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Nitrous oxide is used as an anaesthetic and analgesic agent in the medical setting and is known to cause raised intracranial pressure. The use of nitrous oxide recreationally for the drug's euphoric and relaxant properties has been linked to multiple neurological and psychiatric sequelae including neuropathy, myelopathy, and psychosis. We describe a case of a young person who declared heavy nitrous oxide use resulting in vision-threatening papilloedema secondary to raised intracranial pressure. He underwent emergency lumbar drainage alongside high-dose acetazolamide and parenteral vitamin B12 injections. To our knowledge, there have yet to be other reports of cases where heavy nitrous oxide use has caused secondary pseudotumor cerebri syndrome. [ABSTRACT FROM AUTHOR]

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Arachnoid cysts (ACs) are cerebrospinal fluid collections between the two layers of the normal arachnoid membrane. Although they are often asymptomatic with a stationary course, eventual complications may occur. Herein, we report the case of a 9-year-old boy who developed bilateral papilloedema secondary to spontaneous rupture of an AC in the left middle cranial fossa. Although the papilloedema worsened during follow-up, his visual field remained bilaterally stable, supporting the expectant management and obviating the potential morbidity associated with neurosurgical intervention. This case report highlights the importance of a multidisciplinary approach to patients with secondary intracranial hypertension, including serial ophthalmological examinations, which provide a useful guide to surgical decision-making. [ABSTRACT FROM AUTHOR]

Resumen: Introducción: La trombosis venosa cerebral (TVC) es una causa poco común de ictus que afecta principalmente a adultos jóvenes. Un diagnóstico precoz y preciso puede reducir la tasa y gravedad de las complicaciones. Objetivo: Analizar las características clínicas, manejo y tratamiento de la TVC en diferentes centros de nuestro país. Métodos: Estudio descriptivo retrospectivo multicéntrico de pacientes hospitalizados por TVC entre 2008 y 2017 en 11 centros sanitarios en nuestro país. Resultados: Se incluyeron 256 pacientes, edad media 49,8 ± 18,7 años y el 51% fueron mujeres. Los síntomas más frecuentes fueron: cefalea (73%), déficits focales (50%), crisis epilépticas (33%) y encefalopatía (21%). Las localizaciones más frecuentes fueron: seno longitudinal superior (12,5%), transverso (10,9%) y afectación de dos o más senos o venas (66,4%). La etiología conocida más frecuente fue la trombofilia (24%), siendo la mutación de la protrombina G20210A la más común (19%). El 46% fue tratado con antitrombóticos durante 3-6 meses, el 21% durante un año y un 22,6% de los pacientes requirieron anticoagulación indefinida. En un 5% de los sujetos fue preciso terapia endovascular y un 33% requirió neurocirugía. En relación al pronóstico, el 75% fueron independientes a los 3 meses con una puntuación en la escala de Rankin modificada (mRS) ≤ 2 y la presencia de papiledema (p = 0,03), déficit focal (p = 0,001) y encefalopatía (p 3). La tasa de mortalidad intrahospitalaria fue del 4,3% y el 6,3% de los pacientes fallecieron a los 3 meses. Conclusión: La diversidad de factores de riesgo y la presentación variable suponen un desafío en el diagnóstico y tratamiento de la TVC. Para mejorar el pronóstico y reducir la mortalidad es fundamental la instauración de protocolos en el manejo de esta patología. Abstract: Introduction: Cerebral venous thrombosis (CVT) is an uncommon cause of stroke that mainly affects young adults. Early, accurate diagnosis can reduce the rate and severity of complications. Objective: The aim of this study was to analyse the clinical characteristics, management, and treatment of CVT in different centres in Spain. Methods: We conducted a multicentre, retrospective, descriptive study of patients hospitalised due to CVT between 2008 and 2017 at 11 Spanish centres. Results: We included 256 patients, with a mean age (SD) of 49.8 (18.7) years; 51% of patients were women. The most frequent symptoms were headache (73%), focal deficits (50%), epileptic seizures (33%), and encephalopathy (21%). The most frequent localisations were the superior sagittal sinus (12.5%), the transverse sinus (10.9%), and 2 or more sinuses or veins (66.4%). Thrombophilia was the most frequent known aetiology (24%), and was most commonly associated with the prothrombin G20210A mutation (19%). Forty-six percent of patients were treated with antithrombotics for 3-6 months, 21% for one year, and 22.6% required indefinite anticoagulation. Endovascular therapy was performed in 5% of cases, and 33% required neurosurgery. Regarding outcomes, 75% of patients were independent at 3 months (modified Rankin Scale [mRS] score ≤ 2), with papilloedema (P = .03), focal deficits (P = .001), and encephalopathy (P 3). The in-hospital mortality rate was 4.3%, with a 3-month mortality rate of 6.3%. Conclusion: The diverse risk factors and variable presentation of CVT represent a challenge in the diagnosis and treatment of this condition. To improve prognosis and reduce mortality, it is essential to establish management protocols for this entity.

Purpose: Idiopathic intracranial hypertension (IIH) is a rare condition in children, but if diagnosed needs to be promptly treated to avoid clinical sequalae. The main purpose of this paper was to test our clinical experience with a cohort of normocephalic children with craniosynostosis who do not present in the routine way to craniofacial services, due to the normal head shape and age, diagnosed with IIH. Methods: We retrospectively reviewed all children who were referred to neurosurgery from 2012 to 2022 for management of IIH on our prospectively kept database. We determined what treatments were offered and if there was an associated craniosynostosis. Results: In total, 19 children were identified with an average age at referral of 11.5 years (st dev 4.0 years) with 11 male and 8 female. The most common presenting symptoms and signs were papilloedema (18/19), headaches (15/19), visual deterioration (9/19), nausea and vomiting (7/19) and diplopia (4/19). Five out of 19 children (26.3%) had a sagittal suture fused that was not identified at the time of treatment and all children were normocephalic. Conclusion: There is a cohort of children with IIH who will have concomitant craniosynostosis and ideally would benefit from cranial vault expansion as primary surgery rather than cerebrospinal fluid (CSF) diversion. We suggest all children with IIH requiring neurosurgical intervention have cross-sectional imaging to look for occult craniosynostosis prior to intervention. [ABSTRACT FROM AUTHOR]

