matched controls. RESULTS: Between and within groups comparisons showed that patients had significantly smaller left (p< 0.0001) and right (p < 0.00002) LGN volumes at baseline as compared to the one-year follow-up volumes. The same baseline and one year comparison in controls was not significant. Significant positive correlations were observed between the incremental volume increase after gene therapy of the left LGN and the incremental increase in the right (r = 0.71, p < 0.02) and left (r = 0.72, p = 0.018) visual fields. Incremental volume increase of the right LGN also showed a similar positive slope but did not reach significance. DISCUSSION: These results show that despite significantly less volume at baseline, retinal gene therapy promotes robust expansion and increase in LGN volume. Reinstating vision may have facilitated the establishment of new connections between the retina and the LGN and/or unmasking of the dormant connections. The exact trajectory of the structural changes taking place in LGN is unclear but our data shows that even after years of low vision, the LGN in RPE65 patients has the potential for plasticity and expansion to a nearly normal volume one year after gene therapy administration.
PURPOSE: Prenatal growth restriction is associated with impaired neurodevelopment in childhood. This study investigated the effects of being born small for gestational age (SGA) on peripapillary retinal nerve fiber layer...PURPOSE: Prenatal growth restriction is associated with impaired neurodevelopment in childhood. This study investigated the effects of being born small for gestational age (SGA) on peripapillary retinal nerve fiber layer (pRNFL) thickness in adults born at term. METHODS: A retrospective cohort study was conducted with a prospective ophthalmologic examination of participants born at full-term (gestational age ≥37 weeks) between 1969 and 2002. All participants were examined with spectral-domain optical coherence tomography and grouped according to their birth weight in correlation to gestational age as former moderate (birth weight (BW) percentile 3rd to <10th) and severe SGA (<3rd percentile), normal (10th-90th percentile, AGA), and moderately (>90th to 97th percentile) and severely (>97th percentile) large for gestational age (LGA) adults (18 to 52 years). RESULTS: Overall, 547 eyes of 285 individuals (age 29.9±9.4 years, 151 females) born at term were included. Multivariable regression analyses revealed a strong association between a lower global pRNFL thickness in the severe SGA (B=-8.99 [95%-CI: -12.68; -5.30] µm; p<0.001) and in the moderate SGA groups (B=-6.40 [95%-CI: -10.29; -2.50] µm; p=0.001) compared to the reference AGA group. CONCLUSION: Our results indicate that restricted fetal growth affects neurologic tissue development of the optic nerve head, particularly in individuals born severely SGA at term. This indicates that fetal growth restriction may exert disturbances in the development of neurologic tissue, which persists in adulthood.
BACKGROUND: Ultrasonographic measurement of optic nerve sheath diameter is a simple, non-invasive, and reliable method of detecting elevated intracranial pressure (ICP) in critical patients. Optic nerve sheath communicat...BACKGROUND: Ultrasonographic measurement of optic nerve sheath diameter is a simple, non-invasive, and reliable method of detecting elevated intracranial pressure (ICP) in critical patients. Optic nerve sheath communicates with the dura mater covering the brain and contains cerebrospinal fluid, allowing pressure transmission from the cranium. Therefore, changes in cerebrospinal fluid (CSF) pressure have been shown to produce changes in ONSD. OBJECTIVE: This study aimed to assess the accuracy of optic nerve sheath diameter (ONSD) in diagnosis and follow-up patients with disturbed conscious levels compared with CT brain and fundus examination. PATIENTS AND METHODS: One hundred forty-one participants were included in the study, classified into 76 cases admitted with disturbed conscious levels due to elevated ICP and 65 controls. All patients were subjected to CT brain and optic nerve US and fundus examination at the time of admission and follow-up after 48 h after proper management. RESULTS: The current study showed that ONSD is significant in predicting elevated ICP at the cut-off point of average ONSD of 5.19 mm with 97% sensitivity and 98% specificity, and the area under the curve (AUC) was 0.996. The present study revealed a significant inverse correlation between ONSD and GCS in patients with increased ICP. CONCLUSION: Ultrasonic measurement of ONSD is a promising technique in diagnosing and following patients with disturbed conscious levels.
