The impact of eating disorders on idiopathic intracranial hypertension.

BACKGROUND: Idiopathic intracranial hypertension (IIH) occurs more frequently in obese females of childbearing age. A link between eating disorders and poor outcome has been suggested but remains unproven. METHODS: This prospective field study at two tertiary headache centers included patients with clinically suspected IIH after standardized diagnostic work-up. Eating disorders were evaluated using validated questionnaires (EDQs). Primary outcome was the impact of eating disorders on IIH severity and outcome, secondary outcome was the prevalence and type of eating disorders in IIH compared to controls. RESULTS: We screened 326 patients; 143 patients replied to the EDQs and were classified as 'IIH' or 'non-IIH' patients. The demographic profile of EDQ-respondents and non-respondents was similar. Presence of an eating disorder did not impact IIH severity (lumbar puncture opening pressure (p = 0.63), perimetric mean deviation (p = 0.18), papilledema (Frisén grad 1-3; p = 0.53)) nor IIH outcome (optic nerve atrophy (p = 0.6), impaired visual fields (p = 0.18)). Moreover, we found no differences in the prevalence and type of eating disorders when comparing IIH with non-IIH patients (p = 0.09). CONCLUSION: Eating disorders did not affect IIH severity or outcome. We found the same prevalence and distribution pattern of eating disorders in IIH and non-IIH patients advocating against a direct link between IIH and eating disorders.

Systemic lupus erythematosus with chronic persistent intracranial hypertension: A case report.

OBJECTIVE: The aim is to investigate the clinical characteristics of systemic lupus erythematosus with intracranial hypertension. METHODS: The clinical characteristics of one case of systemic lupus erythematosus with chronic persistent intracranial hypertension were analyzed, and related literature was reviewed by searching Medline and Wanfang databases. RESULTS: Intracranial hypertension in SLE patients may occur at the onset or during the course of the disease. Our patient was diagnosed with IH 3 years after the onset of SLE. Headache and papilledema were the most common symptoms of intracranial hypertension, followed by nausea or vomiting, vision changes, and cerebral palsy. Our patient had a headache and cranial hypertension that lasted for years, but no papilledema was found. Corticosteroid is currently the mainstay of the treatment of IIH in patients with SLE, and immunosuppressive agents, acetazolamide, intravenous mannitol and furosemide are also used. However, our patient did not respond to these treatments and presents the characteristics of chronic persistent intracranial hypertension. CONCLUSION: Systemic lupus erythematosus with intracranial hypertension is a rare manifestation of SLE, which is not completely parallel to SLE activity. Headache and papilledema were the most common presenting symptoms. Different from previous reported cases, our patient had poor response to treatments, showing chronic and persistent characteristics.

Advantages and Pitfalls of the Use of Optical Coherence Tomography for Papilledema.

PURPOSE OF REVIEW: Papilledema refers to optic disc swelling caused by raised intracranial pressure. This syndrome arises from numerous potential causes, which may pose varying degrees of threat to patients. Manifestations of papilledema range from mild to severe, and early diagnosis is important to prevent vision loss and other deleterious outcomes. The purpose of this review is to highlight the role of optical coherence tomography (OCT) in the diagnosis and management of syndromes of raised intracranial pressure associated with papilledema. RECENT FINDINGS: Ophthalmoscopy is an unreliable skill for many clinicians. Optical coherence tomography is a non-invasive ocular imaging technique which may fill a current care gap, by facilitating detection of papilledema for those who cannot perform a detailed fundus examination. Optical coherence tomography may help confirm the presence of papilledema, by detecting subclinical peripapillary retinal nerve fiber layer (pRNFL) thickening that might otherwise be missed with ophthalmoscopy. Enhanced depth imaging (EDI) and swept source OCT techniques may identify optic disc drusen as cause of pseudo-papilledema. Macular ganglion cell inner plexiform layer (mGCIPL) values may provide early signs of neuroaxonal injury in patients with papilledema and inform management for patients with syndromes of raised intracranial pressure. There are well-established advantages and disadvantages of OCT that need to be fully understood to best utilize this method for the detection of papilledema. Overall, OCT may complement other existing tools by facilitating detection of papilledema and tracking response to therapies.  Moving forward, OCT findings may be included in deep learning models to diagnose papilledema.