Idiopathic intracranial hypertension (IIH) predominantly affects reproductive-aged females with obesity. However, the prevalence and impact of a healthy weight body mass index (BMI) at disease presentation is not known. This study aimed to evaluate the visual and headache outcomes stratified by the presenting BMI. This was a longitudinal prospective cohort study (IIH Life) based on a tertiary neuro-ophthalmology IIH service, University Hospitals Birmingham NHS Trust, United Kingdom, recruiting consecutive patients living with IIH between 2012 and 2021. Those with a presenting BMI were included. The outcome measures included visual outcomes of LogMAR visual acuity, Humphrey visual field perimetric mean deviation (PMD), optical coherence tomography (OCT) measurements, and headache outcomes of frequency, severity, and Headache Impact Test-6 score. Three hundred seventy-five people with IIH and a documented baseline BMI. About 3.7% of the entire cohort had a healthy weight BMI at IIH presentation and 15.5% BMI < 30 kg/m2. The baseline PMD was worse in patients without obesity; however, OCT papilloedema measures were similar. The presence of obesity was associated with a small but significant greater worsening in visual acuity but slower macular ganglion cell layer loss. There was no impact on PMD or papilloedema prognosis related to baseline obesity. The headache outcomes showed heterogeneity, with worse baseline headache frequency in patients with obesity. No BMI group was associated with worse headache outcomes. Patients with a healthy weight BMI or those without obesity at baseline make up a small proportion of IIH patients. BMI at presentation does not appear to influence long-term visual or headache outcomes. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Neuro-ophthalmologists commonly receive referrals for possible papilloedema. One common cause of oedematous-appearing optic nerves is optic disc drusen (ODD). We describe a novel, handheld, portable ultrasound device that can be used to identify ODD in cases of suspected papilloedema. We demonstrate here that this portable ultrasound system can detect ODD in the outpatient ophthalmology and neurology settings. The portable version demonstrates several advantages. The device can be carried into the office, readily reducing visit times and office space. It is much more affordable than a traditional ultrasound machine. Images can be uploaded to a secure server and imported into the patient's chart. These cases represent one potential ophthalmological application of this novel technology that could augment the ophthalmological examination and improve patient care in the future. [ABSTRACT FROM AUTHOR]

Background: Any condition which causes increased intracranial pressure can result in Papilloedema, of which greatest number are due to Meningitis and Tumours of Brain. Papilloedema is much more frequently observed in children than in older people. It can cause various visual field defects of which concentric enlargement of blind spot is most common. Papilledema may lead to diagnostic difficulties in its different stages. It should be differentiated from optic neuropathies and structural abnormalities of optic disk known as pseudo Papilloedema. This study was done for evaluating causes of Papilloedema and to study age, sex and causal distribution of Papilloedema. Methodology: This Descriptive, Observational study was conducted in Department of Ophthalmology, in our Hospital from September 2021 to September 2022. Detailed history was recorded. Assessment of anterior segment via slit lamp biomicroscopy was done. Depending on cause of Papilloedema, CT or MRI scan of head was done. Ancillary investigations like CBC, CSF analysis and other blood investigations were done whenever required. Result: A total of 50 patients with Papilloedema were included in the study. Maximum number of patients belonged to third and fourth decade. Bilateral papilloedema was usual presentation. The major etiological factor was Meningitis followed by brain tumours and other causes. Conclusion: Papilloedema is seen in all age groups but is more common in younger and middle age groups. Meningitis and Brain Tumours were major etiological factors for Papilloedema. Tuberculous meningitis causes Papilloedema more often than any other form of meningitis.

Abstract Background Case presentation Conclusion Antiphospholipid antibody (APLA) syndrome is an autoimmune disorder predisposing to thrombotic complications affecting CNS either by arterial vaso occlusion or venous thrombosis. Cerebral venous sinus thrombosis (CVST) secondarily produces raised intracranial pressure (ICP). However intracranial hypertension without evidence of CVST is rare entity.We present two cases of elevated ICP with absence of identifiable CVST. Case 1, a 28-year-old female presented with a 2 months history of headache followed by bilateral vision loss. Cerebrospinal fluid (CSF) opening pressure and fundoscopy along with Contrast Magnetic resonance imaging (MRI) was suggestive of Idiopathic intracranial hypertension (IIH) and patient improved with acetazolamide. 5 months later she presented with acute onset right sided hemiparesis. MRI showed acute left Middle cerebral artery (MCA) territory infarct with normal contrast Magnetic resonance venography (MRV). Anti-cardiolipin and Beta 2 glycoprotein (both IgG and IgM) titres were elevated. Case 2, a 23-year-old female presented with headache and diplopia of 2 months duration. Based on CSF, fundoscopy and contrast MRI brain was diagnosed as IIH and she too responded to diuretics. 2 years later she presented with recurrence of headache and APLA profile showed elevated beta 2 glycoprotein IgG and IgA.This is an important non thrombotic complication of APLA syndrome and requires further large-scale study for insight into the pathogenesis and early recognition to avoid future complications. [ABSTRACT FROM AUTHOR]

Styloidogenic jugular venous compression syndrome has been recently described as a new cause of idiopathic intracranial hypertension. We present a 69-year-old patient, without other relevant medical history, presenting with 3 years of positional headache associated with decreased vision when reading and while turning her head to the right or left. She also reported pulsatile low-frequency tinnitus. Papilloedema was noted on the physical examination and, on imaging, an enlarged styloid process that induced jugular vein compression. The patient underwent styloidectomy with resolution of her symptoms and normalisation of her visual fields.

Suspected optic disc swelling is a common presentation in children. The delineation between true optic disc swelling and pseudopapilloedema, its common masquerade, requires careful evaluation. A streamlined pathway is required to avoid unnecessary investigations. Papilloedema requires urgent neuroimaging, however, perceived optic disc swelling is not always true papilloedema. This study aims to investigate the outcome of referrals for optic disc swelling and formulate features that may assist in investigation and diagnosis. A retrospective review of referrals for optic disc swelling to the Queensland Children's Hospital, Australia, between January 2014 and June 2020 was undertaken. Four hundred and ten children were referred for optic disc swelling. Sixty-six patients were confirmed with optic disc swelling, and 344 patients had pseudopapilloedema. The average age was 10.10 ± 3.57 and 9.90 ± 3.50 years, respectively. The most common aetiology of optic disc swelling was idiopathic intracranial hypertension (n = 25). Optic disc drusen constituted the majority of pseudopapilloedema (n = 239) and the remainder were crowded/tilted discs (n = 105). True optic disc swelling patients were more likely to experience headache (OR = 8.68, p < 0.01) and visual disturbance (OR = 2.14, p = 0.03). B-scan was the most sensitive for the detection of optic disc drusen (100%), followed by optical coherence tomography (70.38%) and fundus autofluorescence (44.86%). The retinal nerve fibre layer thickness was significantly thicker in true optic disc swelling compared to pseudopapilloedema (p < 0.01). Twenty-two (33.33%) true optic disc swelling patients and 33 (9.59%) pseudopapilloedema patients underwent neuroimaging prior to ophthalmology review. Suspected optic disc swelling in children is most likely pseudopapilloedema. Referrals should include neurological assessment, visual acuity, fundus photography, and optical coherence tomography to assist in the triage for ophthalmic review. Neuroimaging prior to ophthalmic review should be discouraged for children without neurological symptoms. [ABSTRACT FROM AUTHOR]

The quality of clinical trials is essential to advance treatment, inform regulatory decisions and meta-analysis. With the increased incidence of idiopathic intracranial hypertension and the emergence of clinical trials for novel therapies in this condition, the International Headache Society Guidelines for Controlled Clinical Trials in Idiopathic Intracranial Hypertension aims to establish guidelines for designing state-of-the-art controlled clinical trials for idiopathic intracranial hypertension. [ABSTRACT FROM AUTHOR]