Glaucoma is a common condition that relies on careful clinical assessment to diagnose and determine disease progression. There is growing evidence that glaucoma is associated not only with loss of retinal ganglion cells...Glaucoma is a common condition that relies on careful clinical assessment to diagnose and determine disease progression. There is growing evidence that glaucoma is associated not only with loss of retinal ganglion cells but also with degeneration of cortical and subcortical brain structures associated with vision and eye movements. The effect of glaucoma pathophysiology on eye movements is not well understood. In this review, we examine the evidence surrounding altered eye movements in glaucoma patients compared to healthy controls, with a focus on quantitative eye tracking studies measuring saccades, fixation, and optokinetic nystagmus in a range of visual tasks. The evidence suggests that glaucoma patients have alterations in several eye movement domains. Patients exhibit longer saccade latencies, which worsen with increasing glaucoma severity. Other saccadic abnormalities include lower saccade amplitude and velocity, and difficulty inhibiting reflexive saccades. Fixation is pathologically altered in glaucoma with reduced stability. Optokinetic nystagmus measures have also been shown to be abnormal. Complex visual tasks (eg reading, driving, and navigating obstacles), integrate these eye movements and result in behavioral adaptations. The review concludes with a summary of the evidence and recommendations for future research in this emerging field.
PURPOSE: Visual impairment is a major cause of disability and impairment of cognitive function in older people. Brain structural changes associated with visual function impairment are not well understood. The objective o...PURPOSE: Visual impairment is a major cause of disability and impairment of cognitive function in older people. Brain structural changes associated with visual function impairment are not well understood. The objective of this study was to assess the association between visual function and cortical thickness in older adults. METHODS: Participants were selected from the French population-based ESPRIT cohort of 2259 community-dwelling adults ≥65 years old enrolled between 1999 and 2001. We considered visual function and brain MRI images at the 12-year follow-up in participants who were right-handed and free of dementia and/or stroke, randomly selected from the whole cohort. High-resolution structural T1-weighted brain scans acquired with a 3-Tesla scanner. Regional reconstruction and segmentation involved using the FreeSurfer image-analysis suite. RESULTS: A total of 215 participants were included (mean [SD] age 81.8 [3.7] years; 53.0% women): 30 (14.0%) had central vision loss and 185 (86.0%) normal central vision. Vision loss was associated with thinner cortical thickness in the right insula (within the lateral sulcus of the brain) as compared with the control group (mean thickness 2.38 [0.04] vs 2.50 [0.03] mm, 4.8% thinning, p= 0.04) after adjustment for age, sex, lifetime depression and cardiovascular disease. CONCLUSION: The present study describes a significant thinning of the right insular cortex in older adults with vision loss. The insula subserves a wide variety of functions in humans ranging from sensory and affective processing to high-level cognitive processing. Reduced insula thickness associated with vision loss may increase cognitive burden in the ageing brain.