A case report: co-occurrence of probable Vogt-Koyanagi-Harada disease and diabetic retinopathy.

BACKGROUND: Bilateral retinal detachment and choroidal detachment in a patient are rare occurrences. The presence of bilateral diabetic retinopathy (DR) in such a case is even rarer and complicates the condition. CASE PRESENTATION: In this study, we document a case of unconventional VKH. Manifestations in this patient included intense peripheral retinal detachment and choroidal detachment, along with vitreous opacities akin to cotton wool spots, concurrent with DR. The diagnosis was considered as probable VKH with DR. Treatment according to VKH protocols, including high-dose corticosteroids, yielded positive results. CONCLUSIONS: VKH can co-occurrence with DR. VKH manifestations vary, and early, aggressive, and long-term treatment is essential. The complexity of treatment increases with concurrent DR, necessitating the use of immunosuppressants.

Papilloedema: a highly specific predictor of raised intracranial pressure in a complex neurosurgical paediatric cohort.

PURPOSE: Papilloedema is recognised as an indicator of raised intracranial pressure, although there is a paucity of literature describing the utility of fundoscopy in screening for raised ICP in children with craniofacial synostosis, particularly young children. We sought to investigate the association of optic disc morphology with ICP in children, and to define the sensitivity and specificity of papilloedema as a clinical indicator of raised ICP and determine if age, or underlying conditions impact the findings. METHOD: Retrospective analysis of all patients undergoing ICP monitoring at a designated paediatric neurosurgical and craniofacial unit in the United Kingdom between October 2009 and October 2018. The fundoscopy findings and ICP monitoring data were analysed for 31 children with craniosynostosis and 29 children without craniosynostosis. RESULTS: All children who had papilloedema had raised ICP confirmed with monitoring. Across the 60-patient cohort, confirmed papilloedema on fundoscopy had Positive Predictive Value (PPV) of 1.00, Negative Predictive Value (NPV) of 0.64 with sensitivity 48% and specificity 100% for the presence of raised ICP (p = < 0.0001). In the craniosynostosis group, PPV was 1.00, NPV was 0.39, sensitivity 48% and specificity 100% (p = < 0.03). There is no correlation between severity of optic disc swelling using Frisen grading and elevation of ICP. Age did not affect the presence of papilloedema in those with raised ICP. CONCLUSION: The presence of papilloedema is a strong indicator of raised ICP in a child, regardless of underlying aetiology. Detailed fundoscopy can prevent the need for further investigations including imaging-related radiation and invasive CSF pressure monitoring.

Transorbital point-of-care ultrasound versus fundoscopic papilledema to support treatment indication for potentially elevated intracranial pressure in children.

PURPOSE: To compare transorbital point-of-care ultrasound techniques -optic nerve sheath diameter (US-ONSD) and optic disc elevation (US-ODE)- with fundoscopic papilledema to detect potentially raised intracranial pressure (ICP) with treatment indication in children. METHODS: In a prospective study, 72 symptomatic children were included, 50 with later proven disease associated with raised ICP (e.g. pseudotumour cerebri, brain tumour, hydrocephalus) and 22 with pathology excluded. Bilateral US-ONSD and US-ODE were quantified by US using a 12-MHz-linear-array transducer. This was compared to fundoscopic optic disc findings (existence of papilledema) and, in 28 cases, invasively measured ICP values. RESULTS: The sensitivity and specificity of a cut-off value of US-ONSD (5.73 mm) to detect treatment indication for diseases associated with increased ICP was 92% and 86.4%, respectively, compared to US-ODE (0.43 mm) with sensitivity: 72%, specificity: 77.3%. Fundoscopic papilledema had a sensitivity of 46% and a specificity of 100% in this context. Repeatability and observer-reliability of US-ODE examination was eminent (Cronbach's α = 0.978-0.989). Papilledema was detected fundoscopically only when US-ODE was > 0.67 mm; a US-ODE > 0.43 mm had a positive predictive value of 90% for potentially increased ICP. CONCLUSION: In our cohort, transorbital point-of-care US-ONSD and US-ODE detected potentially elevated ICP requiring treatment in children more reliably than fundoscopy. US-ONSD and US-ODE indicated the decrease in ICP after treatment earlier and more reliably than fundoscopy. The established cut-off values for US-ONSD and US-ODE and a newly developed US-based grading of ODE can be used as an ideal first-line screening tool to detect or exclude conditions with potentially elevated ICP in children.

