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Introduction
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Publications
Publications (262)
Purpose of Review
Development of safe targeted therapies for idiopathic intracranial hypertension requires a thorough understanding of recent evidence discovering the pathophysiology of the condition. The aim is to provide a review of studies that inform on the underpinning mechanisms that have been associated with idiopathic intracranial hypertens...
OBJECTIVE
This article describes the recognition, investigation, and management of papilledema.
LATEST DEVELOPMENTS
Papilledema describes optic disc swelling (usually bilateral) arising from raised intracranial pressure. Due to the serious nature of papilledema, there is a fear of underdiagnosis and hence one of the major stumbling points is the c...
There is unmet clinical need for biomarkers to predict recovery or the development of long-term sequelae of mild traumatic brain injury, a highly prevalent condition causing a constellation of disabling symptoms. A substantial proportion of patients live with long-lasting sequelae affecting their quality of life and ability to work. At present, sym...
There is unmet clinical need for biomarkers to predict recovery or the development of long-term sequelae of mild traumatic brain injury, a highly prevalent condition causing a constellation of disabling symptoms. A substantial proportion of patients live with long-lasting sequelae affecting their quality of life and ability to work. At present, sym...
Objective
To gain initial insight into the efficacy to lower intracranial pressure (ICP), side effects, and effects on cognition of five drugs commonly used to treat idiopathic intracranial hypertension (IIH).
Background
Limited clinical data exist for the treatment for IIH. Impaired cognition is recognized in IIH and can be exacerbated by medicat...
Background/objectives
Idiopathic intracranial hypertension (IIH) is a disease which threatens vision and causes disabling headaches, affecting women of childbearing age with obesity. It is characterised by raised intracranial pressure (ICP), measured invasively either with lumbar punctures or intracranially-inserted monitors. There is an unmet clin...
This is a practical resource dealing with the diagnosis and management of common and rare headache types. It is edited by a general practitioner and an academic neurologist and will be of value to all those who work in headache at all clinical levels. The book is published under the auspices of the British Association for the Study of Headache (BAS...
Question
What is the effectiveness of bariatric surgery beyond 5 years in people living with Idiopathic Intracranial Hypertension (IIH)?
Methods
The IIH Weight Trial enrolled women with active IIH and a body mass index (BMI) ≥35kg/m² between March 1, 2014, and May 25, 2017, for randomization to either bariatric surgery or community weight manageme...
This review article represents a collaborative effort across continents, bringing together the latest developments in neuro-ophthalmology with a focus on innovative diagnostic and therapeutic modalities that are shaping the future of the field. Among the most significant advancements is the rise of optical coherence tomography (OCT), now recognized...
Objectives
This project aimed to determine whether cranial ischaemic complications at the presentation of giant cell arteritis (GCA) were associated with pre-existing cardiovascular (CV) risk factors, CV disease or genetic risk of CV-related traits.
Methods
1946 GCA patients with clinicodemographic data at GCA presentation were included. Associati...
The occurrence of Leber Hereditary Optic Neuropathy in association with dystonia is exceedingly rare. There have been only a few previously reported cases describing this clinical phenotype with the mitochondrial DNA 14459 G>A/ND6 mutation. This mutation has been described to also manifest as isolated Leber Hereditary Optic Neuropathy or Leigh Synd...
Introduction
Mild traumatic brain injury (TBI) affects a significant number of military personnel, primarily because of physical impact, vehicle incidents, and blast exposure. Post-traumatic headache (PTH) is the most common symptom reported following mild TBI and can persist for several years. However, the current International Classification of H...
Introduction:
Idiopathic intracranial hypertension is a neurological condition characterized by a raised intracranial pressure and papilledema, leading to chronic headaches and visual disturbances. By recognizing modifiable risk factors and deploying evidence-based interventions, healthcare providers have the potential to mitigate the burden of th...