Optic disc swelling is a manifestation of a broad range of processes affecting the optic nerve head and/or the anterior segment of the optic nerve. Accurately diagnosing optic disc oedema, grading its severity, and recognising its cause, is crucial in order to treat patients in a timely manner and limit vision loss. Some ocular fundus features, in light of a patient's history and visual symptoms, may suggest a specific mechanism or aetiology of the visible disc oedema, but current criteria can at most enable an educated guess as to the most likely cause. In many cases only the clinical evolution and ancillary testing can inform the exact diagnosis. The development of ocular fundus imaging, including colour fundus photography, fluorescein angiography, optical coherence tomography, and multimodal imaging, has provided assistance in quantifying swelling, distinguishing true optic disc oedema from pseudo-optic disc oedema, and differentiating among the numerous causes of acute optic disc oedema. However, the diagnosis of disc oedema is often delayed or not made in busy emergency departments and outpatient neurology clinics. Indeed, most non-eye care providers are not able to accurately perform ocular fundus examination, increasing the risk of diagnostic errors in acute neurological settings. The implementation of non-mydriatic fundus photography and artificial intelligence technology in the diagnostic process addresses these important gaps in clinical practice. [ABSTRACT FROM AUTHOR]

Bhim B Rai,1,2 Ozge Sarac,3,4 Joshua P van Kleef,1 Ted Maddess1 1John Curtin School of Medical Research, Australian National University, Canberra, ACT, Australia; 2Department of Ophthalmology, Jigme Dorji Wangchuck National Referral Hospital, Thimphu, Bhutan; 3Department of Ophthalmology, AYBU Ankara City Hospital, Ankara, Turkey; 4Wills Eye Hospital Neuro-Ophthalmology Department, Philadelphia, PA, USACorrespondence: Bhim B Rai, John Curtin School of Medical Research, Australian National University, 131 Garran Road, Acton, Canberra, ACT, 2607, Australia, Tel +61 2 6125 9253, Email bhim.rai@anu.edu.auPurpose: To inform national health policy, we quantified the pattern of neuro-ophthalmological disorders (NODs) presenting to the national vitreoretinal clinics in Bhutan.Study Design: Retrospective cross-sectional study.Methods: We reviewed all new NODs patients over three years. Demographic data, presenting complaints, treatment history, systemic diseases, diagnostic procedures, and diagnoses were quantified. Logistic regression examined the odds of factors linked to more common NODs.Results: Of 226 patients, the majority were males (54.0%), farmers (60.2%), and urbanites (55.8%). Loss of vision was the most common presenting complaint (57.9%), followed by head or orbital trauma (19.5%). The best corrected visual acuity (BCVA) of 216 eyes (47.8%) was ≤ 6/60. Hypertension was the most common systemic disease (16.4%), followed by diabetes (3.5%), and intracranial space-occupying lesions (3.5%). Neuroimaging (37.6%) was the most common diagnostic test performed, followed by visual field testings (VFTs) (22.9%). With a NOD incidence of 7.8% p.a. (226/2913), optic atrophy (OA) was diagnosed in 134 patients (59.3%). Other common NODs were optic neuritis (15.5%), papilloedema (9.3%), and traumatic optic neuropathy (8.4%). Female gender increased the odds for glaucomatous OA by 2.65× (p = 0.044), and age by 1.09× per year (p < 0.001). Being female increased the odds of optic neuritis by 2.57× (p = 0.03).Conclusion: Over half of the NODs were OA, which could be curable with timely treatment. Improved treatment of glaucoma and non-communicable diseases would reduce the risk of NODs-induced visual loss in Bhutan. The need for improved neuro-ophthalmological assessment and a coordinated multidisciplinary approach to NODs are the highest priorities.Keywords: Bhutan eye diseases, neuro-ophthalmological disorders, optic atrophy, optic neuritis, papilloedema

A 10‐month‐old female, neutered French Bulldog presented with a history of unilateral right‐sided intermittent conjunctivitis and exophthalmos. The patient suffered blunt force trauma to the right eye after a tennis ball impact approximately five months prior to presentation. Examination identified the patient was visual with exophthalmos, lateral strabismus, conjunctival hyperaemia, episcleral congestion, and papilloedema. Magnetic resonance imaging and ultrasonography identified an approximately two centimeter diameter fluid‐filled structure directly posterior to the globe leading to displacement of the optic nerve and distortion of the posterior globe wall. Centesis of the lesion demonstrated neutrophilic and macrophagic inflammation with evidence of prior hemorrhage. Within four weeks, the structure had re‐filled to its original size and was subsequently excised in its entirety, via a lateral orbitotomy. Histopathologic findings indicated a non‐keratinising orbital cyst, the lining of which was consistent with a respiratory epithelial cyst. Recurrence had not occurred seven months' post‐surgery. To the author's knowledge, previous reports of retrobulbar respiratory epithelial cysts have not been documented in animals. [ABSTRACT FROM AUTHOR]

Idiopathic intracranial hypertension (IIH) affects both children and adults. There are currently no clinical trials in IIH for those who are adolescents or children. The aims of this narrative review were to characterise the differences between pre- and post-pubertal IIH and to highlight the need to be more inclusive in clinical trial planning and recruitment. A detailed search of the scientific literature was performed using the PubMed database, from inception until 30 May 2022 using keywords. This included English language papers only. The abstracts and full texts were reviewed by two independent assessors. The literature revealed that the pre-pubertal group had a more variable presentation. The presenting features in the post-pubertal paediatric group were more akin to adults with headache as the dominant feature. They were also more likely to be female and have an increased body mass index. A clear limitation of the literature was that a number of paediatric studies had variable inclusion criteria, including secondary causes of raised intracranial pressure. Pre-pubertal children do not display the same predilection towards the female sex and obesity as post-pubertal children, who have a similar phenotype to the adult cohort. Inclusion of adolescents in clinical trials should be considered given the similar phenotype to adults. There is a lack of consistency in the definition of puberty, making the IIH literature difficult to compare. Inclusion of secondary causes of raised intracranial pressure has the potential to confound the accuracy of analysis and interpretation of the results. [ABSTRACT FROM AUTHOR]

A 38-year-old woman who presented with painless vision loss in the left eye over the course of 1 week was investigated at a tertiary neuro-ophthalmology clinic. She was otherwise asymptomatic with no reported headaches, focal neurological deficits, anosmia, or behavioural changes. Bilateral optic disc oedema was identified on examination. Neuroimaging and then resection and histopathological evaluation demonstrated a meningothelial meningioma centred on the left sphenoid ridge. The left optic disc later became atrophic. We have therefore described a case of type 2 Foster Kennedy syndrome with unilateral vision loss as the only initial manifestation. [ABSTRACT FROM AUTHOR]