Global trends and decision-making in the management of arachnoid cysts.

OBJECTIVE: In pediatric patients, middle cranial fossa (MCF) arachnoid cysts are often discovered incidentally on imaging in asymptomatic patients during workup for other indications. This study aims to describe current management gestalt and threshold for surgical intervention by surveying an international cohort of neurosurgeons. METHODS: A web-based survey was circulated via email list of attendants of the 2019 Canadian Pediatric Neurosurgery Study Group (CPNSG) and International Society of Pediatric Neurosurgery (ISPN) mailing list. The survey consisted of 8 clinical scenarios involving patients with MCF arachnoid cysts. Demographic variables of respondents and their decisions regarding management for each scenario were analyzed using R computing software. RESULTS: A total of 107 respondents were included. Cysts in asymptomatic patients (92%), younger age at diagnosis (81%), and presence of a mild learning delay were predominantly managed non-surgically (80.7 ± 9.4%). Patients with cyst enlargement, headaches, new seizures, or hemorrhage were divided between non-surgical (55.8 ± 3.3%) and surgical (44.2 ± 2.9%) management. Patients with contralateral hemiparesis were treated predominantly surgically (67%). For both Galassi I and II, papilledema was favored as the primary indication for surgical intervention in 54% of patients. Those inclined to surgery (n = 17) were more likely to practice and train outside North America compared to those not pro-surgical (adjusted P = 0.092). CONCLUSION: Incidental MCF arachnoid cysts in asymptomatic patients and younger age of diagnosis are predominantly managed non-surgically. Mild learning delay was not considered an indication to intervene. In contrast, radiological progression, hemorrhagic evolution, or non-focal neurological deficits lead to uncertainty in management, while focal neurological deficits and papilledema with MCF cysts were favored to be intervened surgically. Among the provider level factors, only location of training and practice trended towards a pro-surgery approach.

Analysis of intracranial pressure waveform using a non-invasive method in individuals with craniosynostosis.

PURPOSE: Craniosynostosis can lead to symptoms resulting from cranial compliance (CC) changes and intracranial hypertension (ICH), which may cause cognitive and visual impairment. Non-invasive methods have emerged, including a new device that captures and processes the intracranial pressure waveform (ICPw) by the skull's oscillation. The present study evaluates ICPw obtained non-invasively (NIICPw) in patients with craniosynostosis. METHODS: This prospective, cross-sectional, and descriptive study was conducted at a single center. Patients diagnosed with craniosynostosis and who provided informed consent were included. A US Food and Drug Administration-approved mechanical extensometer device (Brain4Care Corp.) was used to obtain a NIICPw. An ophthalmologist did a point-of-care retinography to check the optic nerve papilla. The P2/P1 ratio and the morphology of the NIICPw were analyzed, as well as the retinography. RESULTS: Thirty-five patients were evaluated, and 42 registers were obtained because seven were assessed before and after the surgery. The two patients who presented papilledema had low CC (NIICPw shape Class 3 or 4). There was a significant association between NIICPw and papilledema. CONCLUSION: The ratio P2/P1 and the NIICPw morphology provided by a non-invasive monitor are related to CC changes before papilledema occurs. This is especially useful in patients with craniosynostosis because invasive ICP monitoring is not always feasible. Further studies are warranted to establish the clinical utility of NIICPw in patients with craniosynostosis.