Idiopathic intracranial hypertension (IIH) is a neuro-ophthalmological condition characterised by a raised intracranial pressure and papilloedema that causes disabling headaches. The main risk factors of female sex and living with obesity have been known for some time, however the knowledge of the underlying pathophysiology is evolving. Papilloedem...
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 prospectiv...
Most cases of optic neuritis (ON) occur in women and in patients between the ages of 15 and 45 years, which represents a key demographic of individuals who seek health information using the internet. As clinical providers strive to ensure patients have accessible information to understand their condition, assessing the standard of online resources...
The European Neuro-Ophthalmology Society (EUNOS) practical neuro-ophthalmology course, henceforth referred to as the EUNOS course, took place in 2023 in Groningen, the Netherlands. This two-day international event featured a blend of plenary lecture sessions, workshops, and case reports, providing a practical update on fundamental neuro-ophthalmolo...
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...
Headache in intracranial disorders drives significant disability in our patients. Management principles for secondary headaches require a multidisciplinary approach with expertise from the neurological, neuro-ophthalmological, neuroradiological, and neurosurgical specialities. The Pressure of Headache was the theme of the annual United Kingdom CSF...
Mild traumatic brain injury (mTBI) is common with many patients suffering disabling long-term sequelae, with visual symptoms frequently reported. There are no objective biomarkers of mTBI that are routinely used in clinical practice. Optical coherence tomography (OCT) has been used in mTBI research, as it enables visualisation of the neuroretina, a...
Background
Cognitive function can be affected in conditions with raised intracranial pressure (ICP) such as idiopathic intracranial hypertension (IIH). Drugs used off label to treat raised ICP also have cognitive side effects, underscoring the unmet need for effective therapeutics which reduce ICP without worsening cognition. The Glucagon Like Pept...
Background
Idiopathic intracranial hypertension (IIH) is a neurometabolic condition severely impacting the quality of life of people living with IIH (PwIIH). Most PwIIH are overweight or live with obesity, and weight loss is recommended by healthcare professionals (HCPs) as it is central to disease management. There is currently no research evaluat...
Introduction:
Idiopathic intracranial hypertension is a neurological condition characterized by a raised intracranial pressure and papilledema that causes debilitating headaches. While the extent of the pathophysiology is being discovered, the condition is emerging as a systemic metabolic disease distinct to people living with obesity alone. Idiop...
Idiopathic intracranial hypertension (IIH) is a disease characterised by elevated intracranial pressure (ICP). The impact of straining and exercise on ICP regulation is poorly understood yet clinically relevant to IIH patient care. We sought to investigate the impact of Valsalva manoeuvres (VMs) and exercise on ICP and cerebrovascular haemodynamics...
The understanding of idiopathic intracranial hypertension (IIH) has evolved over the past few years. Previously, IIH was considered a disease exclusively affecting the neuro-ophthalmic axis, characterized by raised intracranial pressure, headache and papilloedema, and resulting in the risk of severe and permanent visual loss and life-changing disab...
Introduction
Cerebral venous sinus thrombosis (CVST) is a rare and fatal disorder in pregnancy. Antico- agulation with heparin or low molecular weight heparin (LMWH) is the recommended treatment. We report the case of a pregnant woman with CVST and progressive visual loss despite anticoagulation. Her symptoms resolved following delivery and lumbar...
Idiopathic intracranial hypertension, a disease classically occurring in women with obesity, is characterized by raised intracranial pressure. Weight loss leads to the reduction in intracranial pressure. Additionally, pharmacological glucagon-like peptide-1 agonism reduces cerebrospinal fluid secretion and intracranial pressure. The potential mecha...
Background and Objectives
Idiopathic intracranial hypertension (IIH) is associated with obesity, however there is a lack of clinical consensus on how to manage weight in IIH. The aim of this systematic review is to evaluate weight loss interventions in people with IIH to determine which intervention is superior in terms of weight loss, reduction in...