An initial misdiagnosis of papilloedema in a patient with optic nerve head swelling can be anxiety-provoking and may result in unnecessary, invasive, and costly tests. Cirrus high definition, spectral domain-optical coherence tomography (Cirrus HD-OCT) may provide a rapid and non-invasive test. We sought to determine an upper limit of average retinal nerve fibre layer (RNFL) thickness in patients with pseudopapilloedema without visible drusen using Cirrus HD-OCT that could be utilised in conjunction with the clinical presentation and physical examination when managing patients with optic nerve head swelling. Inclusion criteria consisted of at least two neuro-ophthalmological visits and repeated imaging of the optic nerve head with Cirrus HD-OCT at least 6 months apart. Exclusion criteria included clinically visible drusen along with previous or concomitant diagnosis of retinal or other optic nerve pathology. Thirty-eight eyes from 19 patients with pseudopapilloedema were included in this study. The upper limit of average RNFL thickness was defined as two standard deviations above the mean of the average RNFL thickness and was calculated to be 158.65 µm for scans obtained with Cirrus HD-OCT devices. A patient with suspected optic nerve head swelling, an average RNFL thickness less than 158.65 µm, and no other evidence of papilloedema or neurological signs or symptoms can be managed with serial follow-ups with OCT imaging for at least 6 months. If the patient continues to have no clinical symptoms suggesting increased intracranial pressure and the average RNFL thickness is stable, the likelihood of papilloedema is minimal. [ABSTRACT FROM AUTHOR]

Objective: Idiopathic intracranial hypertension (IIH) predominantly affects women of reproductive age with obesity, and these women have a distinct profile of hyperandrogenism and insulin resistance. Polycystic ovary syndrome (PCOS) has an established adverse fertility phenotype that typically affects obese women. As IIH may impact reproductive health, we sought to evaluate fertility, gestational complications and pregnancy outcome in IIH. Design: Prospective cohort study from English Hospital Episode Statistics dataset. Setting: English hospitals, UK. Population: Women aged 18–45 years seen in English hospitals between 1 April 2002 and 31 March 2019. Patients were required to have an IIH diagnosis and were compared with those with PCOS and general population female controls. Main outcome measures: Pregnancies resulting in live births, complications of gestational diabetes and pre‐eclampsia, and method of delivery. Results: Data was collected from 17 587 IIH, 199633 PCOS and 10 947 012 women in the general population. The live birth rate, adjusted for age, was significantly lower among women with IIH (54.1%) than PCOS (67.9%), p < 0.0001 and the general population (57.7%), p < 0.0001. Pre‐eclampsia and gestational diabetes risks were higher following a diagnosis of IIH (5.3‐fold and 2.7‐fold, respectively, p < 0.0001) compared with the general population controls. Following a diagnosis of IIH, elective caesarean section rates were more than twice that of general population (odds ratio [OR] 2.4) and prior to a diagnosis of IIH (OR 2.2). Conclusions: These data indicate there are lower age‐adjusted total pregnancy rates, increased risk of pre‐eclampsia and gestational diabetes, and a doubling of elective caesarean section rates in those with a diagnosis of IIH. This article includes Author Insights, a video abstract available at: https://vimeo.com/bjogabstracts/authorinsights17241 [ABSTRACT FROM AUTHOR]

Abstract Background There is only one documented case of intracranial hypertension (IH) and empty sella from cortisol-producing adrenal adenoma so far. And IH and empty sella caused by long-term exogenous hypercortisolism has never been reported before. The purpose of this case report is to alert clinicians to glucocorticoid-induced IH. Case presentation We present retrospectively a 50-year-old woman with cortisol-secreting adrenal adenoma, who progressed to intractable intracranial hypertension and a markedly expanded empty sella due to improper treatment. In 2011, the patient presented with hypertension, lack of cortisol circadian rhythm, low ACTH, a left adrenal adenoma and a partial empty sella, but did not receive low-dose dexamethasone suppression test (LDDST) and 24-h urinary cortisol. In 2014, she exhibited truncal obesity, raised cortisol, LDDST non-suppression, high urinary free cortisol and low ACTH, proving her cortisol-producing adrenal adenoma. She was simultaneously diagnosed with unexplained IH because of papilledema and elevated intracranial pressure, and her partial empty sella changed to a complete empty sella. In 2015, she underwent adrenal adenoma resection. From 2015 to 2018, she kept taking dexamethasone at least 2 mg daily without her doctors’ consent. During this period, she developed transient cerebrospinal fluid rhinorrhea, and her empty sella further worsened. After switching to low dose hydrocortisone, her papilledema disappeared completely, but optic atrophy has become irreversible. Conclusions The patient seems to be just an extreme case, but it may reveal and illustrate a general phenomenon: Both cortisol-producing adrenal adenoma and long-term exogenous hypercortisolism could cause varying degrees of elevated intracranial pressure and empty sella. Clinicians should remain vigilant for this phenomenon in patients with cortisol-producing adrenal adenoma or excessive and prolonged steroid usage and give them corresponding examinations to identify this complication.

Abstract Background Idiopathic intracranial hypertension is an infrequent condition of childhood, and is extremely rare in infants, with only 26 cases described. The etiology is still unknown. Typical clinical manifestations change with age, and symptoms are atypical in infants, thus the diagnosis could be late. This is based on increased opening pressure at lumbar puncture, papilloedema and normal cerebral MRI. The measurement of cerebrospinal fluid opening pressure in infants is an issue because many factors may affect it, and data about normal values are scanty. The mainstay of treatment is acetazolamide, which allows to relieve symptoms and to avoid permanent visual loss if promptly administered. Case presentation We report the case of an 8-month-old infant admitted because of vomit, loss of appetite and irritability; later, also bulging anterior fontanel was observed. Cerebral MRI and cerebrospinal fluid analysis resulted negative and after two lumbar punctures he experienced initial symptom relief. Once the diagnosis of idiopathic intracranial hypertension was made, he received oral acetazolamide, and corticosteroids, with progressive symptom resolution. Conclusions Infantile idiopathic intracranial hypertension is extremely rare, and not well described yet. Bulging anterior fontanel in otherwise healthy infants with normal neuroimaging should be always considered suggestive, but can be a late sign, while irritability and anorexia, especially if associated with vomiting, may represent an early sign. In such cases, lumbar puncture should be always done, hopefully with cerebrospinal fluid opening pressure measurement, which is among coded diagnostic criteria, but whose threshold is controversial in infants. Early diagnosis, timely treatment and strict follow-up help to prevent vision loss or death of affected infants.

Introduction: All metastatic malignancies commonly metastasise to choroid tissue in eye due to its peculiarity in vascular properties; thereof ocular manifestations are most common in the practice of oncology and ophthalmology. Aim: To investigate the prevalence and clinical pattern of choroidal metastases in myriad of primary malignancies Materials and Methods: This cross-sectional study was conducted from December 2017 to July 2019 , at Krishna Institute of Medical Sciences, Karad, Maharashtra, on 253 patients with confirmed diagnosis of primary malignancies of breast, lung and ovary with one or more distant metastases with or without ocular symptoms. All patients underwent thorough ophthalmic examination, Ultrasonography (USG) and Magnetic Resonance Imaging (MRI). The continuous and categorical variables were presented in mean±standard deviation and percentages, respectively. Results: Out of 253 patients, 49 (19.4%) were of breast carcinoma, 52 (20.5%) of lung carcinoma, 127 (50.2%) had gastrointestinal tract malignancy, 8 (3.2%) were of renal cell carcinoma, and 17 (6.7%) had prostate carcinomas. Prevalence of choroidal metastases was 5 (1.9%) of which 1 (20%) in breast and 2 (40%) each in lung, and gastrointestinal carcinomas. Patients with choroidal metastasis were presented with diplopia, pain, and diminished vision. Out of five patients with choroidal metastasis, three patients had choroidal metastasis on right and two had on left-side. Amongst these patients, three had advanced disease, two had retinal detachment, and one had vitreous haemorrhage. USG revealed polygonal heterogeneous echoic mass lesion with irregular surface contour and internal vascularity. MRI revealed T1 isointense, T2 hypointensity well demarcated nodular mass lesion with broad base. Conclusion: The prevalence of choroidal metastases is very less and tends to occur in the patients with advanced disease.