Evaluation of Peripapillary and Macular Optical Coherence Tomography Angiography Characteristics in Different Stages of Papilledema.

BACKGROUND: Prospective evaluation of optical coherence tomography (OCT) and OCT angiography (OCT-A) characteristics in different stages of papilledema in idiopathic intracranial hypertension (IIH). METHODS: In this prospective, observational study patients of IIH with papilledema were recruited and divided into 3 groups-early/established (Group 1), chronic (Group 2), and atrophic papilledema (Group 3). Peripapillary retinal nerve fiber layer (RNFL) and macular ganglion cell inner plexiform layer (GC-IPL) were recorded on OCT. Peripapillary and macular perfusion was documented at superficial retinal, deep retinal, and choriocapillary level using OCT-A. The investigations were repeated at 3 months. RESULTS: RNFL showed significant thinning in all groups on follow-up with the atrophic group showing maximum thinning ( P = 0.01-Group 3). GC-IPL was significantly reduced in all stages of papilledema at baseline compared with the controls. Thinnest GC-IPL was noted in the atrophic group (52.75 ± 7.44 µm; P = 0.00 in Group 3 vs controls) that showed further deterioration on follow-up. On Image J analysis, significant decrease was noted at various levels in the peripapillary and macular perfusion at baseline especially in the atrophic group which showed further deterioration noted on follow-up. The final visual acuity showed a statistically significant weak negative correlation with baseline RNFL (r = -0.306) and GC-IPL (r = -0.384) and moderately negative correlation with baseline superficial peripapillary retinal perfusion (r = -0.553). A significant negative correlation was seen between increasing grade of papilledema and superficial peripapillary retinal perfusion with both Image J and automated indices (r = -0.46; r = -0.61), respectively. CONCLUSIONS: GC-IPL may help identify early damage in papilledema even in the presence of thicker RNFL. Significant vascular changes can be observed on OCT-A that may help predict the final visual outcome in papilledema due to IIH.

Early Detection of Optic Nerve Changes on Optical Coherence Tomography Using Deep Learning for Risk-Stratification of Papilledema and Glaucoma.

BACKGROUND: The use of artificial intelligence is becoming more prevalence in medicine with numerous successful examples in ophthalmology. However, much of the work has been focused on replicating the works of ophthalmologists. Given the analytical potentials of artificial intelligence, it is plausible that artificial intelligence can detect microfeatures not readily distinguished by humans. In this study, we tested the potential for artificial intelligence to detect early optic coherence tomography changes to predict progression toward papilledema or glaucoma when no significant changes are detected on optical coherence tomography by clinicians. METHODS: Prediagnostic optical coherence tomography of patients who developed papilledema (n = 93, eyes = 166) and glaucoma (n = 187, eyes = 327) were collected. Given discrepancy in average cup-to-disc ratios of the experimental groups, control groups for papilledema (n = 254, eyes = 379) and glaucoma (n = 441, eyes = 739) are matched by cup-to-disc ratio. Publicly available Visual Geometry Group-19 model is retrained using each experimental group and its respective control group to predict progression to papilledema or glaucoma. Images used for training include retinal nerve fiber layer thickness map, extracted vertical tomogram, ganglion cell thickness map, and ILM-RPE thickness map. RESULTS: Trained model was able to predict progression to papilledema with a precision of 0.714 and a recall of 0.769 when trained with retinal nerve fiber layer thickness map, but not other image types. However, trained model was able to predict progression to glaucoma with a precision of 0.682 and recall of 0.857 when trained with extracted vertical tomogram, but not other image types. Area under precision-recall curve of 0.826 and 0.785 were achieved for papilledema and glaucoma models, respectively. CONCLUSIONS: Computational and analytical power of computers have become an invaluable part of our lives and research endeavors. Our proof-of-concept study showed that artificial intelligence (AI) algorithms have the potential to detect early changes on optical coherence tomography for prediction of progression that is not readily observed by clinicians. Further research may help establish possible AI models that can assist with early diagnosis or risk stratification in ophthalmology.