Cerebrospinal fluid disorders have a wide-ranging impact on vision, headache, cognition and a person’s quality of life. Due to advances in technology and accessibility, intracranial pressure measurement and monitoring, usually managed by neurosurgeons, are being employed more widely in clinical practice. These developments are of direct importance...
Susac syndrome is a likely autoimmune microangiopathy affecting the brain, retina and inner ear. Due to the rarity of this condition, diagnosis and treatment can be challenging. Diagnosis is based on the presence of the clinical triad of central nervous system dysfunction, branch retinal artery occlusions and sensorineural hearing loss. Typical MRI...
Purpose
Retinal vein occlusion (RVO) is the second leading cause of visual loss due to retinal disease. Retinal vein occlusion increases the risk of cardiovascular mortality and the risk of stroke. This article describes the data contained within the INSIGHT eye health data set for RVO and cardiovascular disease.
Design
Data set descriptor for rou...
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 I...
Background:
There are an increasing number of controlled clinical trials and prospective studies, ongoing and recently completed, regarding management options for idiopathic intracranial hypertension (IIH). We present a Common Design and Data Element (CDDE) analysis of controlled and prospective IIH studies with the aim of aligning essential desig...
Background
The treat-to-target (T2T) concept is not yet a recognized treatment approach in giant cell arteritis (GCA) and polymyalgia rheumatica (PMR). Developing such recommendations is an unmet medical need.
Objectives
To determine therapeutic targets and develop treat-to-target (T2T) recommendations in GCA and PMR.
Methods
We conducted a syste...
Background:
Little is known about the presentation and prognosis of asymptomatic idiopathic intracranial hypertension (IIH). Papilloedema can be found incidentally on routine fundus examination, with many of these patients actually having symptoms on direct questioning. The aim was to evaluate visual and headache outcomes in people with IIH who pr...
Introduction:
Idiopathic intracranial hypertension (IIH) and polycystic ovary syndrome (PCOS) are hyperandrogenic metabolic disorders that affect women of reproductive age living with obesity. The previously reported prevalence of comorbid PCOS in IIH patients is highly variable and the longitudinal impact on visual and headache outcomes are unkno...
Purpose:
This study was designed to determine if point analysis of the Humphrey visual field (HVF) is an effective outcome measure for people with idiopathic intracranial hypertension (IIH) compared with mean deviation (MD).
Methods:
Using the IIH Weight Trial data, we performed a pointwise analysis of the numerical retinal sensitivity. We then...
Introduction:
Idiopathic intracranial hypertension is characterized by raised intracranial pressure that triggers disabling headaches and can cause permanent visual loss. There is an increased incidence and prevalence of the condition linked to location-specific obesity rates. There are no licensed treatments for the condition. The majority of app...
A significant role of the neuro-ophthalmologist is to counsel patients on appropriate management and anticipated visual prognosis for conditions affecting the afferent and efferent visual systems, including those requiring neurosurgical treatment. However, the literature regarding anticipated neuro- ophthalmologic prognosis after neurosurgical inte...
Background
The M.scio telesensor (Aesculap-Miethke, Germany) is a device integrated within a ventriculoperitoneal (VP) shunt for non-invasive measurement of the intracranial pressure (ICP). The purpose of this study was to analyze the telemetric recordings with the M.scio system in shunted patients with idiopathic intracranial hypertension (IIH), i...
Therapeutics to reduce intracranial pressure are an unmet need. Preclinical data have demonstrated a novel strategy to lower intracranial pressure using glucagon-like peptide-1 (GLP-1) receptor signalling.
Here, we translate these findings into patients by conducting a randomized, placebo-controlled, double-blind trial to assess the effect of exena...
Optic neuritis (ON) often occurs at the presentation of multiple sclerosis (MS), neuromyelitis optica spectrum disorders (NMOSD), and myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD). The recommended treatment of high-dose corticosteroids for ON is based on a North American study population, which did not address treatm...