INTRODUCTION: Diabetic patients routinely have high levels of high mobility group box 1 (HMGB1) protein in their plasma, vitreous and ocular membranes, which is strongly correlated with subclinical chronic inflammation i...INTRODUCTION: Diabetic patients routinely have high levels of high mobility group box 1 (HMGB1) protein in their plasma, vitreous and ocular membranes, which is strongly correlated with subclinical chronic inflammation in the eye. Our previous work has suggested that high HMGB1 in diabetes plays a role in retinal inflammation and angiogenesis, but its role in the optic nerve damage is unclear. Therefore, our goal is to examine the role of HMGB1 in optic nerve damage in diabetes. METHODS: Gene expression of HMGB1 was quantified in the optic nerve from streptozotocin-induced diabetic mice by qRT-PCR, and their protein expressions by Western blot analysis and immunofluorescence staining. Using immunohistochemical technique, expression of reactive astrogliosis (indicator of neuroinflammation) and nerve demyelination/damage were determined by quantifying glial fibrillary acid protein (GFAP) and myelin basic protein (MBP), respectively. The role of HMGB1 in the optic nerve damage and alteration visual pathways was confirmed in mice receiving glycyrrhizin, a HMGB1 inhibitor. Similar parameters were measured in the optic nerve from human donors with diabetes. RESULTS: Compared to normal mice, diabetic mice exhibited increased levels of HMGB1, higher GFAP expression, and decreased MBP in the optic nerve. Double immunofluorescence microscopy revealed that diabetes induced increased HMGB1 immunoreactivities were significantly colocalized with GFAP in the optic nerve. Glycyrrhizin supplementation effectively reduced HMGB1 and maintained normal axonal myelination and visual conduction. Results from mice optic nerve confirmed the results obtained from human donors with diabetes. DISCUSSIONS: Thus, diabetes-induced HMGB1 upregulation promotes optic nerve demyelination and inflammation. The regulation of HMGB1 activation has potential to protect optic nerve damage and the abnormalities of visual pathways in diabetic patients.
Spaceflight-associated neuro-ocular syndrome (SANS) has been well documented in astronauts both during and after long-duration spaceflight and is characterized by the development of optic disc edema, globe flattening, ch...Spaceflight-associated neuro-ocular syndrome (SANS) has been well documented in astronauts both during and after long-duration spaceflight and is characterized by the development of optic disc edema, globe flattening, choroidal folds, and hyperopic refractive error shifts. The exact mechanisms underlying these ophthalmic abnormalities remain unclear. New findings regarding spaceflight-associated alterations in cerebrospinal fluid spaces, specifically perivascular spaces, may shed more light on the pathophysiology of SANS. The preliminary results of a recent brain magnetic resonance imaging study show that perivascular spaces enlarge under prolonged microgravity conditions, and that the amount of fluid in perivascular spaces is linked to SANS. The exact pathophysiological mechanisms underlying enlargement of perivascular spaces in space crews are currently unclear. Here, we speculate that the dilation of perivascular spaces observed in long-duration space travelers may result from impaired cerebral venous outflow and compromised cerebrospinal fluid resorption, leading to obstruction of glymphatic perivenous outflow and increased periarterial cerebrospinal fluid inflow, respectively. Further, we provide a possible explanation for how dilated perivascular spaces can be associated with SANS. Given that enlarged perivascular spaces in space crews may be a marker of altered venous hemodynamics and reduced cerebrospinal fluid outflow, at the level of the optic nerve and eye, these disturbances may contribute to SANS. If confirmed by further studies, brain glymphatic dysfunction in space crews could potentially be considered a risk factor for the development of neurodegenerative diseases, such as Alzheimer's disease. Furthermore, long-duration exposure to microgravity might contribute to SANS through dysregulation of the ocular glymphatic system. If prolonged spaceflight exposure causes disruption of the glymphatic systems, this might affect the ability to conduct future exploration missions, for example, to Mars. The considerations outlined in the present paper further stress the crucial need to develop effective long-term countermeasures to mitigate SANS-related physiologic changes during long-duration spaceflight.