Can optic disc vessel density help in cases of residual disc elevation after shunt surgery in cases of idiopathic intracranial hypertension?

AIM: To detect if we can use the reduction in the optic disc vessel density as an indicator to the reduction in intracranial tension in patients with residual optic disc elevation after shunt surgery as fundus examination in those cases is not conclusive. PATIENTS AND METHOD: 21 patients with papilledema due to idiopathic intracranial hypertension underwent shunt surgery. Full neurological and ophthalmological assessments were done. The optic disc vessel density was measured before and 3 months after surgery. Patients were then divided according to the resolution of papilledema into 2 groups: 1) Residual disc elevation group. 2) Completely resolved disc edema group. CSF pressure was measured via lumber puncture preoperative for all patients and 3 months post-operative only for patients with residual disc edema. A comparison between both groups was done. RESULTS: There was a highly statistically significant difference between the two groups as regard the papilledema grade (the residual disc elevation group had a higher grade of papilledema) with P-value=0.000. As regard the difference in the preoperative optic disc vessel density between the two groups, there were statistically significant differences (optic disc vessel density was more in the residual disc elevation group). As regard the postoperative optic disc vessel density, there were non-significant differences between the two groups in whole image, inside disc and peripapillary vessel density (either in macro or microvasculature). CONCLUSION: The optic disc vessel density decreased with normal postoperative CSF opening pressure in cases with residual disc elevation postoperatively. Thus, in cases of residual optic disc swelling after shunt surgery, we can detect the reduction of intracranial pressure by the reduction in the optic disc vessel density which is a safe non-invasive technique. That may help in cases of residual disc elevation.

Applicability of the New Myelin Oligodendrocyte Glycoprotein Antibody-associated Disease Diagnostic Criteria in an Israeli Cohort.

BACKGROUND: Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a rare autoimmune demyelinating disorder of the central nervous system. Optic neuritis (ON) is the most common clinical manifestation of MOGAD in adults. In 2023, new MOGAD diagnostic criteria were proposed, highlighting the importance of supplemental criteria when MOG-immunoglobulin G (IgG) titers are unavailable. OBJECTIVES: To investigate the applicability of the 2023 MOGAD criteria in patients diagnosed with MOGAD and treated before the availability of MOG-IgG titers. METHODS: We conducted a retrospective chart review of patients classified as MOGAD between 2010 and 2023 at Rabin Medical Center. Patient demographics as well as clinical and imaging data were collected, including visual acuity, expanded disability status score, core demyelinating events, antibody status, and brain and optic nerve magnetic resonance imaging data. Patients fulfilling the 2023 MOGAD criteria were reported as definite MOGAD. RESULTS: Fifteen patients met the 2023 MOGAD diagnostic criteria despite lack of MOG-IgG titer. The most common supplemental criterion meeting the 2023 MOGAD criteria was optic disc edema (n=12, 80%), followed by longitudinal optic nerve involvement (53%), bilateral ON (40%), and perineural optic sheath enhancement (33%). CONCLUSIONS: All patients with a clinical diagnosis of MOG-ON in our cohort fulfilled the 2023 MOGAD criteria despite the lack of antibody titers. The 2023 MOGAD criteria can be reliably applied to Israeli cohorts, prior to availability of MOGAD IgG titers, with particular attention to additional supplemental criteria. Since the 2023 MOGAD criteria were published, MOGAD IgG titers have been added to routine testing at our facility.

The additive value of complementing diagnostic idiopathic intracranial hypertension criteria by MRI - an external validation study.