Objectives
To develop treat-to-target (T2T) recommendations in giant cell arteritis (GCA) and polymyalgia rheumatica (PMR).
Methods
A systematic literature review was conducted to retrieve data on treatment targets and outcomes in GCA/PMR as well as to identify the evidence for the effectiveness of a T2T-based management approach in these diseases...
Objective:
To evaluate mental health burden in women with idiopathic intracranial hypertension (IIH) compared to matched women with migraine and population controls.
Background:
Depression and anxiety are recognized comorbid conditions in those with IIH and lead to worse predicted medical outcomes. The mental health burden in IIH has not been pr...
Background
Idiopathic intracranial hypertension (IIH) most typically occurs in women of childbearing age with increased weight as a key risk factor for development or exacerbation of the disease. Pregnancy is common in this group of patients. The longer-term impact of pregnancy on IIH has not been established and was the aim of this study.
Methods...
Giant Cell Arteritis (GCA) is well known to be a critical ischaemic disease that requires immediate medical recognition to initiate treatment and where one in five people still suffer visual loss. The immunopathophysiology has continued to be characterised, and the influencing of ageing in the development of GCA is beginning to be understood. Recen...
Purpose:
Diabetic retinopathy (DR) is the most common microvascular complication associated with diabetes mellitus (DM), affecting approximately 40% of this patient population. Early detection of DR is vital to ensure monitoring of disease progression and prompt sight saving treatments as required. This article describes the data contained within...
Objective
GCA is the commonest primary systemic vasculitis in adults, with significant health economic costs and societal burden. There is wide variation in access to secondary care GCA services, with 34% of hospitals in England not having any formal clinical pathway. Quality standards provide levers for change to improve services.
Methods
The mul...
Purpose:
To characterise the phenotype of idiopathic intracranial hypertension (IIH) patients who received cerebrospinal (CSF) diversion surgery and detail the trajectory of recovery.
Design:
Prospective cohort registry study METHODS: IIH patients with sight threatening papilloedema presenting to a single United Kingdom neuroscience centre betwe...
The link between polycystic ovarian syndrome (PCOS) and idiopathic intracranial hypertension (IIH) has long been debated. Historically, there is a wide range of reported occurrence of both conditions, being between 15% and 64%. Both conditions share a common phenotype. The awareness that in a new large data study that there is a 1.5-fold increased...
Background: Idiopathic intracranial hypertension (IIH) is characterized by increased intracranial pressure occurring predominantly in women with obesity. The pathogenesis is not understood. We have applied untargeted metabolomic analysis using ultrahigh-performance liquid chromatography-mass spectrometry to characterize the cerebrospinal fluid (CSF...
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...
Background
Multiple lumbar punctures have historically been a strategy to relieve headaches associated with idiopathic intracranial hypertension despite limited clinical evidence of long-term efficacy. Lumbar puncture is typically a straightforward procedure with minimal complications reported, however, serious complications can occur. Lumbar-punct...
This case report describes the optic nerve features of a male patient aged 23 years with a diagnosis of autosomal recessive spastic ataxia of Charlevoix-Saguenay.
Objectives
Intracranial pressure (ICP) has been thought to vary diurnally. This study evaluates diurnal ICP measurements and quantifies changes in ICP occurring with changes in body posture in active idiopathic intracranial hypertension (IIH).
Methods
This prospective observational study utilized telemetric ICP monitoring in people with active IIH...
Background
There are limited longitudinal data evaluating outcomes in idiopathic intracranial hypertension (IIH). We aimed to evaluate the long-term outcomes in a real-world cohort of patients with IIH and sought to establish the prognostic factors.
Methods
A longitudinal prospective cohort study was conducted over 9 years (2012–2021). Data includ...
