Idiopathic Intracranial Hypertension

14,000,000 Leading Edge Experts on the ideXlab platform

Scan Science and Technology

Contact Leading Edge Experts & Companies

Scan Science and Technology

Contact Leading Edge Experts & Companies

The Experts below are selected from a list of 3105 Experts worldwide ranked by ideXlab platform

Alexandra J Sinclair - One of the best experts on this subject based on the ideXlab platform.

  • Reviewing the Recent Developments in Idiopathic Intracranial Hypertension
    Ophthalmology and Therapy, 2020
    Co-Authors: Jasvir Virdee, Alexandra J Sinclair, Stephanie Larcombe, Vivek Vijay, Margaret Dayan, Susan P Mollan
    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.

  • Emerging themes in Idiopathic Intracranial Hypertension
    Journal of Neurology, 2020
    Co-Authors: Olivia Grech, Susan P Mollan, Benjamin R. Wakerley, Zerin Alimajstorovic, Gareth G. Lavery, Alexandra J Sinclair
    Abstract:

    Purpose Idiopathic Intracranial Hypertension (IIH) is a rare disorder characterised by raised Intracranial pressure. The underlying pathophysiology is mostly unknown and effective treatment is an unmet clinical need in this disease. This review evaluates key emerging themes regarding disease characteristics, mechanisms contributing to raised Intracranial pressure and advances in potential therapeutic targets. Findings IIH is becoming more common, with the incidence rising in parallel with the global obesity epidemic. Current medical management remains centred around weight management, which is challenging. Metabolic investigations of patients have identified specific androgen profiles in cerebrospinal fluid (CSF), which suggest an endocrine dysfunction impacting CSF secretion in IIH. Glucagon-like peptide-1 (GLP-1) and 11β-hydroxysteroid dehydrogenase type 1 (11β-HSD1) have been found to play a role in CSF dynamics in IIH and have formed the basis of the first clinical trials looking at new treatments. Conclusions Identification of novel molecular targets thought to underlie IIH pathology is now being translated to clinical trials.

  • the expanding burden of Idiopathic Intracranial Hypertension
    Eye, 2019
    Co-Authors: Susan P Mollan, Alexandra J Sinclair, Magda Aguiar, Felicity Evison, Emma Frew
    Abstract:

    To quantify the hospital burden and health economic impact of Idiopathic Intracranial Hypertension. Hospital Episode Statistics (HES) national data was extracted between 1st January 2002 and 31st December 2016. All those within England with a diagnosis of Idiopathic Intracranial Hypertension were included. Those with secondary causes of raised Intracranial pressure such as tumours, hydrocephalus and cerebral venous sinus thrombosis were excluded. A total of 23,182 new IIH cases were diagnosed. Fifty-two percent resided in the most socially deprived areas (quintiles 1 and 2). Incidence rose between 2002 and 2016 from 2.3 to 4.7 per 100,000 in the general population. Peak incidence occurred in females aged 25 (15.2 per 100,000). 91.6% were treated medically, 7.6% had a cerebrospinal fluid diversion procedure, 0.7% underwent bariatric surgery and 0.1% had optic nerve sheath fenestration. Elective caesarean sections rates were significantly higher in IIH (16%) compared to the general population (9%), p < 0.005. Admission rates rose by 442% between 2002 and 2014, with 38% having repeated admissions in the year following diagnosis. Duration of hospital admission was 2.7 days (8.8 days for those having CSF diversion procedures). Costs rose from £9.2 to £50 million per annum over the study period with costs forecasts of £462 million per annum by 2030. IIH incidence is rising (by greater than 100% over the study), highest in areas of social deprivation and mirroring obesity trends. Re-admissions rates are high and growing yearly. The escalating population and financial burden of IIH has wide reaching implications for the health care system.

  • evaluation and management of adult Idiopathic Intracranial Hypertension
    Practical Neurology, 2018
    Co-Authors: Susan P Mollan, James L Mitchell, Catherine Hornby, Alexandra J Sinclair
    Abstract:

    This paper summarises the first consensus guidelines for Idiopathic Intracranial Hypertension as an infographic. Following a systematic literature review, a multidisciplinary specialist interest group met and established questions relating to population, interventions, controls and outcomes (PICO). A survey was sent to doctors who manage Idiopathic Intracranial Hypertension (IIH) regularly. Statements were reviewed by national professional bodies, specifically the Association of British Neurologists, British Association for the Study of Headache, the Society of British Neurological Surgeons and the Royal College of Ophthalmologists and by international experts. Key areas are represented based on the guideline, namely: (1) investigation of papilloedema and diagnosis of IIH; (2) management strategies; and (3) investigation and management of acute exacerbation of headache in established IIH. We present an infographic as an aide-memoire of the first consensus guidelines for IIH.

  • european headache federation guideline on Idiopathic Intracranial Hypertension
    Journal of Headache and Pain, 2018
    Co-Authors: Jan Hoffmann, Susan P Mollan, Rigmor Jensen, Koen Paemeleire, Christian Lampl, Alexandra J Sinclair
    Abstract:

    Idiopathic Intracranial Hypertension (IIH) is characterized by an elevation of Intracranial pressure (ICP no identifiable cause. The aetiology remains largely unknown, however observations made in a number of recent clinical studies are increasing the understanding of the disease and now provide the basis for evidence-based treatment strategies. The Embase, CDSR, CENTRAL, DARE and MEDLINE databases were searched up to 1st June 2018. We analyzed randomized controlled trials and systematic reviews that investigate IIH. Diagnostic uncertainty, headache morbidity and visual loss are among the highest concerns of clinicians and patients in this disease area. Research in this field is infrequent due to the rarity of the disease and the lack of understanding of the underlying pathology. This European Headache Federation consensus paper provides evidence-based recommendations and practical advice on the investigation and management of IIH.