PURPOSE: To evaluate the relationship between different macular thickness parameters analyzed by SD-OCT and the central visual field (VF) evaluated with automated kinetic perimetry in a cohort of patients with pituitary...PURPOSE: To evaluate the relationship between different macular thickness parameters analyzed by SD-OCT and the central visual field (VF) evaluated with automated kinetic perimetry in a cohort of patients with pituitary tumors. METHODS: Data from patients with pituitary adenoma treated at Reims University Hospital between October 1st, 2017, and May 31st, 2018 were collected. All patients underwent an automated kinetic perimetry and a SD-OCT to map the ganglion cell complex (GCC), the ganglion cell layer (GCL) thickness and the retinal nerve fiber layer (RNFL) using devices from two different manufacturers. Univariate and multivariate analysis were used to evaluate the correlation between the area of central VF in square degrees (deg2) and the SD-OCT parameters (μm). RESULTS: Eighty-eight eyes were included in the analysis. All the thickness parameters measured in SD-OCT decreased with the visual field alteration. The best correlation was observed between superior thickness parameters (GCC, GCL) and the inferior central visual field. The most pertinent predictive factors for visual field loss were the inferior central GCL and the nasal RNFL (both AUC=0.775) with a sensitivity respectively of 86% and 70%. CONCLUSION: This study suggests that both GCC, GCL thickness parameters could be reliable predictors of central visual field impairment in patients with pituitary tumors. There was no significative difference between both devices.
PURPOSE: Transcranial alternating current stimulation (tACS) is used as a brain stimulation mechanism to enhance learning, ameliorate some psychiatric disorders, and modify behavior. This study assessed the effects of ne...PURPOSE: Transcranial alternating current stimulation (tACS) is used as a brain stimulation mechanism to enhance learning, ameliorate some psychiatric disorders, and modify behavior. This study assessed the effects of near threshold tACS-like currents on Off-center and On-Off retinal ganglion cell responsiveness in the rabbit retina eyecup preparation as a model for central nervous system effects. MATERIALS AND METHODS: We made extracellular recordings in the isolated rabbit eyecup preparation using single electrodes and microelectrode arrays to measure light-evoked spike responses in different classes of Off-center and On-Off retinal ganglion cells before, during, and after brief applications of alternating currents of 1-2 microamperes, at frequencies of 10, 20, 30, and 40 Hz. RESULTS: tACS application sculpted the light-evoked response profiles without directly driving spiking activity of the 20 Off-center and On-Off ganglion cells we recorded from. During tACS application, Off responses were significantly enhanced for 6 cells and significantly suppressed for 14 cells, but after tACS application, Off responses were significantly enhanced for 7 cells and suppressed for 12 cells. The Off responses of the remaining two cells returned to baseline. On responses were less affected during and after tACS. CONCLUSION: tACS sculpts Off-center and On-Off retinal ganglion cell responsiveness. The dissimilarity of effects in different cells within the same class and the differential effects on the On and Off components of the light response within the same cell are consistent with the hypothesis that tACS acts at threshold on amacrine cells in the inner plexiform layer.
PURPOSE: Transcranial alternating current stimulation (tACS) is a stimulation protocol used for learning enhancement and mitigation of cognitive dysfunction. Correlated firing has been postulated to be a meta-code that l...PURPOSE: Transcranial alternating current stimulation (tACS) is a stimulation protocol used for learning enhancement and mitigation of cognitive dysfunction. Correlated firing has been postulated to be a meta-code that links neuronal spike responses associated with a single entity and may be an important component of high-level cognitive functions. Thus, changes in the covariance firing structure of CNS neurons such as retinal ganglion cells are one potential mechanism by which tACS can exert its effects. MATERIALS AND METHODS: We used microelectrode arrays to record light-evoked spike responses of 24 retinal ganglion cells in 7 rabbit eyecup preparations and analyzed the covariance between 30 pairs of neighboring retinal ganglion cells before, during, and after 10-minute application of alternating currents of 1 microampere at 10 or 20 Hz. RESULTS: tACS stimulation significantly changed the covariance structure of correlated firing in 60% of simultaneously recorded retinal ganglion cells. Application of tACS in the retinal preparation increased cross-covariance in 26% of cell pairs, an effect usually associated with increased light-evoked ganglion cell firing. tACS associated decreases in cross-covariance occurred in 37% of cell pairs. Increased covariance was more common in response to the first, 10-minute application of tACS in isolated retina preparation. Changes in covariance were rare after repeated stimulation, and more likely to result in decreased covariance. CONCLUSION: Retinal ganglion cell correlated firing is modulated by 1 microampere tACS currents showing that electrical stimulation can significantly and persistently change the structure of the correlated firing of simultaneously recorded rabbit retinal ganglion cells.