The annual United Kingdom cerebrospinal fluid disorders meeting made its return to the University of Birmingham, United Kingdom, following the break in proceedings due to the coronavirus pandemic. Cerebrospinal fluid disorders require a multi-disciplinary approach with inputs from the neurological, neuro-ophthalmological, neuroradiological, and neu...
Background
Migraine is a highly prevalent disorder with significant economical and personal burden. Despite the development of effective therapeutics, the causes which precipitate migraine attacks remain elusive. Clinical studies have highlighted altered metabolic flux and mitochondrial function in patients. In vivo animal experiments can allude to...
‘EUNOS 2022’ marked the 15th biennial congress of the European Neuro-ophthalmology Society. It was held at the University of Birmingham, England between Monday 20 June and Thursday 23 June 2022. This report summarises the highlights of the Neuro-ophthalmology programme of the congress, including the teaching day, scientific symposia and debates.
Background and Objective
We evaluated the metabolomic profile in CSF, serum and urine of participants with idiopathic intracranial hypertension (IIH) compared to controls and measured changes in metabolism associated with clinical markers of disease activity and treatment.
Methods
A case-control study compared women aged 18-55 years with active II...
Therapeutics to reduce intracranial pressure are an unmet need. Pre-clinical data has demonstrated a novel strategy to lower intracranial pressure using glucagon-like peptide-1 receptor signaling.
Here, we translate these findings into patients by conducting a randomized, placebo controlled, double-blind trial to assess the effect of exenatide, a g...
Introduction
There is limited longitudinal data evaluating visual and headache outcomes in Idiopathic intracranial hypertension and large prospective studies are required.
Aims and Methods
To evaluate the disease course for non-surgical IIH patients, determined by their pres- entation to neuro-ophthalmology with active IIH (papilloedema present) ve...
Introduction
Idiopathic intracranial hypertension (IIH) and Polycystic ovary syndrome (PCOS) affect women of reproductive age with obesity but have different hyperandrogenic profiles. The prevalence of comorbid PCOS in IIH patients is highly variable in the literature; and the longitudinal impact on visual and headache outcomes are unknown.
Aims a...
We present a 50-year-old lady with 6-months’ history of episodes of feeling weak with legs giving way triggered by sudden change of position or head-turning and no loss of consciousness. She had left tinnitus for years and one year worsening migraines with fortification spectra, visual blurring, and vertigo. ENT made a diagnosis of vestibular migra...
We aimed to evaluate adjusted hazard ratio (aHR) for new-onset headache as well as headache medi- cation prescribing, opiate use and headache burden in women with idiopathic intracranial hypertension (IIH) compared to matched women with migraine and population controls.
Data from IQVIA Medical Research Data, an anonymised, nationally representative...
Incidence rates for depression and anxiety has not been previously evaluated in a large matched cohort study in idiopathic intracranial hypertension (IIH).
We analysed data collected between January-1995 and September-2019 from IQVIA Medical Research Data, an anonymised and nationally representative UK database. Crude incident rates for depression...
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.
A sub-study of a multi-cent...
Introduction
IIH is a known metabolic neuro-ophthalmologic disorder which is associated with body weight gain. A common reason for increasing weight in child-bearing aged women is pregnancy. The long-term impact of IIH diagnosed during pregnancy is not known.
Aims and Methods
Evaluate key outcomes such as visual acuity, visual fields, optical cohe...
Introduction
Idiopathic intracranial hypertension (IIH) predominantly affects women of reproductive age with obesity with a distinct profile of hyperandrogenism and insulin resistance. Polycystic ovary syndrome (PCOS) has an established adverse fertility phenotype that typically affects obese women. IIH may impact reproductive health.
Methods
Pros...
Lumbar puncture (LP) is a common diagnostic procedure in clinical neurology. Iatrogenic spinal haema- tomas can be a complication of LPs and range from mild and self-resolving to very severe or even fatal. We describe a case report and review current literature on post-LPs spinal haematomas.
A 28-year-old Lady with a background of post-meningitic h...