Context: Papilloedema is defined as optic disc oedema secondary to raised intracranial pressure (ICP). Grading the degree of oedema using only subjective, examiner-dependent and non-quantitative fundoscopic classification like Modified Friesen Clinical Scale (MFS), may be subject to errors even when used by experienced examiners. The use of spectral-domain optical coherence tomography (SD-OCT), a non-invasive imaging technique, for early identification of raised ICP even before the clinical appearance of papilloedema by quantifying the thickness of the peripapillary retinal nerve fibre layer (ppRNFL) is crucial for such patients. Aims: To evaluate the ppRNFL using SD-OCT in patients with papilloedema. Settings and Design: Hilly North Indian population, Prospective case-control study. Methods and Material: From January 2018 to December 2018, a total of 45 patients diagnosed with papilloedema were included. Age and sex-matched controls were recruited. Detailed demographic information and the history of study subjects were noted. All subjects underwent systemic and detailed ophthalmic examination. Disc photographs were graded as per Modified Frisen Criteria (MFS). SD-OCT of the optic nerve head was done to provide a mean peripapillary RNFL thickness parameters in the different quadrants of the disc in each eye. Statistical Analysis Used: For analysis, statistical software SPSS version 23 and appropriate statistical tests were applied. P value < 0.05 was considered significant. Results: The median age of presentation was 36 years (Range 10–68 years). The male to female ratio was 1:1.3. The mean ppRNFL thickness of both right and left disc in all quadrants was significantly higher in cases in comparison to controls (P-value < 0.05 is significant). The average ppRNFL thickness in all the quadrants of both eyes was significantly higher in the mild grade of papilloedema in comparison to controls (P-value < 0.05 is significant). Conclusions: SD-OCT can be ordered as a routine important non-invasive investigation in addition to basic fundoscopic examination in all patients with papilloedema or suspected to have papilloedema.

Purpose: To map the morphology of the retina and optic disc in adolescents with surgically treated hydrocephalus (HC) in infancy and to compare the results with healthy controls. Methods: The study comprised 26 adolescents (16 male, mean age 15 years) with HC and 31 sex‐ and aged‐matched controls. The following optical coherence tomography (OCT) parameters were obtained: macular retinal thickness (MRT) and volume (MRV), thickness of the macular and peripapillary retinal nerve fibre layer (ppRNFL), and area of the optic disc, cup and rim. Results: The MRT was thinner amongst those with HC compared with controls (right eye (RE) 262.2 ± 15.3 and 275.8 ± 15.1 μm; p = 0.0051), and the MRV was smaller (RE 7.37 ± 0.36 and 7.83 ± 0.35 mm3; p = 0.0002). The HC group showed a thinner ppRNFL (RE 88.3 ± 14.9 and 103.5 ± 8.1 μm; p = 0.0002) but a thicker central macular RNFL (RE 11.6 ± 10.4 and 2.07 ± 3.00 μm; p = <0.0001) and foveal minimum (RE 211.1 ± 32.0 and 186.3 ± 15.9 μm; p = 0.0013). Optic disc variables showed no difference between groups. Correlations were found in the HC group between best corrected visual acuity (expressed in logMAR) and ppRNFL (RE r = −0.56, p = 0.018), and disc area (RE r = −0.52, p = 0.033). Conclusion: Thinner ppRNFL and MRT and smaller MRV were found in adolescents with surgically treated HC in infancy compared with controls. In contrast, the central macular RNFL and foveal minimum were thicker. Further studies are required to evaluate the diagnostic value of OCT to indicate increased intracranial pressure timely and follow‐up in individuals with surgically treated HC. [ABSTRACT FROM AUTHOR]

Predominantly visual loss, is very rare in Wernicke's encephalopathy. A 22 year old lady, in her 28th week of gestation, presented with a confused mental state, bilateral papilloedema with retinal hemorrhages, ophthalmoparesis, and cerebellar signs. Her MRI brain was suggestive of Wernicke's encephalopathy and she recovered with intravenous thiamine [ABSTRACT FROM AUTHOR]

To study the etiology of papilloedema in north indian population. Our hospital based retrospective study enrolled 50 patients of papilloedema, who fullfilled our inclusion criteria. Demographic details, chief complains and detailed history was taken from all the patients. All the patients underwent blood pressure measurement by a mercury sphygmomanometer, Visual Acuity testing, Slitlamp Biomicroscopy, IOP measurement, Fundus examination. Fundus photographs were obtained using a fundus camera. Other investigations included B-scan, MRI, MRV, CT and required blood investigations were done wherever necessary. Although papilloedema occured in a vast variety of age group but maximum patients (32%) were from the age group of >18-31 years and males (64%) were more affected than females according to our study. 66% of the patients had good vision with papilloedema. In our study all the patients had bilateral papilloedema and 54% of patients were diagnosed with early papilloedema. Idiopathic Intracranial Hypertension was the main etiology for papilloedema. In this study we concluded IIH (a diagnosis of exclusion) as the most common underlying etiology for papilloedema. [ABSTRACT FROM AUTHOR]

Background: Idiopathic intracranial hypertension (IIH) remains a poorly understood condition with no standardized treatment. Treatment is therefore generally individualized. Recent isolated reports have begun to describe the use of third ventriculostomy (open or closed) for the treatment of IIH. This review aims to communicate the current evidence for the use of third ventriculostomy in IIH., Methods: A systemic review, using PubMed, was performed of studies describing the use of third ventriculostomy, either open or closed, for the treatment of idiopathic intracranial hypertension., Results: Only 3 studies for a total of 3 patients were found in which a third ventriculostomy was performed for the treatment of IIH., Conclusion: Despite very plausible proposed mechanisms of action, there is currently a paucity of both studies and, therefore, evidence for the use of either endoscopic or open third ventriculostomy for the treatment of IIH. The studies done to date do strongly suggest that further consideration is warranted., (Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.)