Optic nerve cupping or enlargement of the cup-to-disc ratio is widely recognized as a feature of glaucoma, however it may also occur in non-glaucomatous optic neuropathies. The most well-recognized non-glaucomatous optic...Optic nerve cupping or enlargement of the cup-to-disc ratio is widely recognized as a feature of glaucoma, however it may also occur in non-glaucomatous optic neuropathies. The most well-recognized non-glaucomatous optic neuropathies that cause cupping include compressive optic neuropathies, arteritic anterior ischemic optic neuropathies, hereditary optic neuropathies, and optic neuritis. Cupping is thought to consist of two main components: prelaminar and laminar thinning. The former is a shallow form of cupping and related to loss of retinal ganglion cells, whereas the latter involves damage to the lamina cribrosa and peripapillary scleral connective tissue. Differentiating glaucomatous and non-glaucomatous optic nerve cupping remains challenging even for experienced observers. Classically, the optic nerve in non-glaucomatous causes has pallor of the neuroretinal rim, but the optic nerve should not be examined in isolation. The patient's medical history, history of presenting illness, visual function (visual acuity, color vision and visual field testing) and ocular examination also need to be considered. Ancillary testing such as optical coherence tomography of the retinal nerve fiber layer and ganglion cell layer-inner plexiform layer may also be helpful in localizing the disease. In this review, we review the non-glaucomatous causes of cupping and provide an approach to evaluating a patient that presents with an enlarged cup-to-disc ratio.
Vestibular schwannomas (VSs), also called acoustic neuromas, are benign intracranial neoplasms of the vestibulocochlear (VIII) cranial nerve. Management options include "wait-and-scan," stereotactic radiosurgery and surg...Vestibular schwannomas (VSs), also called acoustic neuromas, are benign intracranial neoplasms of the vestibulocochlear (VIII) cranial nerve. Management options include "wait-and-scan," stereotactic radiosurgery and surgical resection. Due to the proximity of the VIII nerve to the facial (VII) nerve in the cerebello-pontine angle, the VII nerve is particularly vulnerable to the effects of surgical resection. This can result in poor eye closure, lagophthalmos and resultant corneal exposure post VS resection. Additionally, compression from the tumor or resection can cause trigeminal (V) nerve damage and a desensate cornea. The combination of an exposed and desensate cornea puts the eye at risk of serious ocular complications including persistent epithelial defects, corneal ulceration, corneal vascularization, corneal melting and potential perforation. The abducens (VI) nerve can be affected by a large intracranial VS causing raised intracranial pressure (a false localizing sign) or as a result of damage to the VI nerve at the time of resection. Other types of neurogenic strabismus are rare and typically transient. Contralaterally beating nystagmus as a consequence of vestibular dysfunction is common post-operatively. This generally settles to pre-operative levels as central compensation occurs. Ipsilaterally beating nystagmus post-operatively should prompt investigation for post-operative cerebrovascular complications. Papilledema (and subsequent optic atrophy) can occur as a result of a large VS causing raised intracranial pressure. Where papilledema follows surgical resection of a VS, it can indicate that cerebral venous sinus thrombosis has occurred. Poor visual function following VS resection can result as a combination of all these potential complications and is more likely with larger tumors.