Purpose of review:
In this review, we consider the challenges of creating a trusted resource for real-world data in ophthalmology, based on our experience of establishing INSIGHT, the UK's Health Data Research Hub for Eye Health and Oculomics.
Recent findings:
The INSIGHT Health Data Research Hub maximizes the benefits and impact of historical,...
Background
Insertion of Cerebrospinal fluid (CSF) shunts in patients with Idiopathic Intracranial Hypertension (IIH) is challenging mainly due to the small ventricles and the phenotypical body habitus. In this report the authors present their surgical protocol for insertion of ventriculoperitoneal shunt (VPS) in patients with IIH and the associated...
Background and objectives
Physician prescribing habits for opiates and headache therapies have not been previously evaluated in a large, matched cohort study in idiopathic intracranial hypertension (IIH). Our objective was to evaluate opiate and headache medication prescribing habits in women with IIH compared to matched women with migraine and pop...
Background and objectives:
The idiopathic intracranial hypertension randomized controlled weight trial (IIH:WT) established that weight loss through bariatric surgery significantly reduced intracranial pressure compared to a community weight management intervention. This sub-study aimed to evaluate the amount of weight loss required to reduce intr...
Objective:
Idiopathic intracranial hypertension (IIH) predominantly affects women of reproductive age with obesity and 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, w...
Purpose
Using the Idiopathic Intracranial Hypertension Weight Trial (IIH:WT) data, this study aimed to determine if point analysis of the Humphrey visual field (HVF) could be more informative than the perimetric mean deviation (PMD) as an IIH trial outcome measure.
Methods
IIH:WT was a randomized controlled trial that recruited 66 people with acti...
Background
Enforced national lockdown due to COVID-19 limited access to medical services. We evaluated the impact on those presenting with papilloedema, and those with a pre-existing diagnosis of idiopathic intracranial hypertension.
Design
A single United Kingdom centre prospective cohort study between May 15, 2020 (start of emergency clinics aft...
Aim
To determine whether Optical Coherence Tomography (OCT) of the optic nerve head in papil- loedema could act as a surrogate measure of intracranial pressure (ICP).
Methods
This is a longitudinal cohort study using data collected from three randomised controlled trials, between from April 1st, 2014 to August, 1st 2019. OCT imaging and automated...
Background
Pre-clinical data demonstrates the ability of Exenatide, a GLP-1R agonist, to reduce CSF secretion and ICP.1 Existing GLP-1R agonists are widely used to treat obesity and diabetes (but do not cause hypoglycaemia).
Methods
Randomised, placebo controlled, double-blind trial of Exenatide in women with active IIH. Participants were randomis...
Headache therapy is an unmet need in idiopathic intracranial hypertension (IIH).
This was a prospective, real world, open-label cohort study over 9 months in the UK. Adult females with confirmed diagnosis of IIH now in ocular remission with chronic migraine (CM)-like headaches and failure of ≥3 preventative medications received Erenumab 70mg monthl...
Objective
Headache is the predominant disabler in idiopathic intracranial hypertension (IIH). The aim was to characterise headache and investigate the association with intracranial pressure (ICP).
Methods
IIH:WT was a randomised controlled parallel group multicentre trial investigating weight manage- ment methods in IIH. Participants with active I...
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 p...
Objective
The IIH weight trial (IIH: WT) aimed to compare the efficacy of bariatric surgery with a community weight management intervention (CWI) in active IIH.
Methods
This was a five-year randomized control trial which enrolled participants between March 1, 2014 and May 25, 2017 at five NHS hospitals in the United Kingdom. Participants with acti...
Idiopathic intracranial hypertension (IIH) is an increasingly prevalent disease, characterised by raised intracranial pressure (ICP). Cognitive impairments have been reported in IIH, however evidence support- ing these deficits are scarce and contributing factors have not been defined. Using a bespoke battery of cognitive tests, we identified multi...