Abstract Predominantly visual loss, is very rare in Wernicke's encephalopathy. A 22 year old lady, in her 28th week of gestation, presented with a confused mental state, bilateral papilloedema with retinal hemorrhages, ophthalmoparesis, and cerebellar signs. Her MRI brain was suggestive of Wernicke's encephalopathy and she recovered with intravenous thiamine

We report a case of 51–year–old medically healthy male who presented with gradual painless diminution of vision in both eyes for 3 months. His visual acuity was hand movement perception in the right eye, and no light perception in the left eye. The intraocular pressure, external eye examination, ocular motility and anterior segment examinations were normal. Fundus examination revealed severe bilateral papilloedema and engorged tortuous veins in both eyes. Imaging exhibited a large intracranial tumour causing raised intracranial pressure. This was debulked by neurosurgery and histological examination revealed that the tumour was an olfactory neuroblastoma (ONB). This case is noteworthy since ONBs rarely present with isolated papilloedema without any accompanying ophthalmoplegia, proptosis, epistaxis, nasal, or neurological symptoms. [ABSTRACT FROM AUTHOR]

Objective: Obesity is a risk factor for idiopathic intracranial hypertension (IIH) and obstructive sleep apnoea (OSA). We aimed to determine the prevalence of OSA in IIH and evaluate the diagnostic performance of OSA screening tools in IIH. Additionally, we evaluated the relationship between weight loss, OSA and IIH over 12 months. Methods: A sub-study of a multi-centre, randomised controlled parallel group trial comparing the impact of bariatric surgery vs. community weight management intervention (CWI) on IIH-related outcomes over 12 months (IIH:WT). OSA was assessed using home-based polygraphy (ApneaLink Air, ResMed) at baseline and 12 months. OSA was defined as an apnoea–hypopnoea index (AHI) ≥ 15 or ≥ 5 with excessive daytime sleepiness (Epworth Sleepiness Scale ≥11). Results: Of the 66 women in the IIH: WT trial, 46 were included in the OSA sub-study. OSA prevalence was 47% (n = 19). The STOP-BANG had the highest sensitivity (84%) compared to the Epworth Sleepiness Scale (69%) and Berlin (68%) to detect OSA. Bariatric surgery resulted in greater reductions in AHI vs. CWI (median [95%CI] AHI reduction of – 2.8 [ – 11.9, 0.7], p = 0.017). Over 12 months there was a positive association between changes in papilloedema and AHI (r = 0.543, p = 0.045), despite adjustment for changes in the body mass index (R2 = 0.522, p = 0.017). Conclusion: OSA is common in IIH and the STOP-BANG questionnaire was the most sensitive screening tool. Bariatric surgery improved OSA in patients with IIH. The improvement in AHI was associated with improvement in papilloedema independent of weight loss. Whether OSA treatment has beneficial impact on papilloedema warrants further evaluation. Trial registration number: IIH: WT is registered as ISRCTN40152829 and on ClinicalTrials.gov as NCT02124486 (28/04/2014). [ABSTRACT FROM AUTHOR]

Investigating potential cerebrospinal fluid (CSF) shunt malfunction can be a challenge. Optical coherence tomography (OCT), a non-invasive imaging technique, is used to monitor changes at the optic nerve head in papilloedema. Conventional teaching suggests that in the presence of optic atrophy the optic nerve head may not re-swell in response to a relapse in raised intracranial pressure (ICP). A retrospective case series of three patients who had prior CSF diversion surgery for idiopathic intracranial cranial hypertension (IIH) is presented demonstrating the benefit of non-invasive OCT imaging confirming raised ICP. Recurrence of raised ICP, due to malfunctioning CSF shunt, was diagnosed in three patients requiring further surgical intervention. All re-presented acutely with headache and visual disturbances. All had a prior diagnosis of optic atrophy. In all patients, OCT peripapillary retinal nerve fibre layer qualitative image analysis and quantified progression analysis permitted easy detection of the recurrence of papilloedema. OCT imaging supports clinical decision making in shunt malfunction, even in the presence of established optic atrophy secondary to IIH. [ABSTRACT FROM AUTHOR]

A 30-year-old woman with idiopathic intracranial hypertension experienced worsening headaches and decreasing vision in her left eye. She underwent an uncomplicated ventriculoperitoneal shunt procedure but the following day was found to have cerebral venous sinus thrombosis. Treatment included venous sinus thrombectomy and anticoagulation. She had a favourable clinical outcome. Extensive evaluation including testing for thrombophilia was unremarkable. Potential causes for this rare association are discussed. [ABSTRACT FROM AUTHOR]

Background: Optic disc oedema (ODE) is an important manifestation in various ocular as well as systemic disorders. Measurement of retinal nerve fibre layer (RNFL) thickness in ODE patients may help in monitoring the progress of the disease and treatment response. Objective: To assess the clinical characteristics, aetiology and retinal nerve fibre layer (RNFL) imaging features of optic disc oedema (ODE) patients. Design: A retrospective observational study. Methods: This hospital-based study included performed for patients presenting with unilateral or bilateral ODE at the ophthalmology outpatient department of a tertiary institute between January 2019 and December 2019. Clinical features of all ODE patients were noted. RNFL thickness was measured by spectral-domain optical coherence tomography (Cirrus 500, Carl Zeiss Meditec, Dublin, CA, USA). Results: Sixty-four patients with ODE were seen with female preponderance (56.2%). The mean age of presentation was 36.8 ± 13.8 years. Bilateral ODE was seen in 57.8% patients. The most common cause of bilateral ODE was idiopathic intracranial hypertension (IIH) and vascular papillopathy. Retinal vein occlusion (RVO)-associated ODE was the most common unilateral presentation. Presenting visual acuity was best with IIH and worst with anterior ischaemic optic neuropathy (AION). The mean duration of symptoms was maximum with intracranial space-occupying lesion associated papilloedema and minimum with AION. The mean RNFL thickness was highest in uveitis-associated ODE, followed by compressive ON, and IIH. A positive correlation was noted between clinical ODE severity and RNFL thickness. All aetiological categories (except uveitic and compressive) had maximum RNFL thickening in the inferior quadrant and minimum in the temporal quadrant. Conclusion: The current study provides a clinical profile and RNFL measurements of ODE patients at this locality. The clinical severity of ODE correlated positively with RNFL thickness and most of the categories of ODE followed the normative pattern of RNFL thickness (inferior > superior > nasal > temporal) despite thickening. [ABSTRACT FROM AUTHOR]

Background: There is only one documented case of intracranial hypertension (IH) and empty sella from cortisol-producing adrenal adenoma so far. And IH and empty sella caused by long-term exogenous hypercortisolism has never been reported before. The purpose of this case report is to alert clinicians to glucocorticoid-induced IH. Case presentation: We present retrospectively a 50-year-old woman with cortisol-secreting adrenal adenoma, who progressed to intractable intracranial hypertension and a markedly expanded empty sella due to improper treatment. In 2011, the patient presented with hypertension, lack of cortisol circadian rhythm, low ACTH, a left adrenal adenoma and a partial empty sella, but did not receive low-dose dexamethasone suppression test (LDDST) and 24-h urinary cortisol. In 2014, she exhibited truncal obesity, raised cortisol, LDDST non-suppression, high urinary free cortisol and low ACTH, proving her cortisol-producing adrenal adenoma. She was simultaneously diagnosed with unexplained IH because of papilledema and elevated intracranial pressure, and her partial empty sella changed to a complete empty sella. In 2015, she underwent adrenal adenoma resection. From 2015 to 2018, she kept taking dexamethasone at least 2 mg daily without her doctors' consent. During this period, she developed transient cerebrospinal fluid rhinorrhea, and her empty sella further worsened. After switching to low dose hydrocortisone, her papilledema disappeared completely, but optic atrophy has become irreversible. Conclusions: The patient seems to be just an extreme case, but it may reveal and illustrate a general phenomenon: Both cortisol-producing adrenal adenoma and long-term exogenous hypercortisolism could cause varying degrees of elevated intracranial pressure and empty sella. Clinicians should remain vigilant for this phenomenon in patients with cortisol-producing adrenal adenoma or excessive and prolonged steroid usage and give them corresponding examinations to identify this complication. [ABSTRACT FROM AUTHOR]