BACKGROUND: Recently, diagnostic criteria including a standardized MRI criterion were presented to identify patients suffering from idiopathic intracranial hypertension (IIH) proposing that IIH might be defined by two out of three objective findings (papilledema, ≥ 25 cm cerebrospinal fluid opening pressure (CSF-OP) and ≥ 3/4 neuroimaging signs). METHODS: To provide independent external validation, we retrospectively applied the proposed diagnostic criteria to our cohort of patients with clinical suspicion of IIH from the Vienna IIH database. Neuroimaging was reevaluated for IIH signs according to standardized definitions by a blinded expert neuroradiologist. We determined isolated diagnostic accuracy of the neuroimaging criterion (≥ 3/4 signs) as well as overall accuracy of the new proposed criteria. RESULTS: We included patients with IIH (n = 102) and patients without IIH (no-IIH, n = 23). Baseline characteristics were balanced between IIH and no-IIH groups, but papilledema and CSF-OP were significantly higher in IIH. For the presence of ≥ 3/4 MRI signs, sensitivity was 39.2% and specificity was 91.3% with positive predictive value (PPV) of 95.2% and negative predictive value (NPV) 25.3%. Reclassifying our cohort according to the 2/3 IIH definition correctly identified 100% of patients without IIH, with definite IIH and suggested to have IIH without papilledema by Friedman criteria, respectively. CONCLUSION: The standardized neuroimaging criteria are easily applicable in clinical routine and provide moderate sensitivity and excellent specificity to identify patients with IIH. Defining IIH by 2/3 criteria significantly simplifies diagnosis without compromising accuracy.

[An analysis of the etiology composition and clinical characteristics of bilateral optic disc swelling].

Objective: To investigate the etiology composition and clinical characteristics of bilateral optic disc swelling(ODS). Methods: The medical records of all newly diagnosed bilateral ODS patients admitted to the neurology ward of Beijing Tongren Hospital from January 2017 to June 2021 were retrospectively searched to classify the etiology, obtain demographic and clinical information, and compare the differences in clinical characteristics. Results: A total of 131 patients with bilateral ODS were included, including 56 males and 75 females, aged 15-73 (39±14) years. The most common cause of the bilateral ODS was increased intracranial pressure (ICP)(56/131, 42.7%), followed by optic neuritis (ON)(40/131, 30.5%). Other causes included vascular optic neuropathy (13/131, 9.9%), pseudopilledema (9/131, 6.9%); uveitis (6/131, 4.6%), toxic optic neuropathy (3/131, 2.3%) and unknown causes (4/131, 3.1%). Idiopathic intracranial hypertension (IIH) (43/56, 76.8%) was the most common etiology for papilledema. In the comparison of the two main causes of intracranial hypertension and the clinical characteristics of ON in ODS, there was no statistically significant difference in the incidence of age, gender, complaints of ocular pain or headache, and hemorrhage of optic disc(P>0.05). Visual acuity abnormalities and low vision were more common in ON group than the increased ICP group[36/40(90%) vs 33/56(58.9%), P=0.001; 35/80(43.8%) vs 22/112(19.6%), P<0.001], while severe papilledema was more common in increased ICP group[38/112(33.9%)vs 9/80(11.3%), P<0.001]. Conclusions: The most common cause for bilateral ODS is increased ICP, but it can also be triggered by a variety of other causes. Optic neuritis(ON) is the most important differentiating disease in the study of Chinese patients.

[Pulmonary and testicular sarcoidosis revealed by optic disc edema]. / Cas clinique. Sarcoïdose pulmonaire et testiculaire révélée par un oedème de la tête du nerf optique.

Sarcoidosis is a chronic inflammatory granulomatous disease of unknown aetiology. Although the lungs and thoracic lymph nodes are the most frequently affected structures, every tissue can be affected. Ophthalmological involvement occurs in 25 % of cases. We report a clinical case in which the diagnosis of sarcoidosis was revealed by isolated unilateral optic disc edema.