PURPOSE: To assess the diagnostic accuracy of visual field results generated by the newly developed software (CU-VF) and the standard automated perimetry (SAP) for detecting hemianopia. PATIENTS AND METHODS: Forty-three...PURPOSE: To assess the diagnostic accuracy of visual field results generated by the newly developed software (CU-VF) and the standard automated perimetry (SAP) for detecting hemianopia. PATIENTS AND METHODS: Forty-three subjects with hemianopia and 33 controls were tested with the CU-VF software on a personal computer and SAP. Hemianopia was defined as the presence of a hemianopic field respecting the vertical meridian on SAP with the corresponding neuroimaging pathology as evaluated by 2 neuro-ophthalmologists. Results of CU-VF were independently evaluated by 2 neuro-ophthalmologists, 1 general ophthalmologist, and 1 general practitioner in terms of the presence of hemianopia. Sensitivity, specificity, and kappa coefficient for inter-observer reliability were calculated. Satisfaction and ease of use were evaluated with a visual analog-scale questionnaire and analyzed using paired -test. RESULTS: The sensitivity (95% CI) and specificity (95% CI) of the CU-VF to detect hemianopia was 74.42% (58.53-85.96) and 93.94% (78.38-99.94). Kappa coefficient between neuro-ophthalmologists versus general ophthalmologist and general practitioner were 0.71 and 0.84, respectively. The mean (SD) test duration was 2.25 (0.002) minutes for the CU-VF and 5.38 (1.34) minutes for SAP (p < 0.001). Subjects reported significantly higher satisfaction and comfort using the CU-VF software compared to SAP. CONCLUSION: The CU-VF screening software showed good validity and reliability to detect hemianopia, with shorter test duration and higher subject satisfaction compared to SAP.
PURPOSE: Management of optic nerve sheath meningiomas (ONSM) remains challenging. Photon radiation therapy (PhRT) is the most common treatment for sight-threatening ONSM. Proton beam therapy (PBT) is less commonly used b...PURPOSE: Management of optic nerve sheath meningiomas (ONSM) remains challenging. Photon radiation therapy (PhRT) is the most common treatment for sight-threatening ONSM. Proton beam therapy (PBT) is less commonly used because it is more expensive and because there are questions about its efficacy specifically in relation to ONSM. PBT has the theoretical advantage of reducing radiation exposure to adjacent structures. We report the visual outcome of patients with primary ONSM managed at the Fondation Ophtalmologique Adolphe de Rothschild, Paris, France, and treated with PBT at the Centre de Protonthérapie, Institut Curie, Orsay, France. METHODS: We conducted a retrospective review of all patients with primary ONSM who received PBT (either by itself or following surgery) between January 2006 and January 2019. Neuro-ophthalmic examinations were performed at presentation and after radiotherapy, and, when applicable, after surgery. Meningiomas were measured at the time of diagnosis and at each follow-up MRI examination. RESULTS: Sixty patients (50 women, 10 men; mean age, 45.2±11.1y) were included, of whom 29 underwent surgery. At presentation, 52 (87%) of them had decreased vision (average visual acuity: 0.6 logMAR). Fundus examination showed optic disc swelling (n=27; 46.5%), optic disc pallor (n=22; 37.9%), optic disc cupping (n=2; 3.4%), opto-ciliary shunt (n=8; 13.8%), or choroidal folds (n=5; 8.6%). Otherwise, it was unremarkable (n=7; 12.1%). After treatment, visual function was stable overall. Fundus examination showed pallor (n=47; 83.9%), swelling (n=3; 5.4%), or cupping (n=2; 3.4%) of the optic disc, or was unremarkable (n=5; 8.9%). The visual field of 8 patients worsened, while 3 developed asymptomatic retinal hemorrhages. Tumor shrunk significantly in 8 patients at 1 year after PBT and remained stable in size in all others. Patients with opto-ciliary shunts had significantly worse visual outcome than other patients. Retinal abnormalities were observed in 11 patients during follow-up. CONCLUSION: PBT alone or in association with surgery appears to be a safe and efficient treatment for ONSM, reducing the tumor size and stabilizing visual function. The risk of developing radiation retinopathy seems to be higher when patients had upfront surgery.