Background: Idiopathic intracranial hypertension is an infrequent condition of childhood, and is extremely rare in infants, with only 26 cases described. The etiology is still unknown. Typical clinical manifestations change with age, and symptoms are atypical in infants, thus the diagnosis could be late. This is based on increased opening pressure at lumbar puncture, papilloedema and normal cerebral MRI. The measurement of cerebrospinal fluid opening pressure in infants is an issue because many factors may affect it, and data about normal values are scanty. The mainstay of treatment is acetazolamide, which allows to relieve symptoms and to avoid permanent visual loss if promptly administered. Case presentation: We report the case of an 8-month-old infant admitted because of vomit, loss of appetite and irritability; later, also bulging anterior fontanel was observed. Cerebral MRI and cerebrospinal fluid analysis resulted negative and after two lumbar punctures he experienced initial symptom relief. Once the diagnosis of idiopathic intracranial hypertension was made, he received oral acetazolamide, and corticosteroids, with progressive symptom resolution. Conclusions: Infantile idiopathic intracranial hypertension is extremely rare, and not well described yet. Bulging anterior fontanel in otherwise healthy infants with normal neuroimaging should be always considered suggestive, but can be a late sign, while irritability and anorexia, especially if associated with vomiting, may represent an early sign. In such cases, lumbar puncture should be always done, hopefully with cerebrospinal fluid opening pressure measurement, which is among coded diagnostic criteria, but whose threshold is controversial in infants. Early diagnosis, timely treatment and strict follow-up help to prevent vision loss or death of affected infants. [ABSTRACT FROM AUTHOR]

A 60-year-old woman noted headaches, transient visual obscurations and pulsatile tinnitus. She had Frisén grade 4 papilloedema. Brain magnetic resonance imaging showed a large subfrontal cystic mass with ring enhancement. Tumour pathology was in-keeping with an intraparenchymal schwannoma. This uniquely rare tumour is discussed. [ABSTRACT FROM AUTHOR]

Purpose: To report the outcome of optic nerve sheath decompression (ONSD) for papilloedema in a teaching hospital in western Turkey. Methods: The charts of 56 patients who had ONSD surgery between April 2007 and September 2019 were collated; and a total of 81 operated and 31 fellow eyes were included. Pre‐ and postoperative ophthalmologic examination including best‐corrected visual acuity (BCVA), colour vision (CV), visual field (VF) analysis, fundoscopic examination and demographic and medical characteristics of the patients were noted and outcomes after surgery were investigated. Results: Of all study eyes, 49 (43.7%) eyes had BCVA 0.2 or less and 62 (55.3%) eyes had mean deviation (MD) below – 20.0 dB. 62 (55.3%) eyes had Frisen grade 4 or 5 papilloedema. Almost half of the eyes had severe vision loss. After ONSD, BCVA, CV and MD in both operated and fellow non‐operated eyes improved significantly (p < 0.001, p = 0.009 and p < 0.001 for operated, p < 0.001, p = 0.007 and p < 0.001 for fellow eyes, respectively). Earlier surgery and higher cerebrospinal fluid opening pressure were related to better outcomes. None of the patients had major operative complications. Conclusion: Optic nerve sheath decompression can safely improve vision not only of the operated but also of the non‐operated eye, even in cases with severe vision loss from severe bilateral papilloedema. Regardless of initial VA and VF, patients may benefit from ONSD; the earlier it is done the more likely the better outcome. [ABSTRACT FROM AUTHOR]

Abstract There is increasing evidence and appreciation of idiopathic intracranial hypertension (IIH) in medicine. The pathological processes underlying raised intracranial pressure are being studied, with new insights found in both hormonal dysregulation and the metabolic neuroendocrine axis. These will potentially lead to novel therapeutic targets for IIH. The first consensus guidelines have been published on the investigation and management of adult IIH, and the International Headache Society criteria for headache attributable to IIH have been modified to reflect our evolving understanding of IIH. Randomized clinical trials have been published, and a number of studies in this disease area are ongoing.

Abstract Background Headache is the dominant factor for quality of life related disability in idiopathic intracranial hypertension (IIH) and typically has migraine-like characteristics. There are currently no evidence-based therapeutics for headache in IIH, and consequently this is an important unmet clinical need. Case series We report a series of seven patients in whom headaches were the presenting feature of IIH and the headaches had migraine-like characteristics, as is typical in many IIH patients. Papilloedema settled (ocular remission) but headaches continued. These headaches responded markedly to erenumab, a monoclonal antibody targeted against the calcitonin gene related peptide (CGRP) receptor. Of note, there was a recurrence of raised ICP, as evidenced by a return of the papilloedema, however the headaches did not recur whilst treated with erenumab. Conclusions Those with prior IIH who have their headaches successfully treated with CGRP therapy, should remain under close ocular surveillance (particularly when weight gain is evident) as papilloedema can re-occur in the absence of headache. These cases may suggest that CGRP could be a mechanistic driver for headache in patients with active IIH.

Sam P Gurney, 1 Sateesh Ramalingam, 2 Alan Thomas, 2 Alex J Sinclair, 3–5 Susan P Mollan 1 1Birmingham Neuro-Ophthalmology, Queen Elizabeth Hospital, University Hospitals Birmingham, Birmingham B15 2WB, UK; 2Neuroradiology Department, Queen Elizabeth Hospital, University Hospitals Birmingham, Birmingham B15 2WB, UK; 3Metabolic Neurology, Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham B15 2TT, UK; 4Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham B15 2TH, UK; 5Department of Neurology, University Hospitals Birmingham NHS Foundation Trust, Queen Elizabeth Hospital, Birmingham B15 2WB, UKCorrespondence: Susan P MollanBirmingham Neuro-Ophthalmology, Queen Elizabeth Hospital, University Hospitals Birmingham, Birmingham B15 2WB, UKTel +44 1213716912 Email susan.mollan@uhb.nhs.ukAbstract: Idiopathic Intracranial Hypertension (IIH) is a debilitating disorder characterised by raised intracranial pressure (ICP), papilloedema with the potential risk of permanent visual loss, and headaches that are profoundly disabling and reduce the quality of life. The first consensus guidelines have been published on investigation and management of adult IIH and one key area of uncertainty is the utility of dural venous sinus stenting for the management of headache and visual loss. There are an increasing number of series published and to help understand the successes and complications. During a patient physician priority setting, the understanding of the best type of intervention to treat IIH was assigned to the top 10 of most desired research questions for the disease. Ultimately randomised clinical trials (RCTs) in neurovascular stenting for IIH would be instructive, as the literature to date may suffer from publication bias. Due to the increasing incidence of IIH, there is no better time to systematically investigate interventions that may reverse the disease process and achieve remission. In this review we discuss the pathophysiology of IIH in relation to venous sinus stenosis, the role of venous sinus stenting with a review of the relevant literature, the advantages and disadvantages of stenting compared with other surgical interventions, and the future of stenting in the treatment of IIH.Keywords: pseudotumor cerebri, raised intracranial pressure, venous sinus stenosis, neurovascular stenting, headache, papilloedema