La sarcoïdose est une pathologie inflammatoire granulomateuse chronique dont l'étiologie reste inconnue. Bien que les poumons et les ganglions thoraciques soient les structures les plus fréquemment atteintes, tous les tissus peuvent être affectés. L'atteinte ophtalmologique est présente dans 25 % des cas. Nous rapportons un cas clinique dont le diagnostic de sarcoïdose fut révélé par un œdème papillaire unilatéral isolé.

Diagnostic dilemma of papilledema and pseudopapilledema.

BACKGROUND: Papilledema is the optic disc swelling caused by increased intracranial pressure (ICP) that can damage the optic nerve and cause subsequent vision loss. Pseudopapilledema refers to optic disc elevation without peripapillary fluid that can arise from several optic disc disorders, with optic disc drusen (ODD) being the most frequent cause. Occasionally, pseudopapilledema patients are mistakenly diagnosed as papilledema, leading to the possibility of unneeded procedures. We aim to thoroughly examine the most current evidence on papilledema and pseudopapilledema causes and several methods for distinguishing between both conditions. METHODS: An extensive literature search was conducted on electronic databases including PubMed and google scholar using keywords that were relevant to the assessed pathologies. Data were collected and then summarized in comprehensive form. RESULTS: Various techniques are employed to distinguish between papilledema and pseudopapilledema. These techniques include Fundus fluorescein angiography, optical coherence tomography, ultrasonography, and magnetic resonance imaging. Lumbar puncture and other invasive procedures may be needed if results are suspicious. CONCLUSION: Papilledema is a sight-threatening condition that may lead to visual affection. Many disc conditions may mimic papilledema. Accordingly, differentiation between papilledema and pseudopailledema is crucial and can be conducted through many modalities.

Idiopathic intracranial hypertension: expanding our understanding.

PURPOSE OF REVIEW: Idiopathic intracranial hypertension (IIH) affects predominantly overweight women of childbearing age, causing chronically-disabling headaches and visual loss. Weight loss remains the most effective management strategy, but innovative treatments and randomized control trials (RCTs) remain few. This paper will review recent IIH research. RECENT FINDINGS: Pregnancy-related complications, but not losses, are increased in IIH, while symptom severity is not affected. Weight loss of 24% results in normalization of intracranial pressure (ICP) and improvement in papilledema. Prolonged periods of papilledema result in delayed thinning of the ganglion cell layer. Less-invasive telemetry has improved understanding of the positional effects on ICP with rises seen in the supine and lateral positions. Exenatide, a GLP-1 agonist, may reduce ICP and improve symptoms. Venous sinus stenting is increasingly popular but its benefits over CSF diversion remain unclear. SUMMARY: Early involvement of obstetric care is recommended with pregnancy in IIH. Early intervention is required to avoid chronic papilledema that confers worse visual outcomes. Positional changes may affect ICP readings. The use of novel ICP telemetric devices has significant potential in future disease monitoring. The dual benefits of weight loss and ICP reduction with exenatide have significant potential in IIH management. Surgical RCTs are still required.

Expanding the clinical spectrum of idiopathic intracranial hypertension.

PURPOSE OF REVIEW: Idiopathic intracranial hypertension (IIH) is a disorder of raised intracranial pressure (ICP). Although the majority of patients with IIH present classically with headache and papilledema, some patients may have unusual presentations or manifestations. Recent advancements in neuroimaging have facilitated the identification of other presentations associated with IIH. This review provides an overview of the expanding clinical spectrum of IIH. RECENT FINDINGS: Presentations of IIH that are considered unusual include highly asymmetric or unilateral papilledema, IIH without papilledema, and IIH associated with cranial nerve involvement. These presentations likely reflect differences in the way cerebrospinal fluid (CSF) pressure is transmitted intracranially. Radiological signs of intracranial hypertension are increasingly recognized in patients with IIH and provide further insights into the effects of raised ICP on intracranial structures. Osseous changes in the skull base leading to formation of meningoceles and encephaloceles have been identified in patients with IIH, spontaneous skull base CSF leak, and drug-resistant temporal lobe epilepsy, suggesting a possible association. SUMMARY: Clinicians should be familiar with the expanding clinical spectrum of IIH and the implications for the management of these presentations.