Introduction: Cerebral venous thrombosis (CVT) is an uncommon cause of stroke that mainly affects young adults. Early, accurate diagnosis can reduce the rate and severity of complications., Objective: The aim of this study was to analyse the clinical characteristics, management, and treatment of CVT in different centres in Spain., Methods: We conducted a multicentre, retrospective, descriptive study of patients hospitalised due to CVT between 2008 and 2017 at 10 Spanish centres., Results: We included 256 patients, with a mean age (SD) of 49.8 (18.7) years; 51% of patients were women. The most frequent symptoms were headache (73%), focal deficits (50%), epileptic seizures (33%), and encephalopathy (21%). The most frequent localisations were the superior sagittal sinus (12.5%), the transverse sinus (10.9%), and 2 or more sinuses or veins (66.4%). Thrombophilia was the most frequent known aetiology (24%), and was most commonly associated with the prothrombin G20210A mutation (19%). Forty-six percent of patients were treated with antithrombotics for 3 to 6 months, 21% for one year, and 22.6% required indefinite anticoagulation. Endovascular therapy was performed in 5% of cases, and 33% required neurosurgery. Regarding outcomes, 75% of patients were independent at 3 months (modified Rankin Scale [mRS] score ≤ 2), with papilloedema (P =  .03), focal deficits (P = .001), and encephalopathy (P < .001) showing a statistically significant association with poor prognosis (mRS > 3). The in-hospital mortality rate was 4.3%, with a 3-month mortality rate of 6.3%., Conclusion: The diverse risk factors and variable presentation of CVT represent a challenge in the diagnosis and treatment of this condition. To improve prognosis and reduce mortality, it is essential to establish management protocols for this entity., (Copyright © 2021 Sociedad Española de Neurología. Published by Elsevier España, S.L.U. All rights reserved.)

Idiopathic intracranial hypertension is a neurological syndrome determined by a rise in intracranial pressure without a detectable cause. Course and prognosis may be changeable, requiring a multidisciplinary approach for its diagnosis and management. Although its precise pathogenesis is still unknown, many studies have been carried out to define the possible causal and associated factors, such as retinoids, steroid hormones, body mass index and recent weight gains, cytokines and adipokines levels. The clinical presentation can be variable including chronic headache, disturbance of vision, diplopia and tinnitus. Even if papilloedema is considered the most specific sign, it could not be observed in more than 5% of patients during the evaluation of the fundus oculi. Neuroradiological signs acquire greater importance in patients who do not present papilloedema and may suggest the diagnosis of idiopathic intracranial hypertension. Other assessments can be useful in the diagnostic process, such as optical coherence tomography, visual evoked potentials, ocular ultrasonography and fundus fluorescein angiography and autofluorescence. Nonetheless, cerebrospinal fluid pressure measurement is required to establish a definite diagnosis. Management may be different, since surgical procedures or lumbar punctures are often required when symptoms develop rapidly leading to a loss of visual function. Apart from these cases, patients can be treated with a pharmacological approach and low-calorie diet, but they also need to be monitored over time since relapses years later are not uncommon. [ABSTRACT FROM AUTHOR]

Background: Diplopia is the double vision of a single object, and can be binocular or monocular. Binocular diplopia is caused by the misalignment of the visual axes, with images falling on the fovea of the fixating eye and on the extra-foveal retina of the non-fixating eye, as a consequence of both neurological (i.e., oculomotor nerve palsies, ocular myopathies, neuromuscular junction disorders) and ophthalmic disorders (i.e., decompensation of a pre-existing strabismus). In contrast, monocular diplopia is generally explained by intraocular pathology (i.e., refractive errors, ocular media abnormalities, dry eyes), causing the image of a single object to fall, at the same time, on the fovea and on the extra-foveal retina of the same eye. Methods: We report the case of a 22-year-old woman presenting with acute-onset monocular diplopia. Results: The diagnosis of idiopathic intracranial hypertension (IIH) was based on the presence of papilloedema and elevated cerebrospinal fluid (CSF) pressure. Monocular diplopia resolved after CSF subtraction. Conclusions: We describe a case of monocular diplopia as a presenting symptom of IIH, and discuss diagnostic issues of this possibly underestimated symptom in neurology clinical practice. Careful ophthalmic and neuro-ophthalmic examination can identify clinical features of diplopia, and drive diagnosis and treatment.

Syphilis produces myriad nonspecific signs and symptoms. For example, optic disk swelling might be seen in patients with syphilis as a result of cranial hypertension (papilloedema), inflammatory optic neuritis with papillitis, or optic perineuritis. We report a case involving differential diagnosis of syphilitic bilateral papillitis mimicking papilloedema.

Aim: The aim of the study was a long-term observation (particularly in terms of visual function) of patients hospitalised at the Neurology Department of St. Adalbert Hospital in Gdańsk, Poland, due to an episode of idiopathic intracranial hypertension. Material and methods: The Department’s archives held records of 13 cases of idiopathic intracranial hypertension treated from 2007 to 2015. The retrospectively retrieved data concerned: age, sex, height, weight, comborbidities, symptoms of idiopathic intracranial hypertension, neurological abnormalities, fundoscopy findings, vision field, brain magnetic resonance findings, visual evoked potentials and employed treatment. Eleven patients reported for a follow-up examination, and 6 patients completed it. The patients were examined neurologically and ophthalmologically with fundoscopy, visual field test, visual acuity and visual evoked potentials. The results were compared with the disease history data. The follow-up period lasted 4–47 months. Results: Nine of 11 patients were females. In 2 of them, the initial diagnosis was revised, and 3 did not complete all procedures. Six patients were included in the analysis. The most common complain upon admission was headache and tunnel vision. Brain magnetic resonance imaging scans revealed changes characteristic of idiopathic intracranial hypertension in 5 patients. Therapeutic drainage of cerebrospinal fluid was performed in all the patients, and all of them were treated with acetazolamide. Visual disturbances (visual field loss or impaired visual evoked potentials) persisted in 3 patients. Significant risk factors for idiopathic intracranial hypertension in the analysed group were: female sex, increased body mass index and reproductive age. Permanent vision impairment was observed in patients who required more intensive treatment. Conclusion: Weight gain is a significant risk factor for poor outcome. Visual field assessment and fundoscopy seem to be superior than visual acuity in the monitoring of disease activity.