Artificial Intelligence to Detect Papilledema from Ocular Fundus Photographs.

BACKGROUND: Nonophthalmologist physicians do not confidently perform direct ophthalmoscopy. The use of artificial intelligence to detect papilledema and other optic-disk abnormalities from fundus photographs has not been well studied. METHODS: We trained, validated, and externally tested a deep-learning system to classify optic disks as being normal or having papilledema or other abnormalities from 15,846 retrospectively collected ocular fundus photographs that had been obtained with pharmacologic pupillary dilation and various digital cameras in persons from multiple ethnic populations. Of these photographs, 14,341 from 19 sites in 11 countries were used for training and validation, and 1505 photographs from 5 other sites were used for external testing. Performance at classifying the optic-disk appearance was evaluated by calculating the area under the receiver-operating-characteristic curve (AUC), sensitivity, and specificity, as compared with a reference standard of clinical diagnoses by neuro-ophthalmologists. RESULTS: The training and validation data sets from 6779 patients included 14,341 photographs: 9156 of normal disks, 2148 of disks with papilledema, and 3037 of disks with other abnormalities. The percentage classified as being normal ranged across sites from 9.8 to 100%; the percentage classified as having papilledema ranged across sites from zero to 59.5%. In the validation set, the system discriminated disks with papilledema from normal disks and disks with nonpapilledema abnormalities with an AUC of 0.99 (95% confidence interval [CI], 0.98 to 0.99) and normal from abnormal disks with an AUC of 0.99 (95% CI, 0.99 to 0.99). In the external-testing data set of 1505 photographs, the system had an AUC for the detection of papilledema of 0.96 (95% CI, 0.95 to 0.97), a sensitivity of 96.4% (95% CI, 93.9 to 98.3), and a specificity of 84.7% (95% CI, 82.3 to 87.1). CONCLUSIONS: A deep-learning system using fundus photographs with pharmacologically dilated pupils differentiated among optic disks with papilledema, normal disks, and disks with nonpapilledema abnormalities. (Funded by the Singapore National Medical Research Council and the SingHealth Duke-NUS Ophthalmology and Visual Sciences Academic Clinical Program.).

Timolol maleate, a ß blocker eye drop, improved edema in a retinal vein occlusion model.

Purpose: To investigate the therapeutic effects of eye drops, namely, timolol maleate, a ß-adrenergic receptor antagonist, and latanoprost, a prostaglandin F2α analog, on retinal edema in a murine retinal vein occlusion (RVO) model. Methods: An RVO model was established using laser-induced RVO in mice, which were administered timolol maleate and latanoprost eye drops several times after venous occlusion. Subsequently, the thickness of the inner nuclear layer (INL) and the expression levels of such genes as Vegf and Atf4, which are stress markers of the endoplasmic reticulum, were examined. Primary human cultured retinal microvascular endothelial cells (HRMECs) were treated with timolol under hypoxic conditions, after which the gene expression pattern was investigated. Importantly, an integrated stress response inhibitor (ISRIB) was used in the RVO model, he known ISRIB, which suppresses the expression of ATF4 in retinal edema. Results: Increased INL thickness was suppressed by timolol eye drops, as were the expressions of Vegf and Atf4, in the RVO model. However, latanoprost eye drops did not induce any change in INL thickness. In HRMECs, hypoxic stress and serum deprivation increased the Vegf and Atf4 expressions; in response, treatment with timolol suppressed the Vegf expression. Furthermore, the ISRIB decreased the Vegf expression pattern and edema formation, which are associated with RVO. Conclusions: These results indicate that timolol eye drops may be a potential option for RVO treatment.