Valerie Biousse - One of the best experts on this subject based on the ideXlab platform.

  • do most patients with a spontaneous cerebrospinal fluid leak have Idiopathic Intracranial Hypertension
    Journal of Neuro-ophthalmology, 2019
    Co-Authors: Samuel Bidot, Amit M Saindane, Nancy J Newman, Joshua M Levy, Nelson M Oyesiku, Valerie Biousse
    Abstract:

    Background:The association between cerebrospinal fluid (CSF) leaks at the skull base and raised Intracranial pressure (ICP) has been reported since the 1960s. It has been suggested that spontaneous CSF leaks might represent a variant of Idiopathic Intracranial Hypertension (IIH). We review the evide

  • Asymmetric papilledema in Idiopathic Intracranial Hypertension.
    Journal of Neuro-Ophthalmology, 2015
    Co-Authors: Samuel Bidot, Amit M Saindane, Nancy J Newman, Beau B Bruce, Valerie Biousse
    Abstract:

    Background Very asymmetric papilledema in Idiopathic Intracranial Hypertension (IIH) is rare, and few studies have dealt with this atypical presentation of IIH. Our aim was to describe the clinical and radiologic features of patients with IIH and very asymmetric papilledema.

  • Brain Imaging in Idiopathic Intracranial Hypertension.
    Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society, 2015
    Co-Authors: Samuel Bidot, Amit M Saindane, Nancy J Newman, Beau B Bruce, Jason H. Peragallo, Valerie Biousse
    Abstract:

    Background:The primary role of brain imaging in Idiopathic Intracranial Hypertension (IIH) is to exclude other pathologies causing Intracranial Hypertension. However, subtle radiologic findings suggestive of IIH have emerged with modern neuroimaging. This review provides a detailed description of th

  • meningoceles in Idiopathic Intracranial Hypertension
    American Journal of Roentgenology, 2014
    Co-Authors: Omer Y Bialer, Valerie Biousse, Nancy J Newman, Beau B Bruce, Mario Perez Rueda, Amit M Saindane
    Abstract:

    OBJECTIVE. MRI abnormalities have been described in patients with increased Intracranial pressure (ICP), including in those with Idiopathic Intracranial Hypertension (IIH). Spontaneous CSF-filled outpouchings of the dura (meningoceles) and secondary CSF leaks can occur from elevated ICP in patients with IIH; however, few studies have evaluated these findings. Our objective was to evaluate the frequency of spontaneous Intracranial meningoceles among IIH patients and determine their association with visual outcome. MATERIALS AND METHODS. We performed a retrospective case-control study of consecutive IIH patients between 2000 and 2011 who underwent MRI that included T2-weighted imaging. Demographics, presenting symptoms, CSF opening pressure, and visual outcome were collected for the first and last evaluations. Control subjects included patients without headache or visual complaints who had normal brain MRI results. Stratified analysis was used to control for potential confounding by age, sex, race, and body...

  • primary spontaneous cerebrospinal fluid leaks and Idiopathic Intracranial Hypertension
    Journal of Neuro-ophthalmology, 2013
    Co-Authors: Mario A Perez, Beau B Bruce, Nancy J Newman, Omer Y Bialer, Valerie Biousse
    Abstract:

    Introduction Idiopathic Intracranial Hypertension (IIH) is increasingly recognized as a cause of spontaneous cerebrospinal (CSF) leak in the ENT and neurosurgical literature. The diagnosis of IIH in patients with spontaneous CSF leaks is classically made a few weeks after surgical repair of the CSF leak when symptoms and signs of elevated Intracranial pressure (ICP) appear.

Keira Markey - One of the best experts on this subject based on the ideXlab platform.

  • 11β hydroxysteroid dehydrogenase type 1 inhibition in Idiopathic Intracranial Hypertension a double blind randomized controlled trial
    bioRxiv, 2019
    Co-Authors: Keira Markey, James L Mitchell, Hannah Botfield, Ryan S Ottridge, Tim Matthews, Anita Krishnan, Rebecca Woolley, Connar Westgate
    Abstract:

    Treatment options for Idiopathic Intracranial Hypertension are limited. The enzyme 11{beta}-hydroxysteroid dehydrogenase type 1 has been implicated in regulating cerebrospinal fluid secretion, and its activity is associated with alterations in Intracranial pressure in Idiopathic Intracranial Hypertension. We assessed therapeutic efficacy, safety and tolerability, and investigate indicators of in vivo efficacy of the 11{beta}-hydroxysteroid dehydrogenase type 1 inhibitor AZD4017 compared to placebo in Idiopathic Intracranial Hypertension. A multicenter, UK, 16-week phase II randomized, double-blind, placebo-controlled trial of 12-weeks treatment with AZD4017 or placebo was conducted. Women aged 18 to 55 years with active Idiopathic Intracranial Hypertension (>25cmH2O lumbar puncture opening pressure and active papilledema) were included. Participants received 400mg twice daily of oral AZD4017 compared to matching placebo over 12-weeks. The outcome measures were initial efficacy, safety and tolerability. The primary clinical outcome was lumbar puncture opening pressure at 12 weeks analysed by intention-to-treat. Secondary clinical outcomes were symptoms, visual function, papilledema, headache and anthropological measures. In vivo efficacy was evaluated in the central nervous system and systemically. 31 subjects (mean age 31.2 (SD=6.9) years and BMI 39.2 (SD=12.6) kg/m2) were randomized to AZD4017 (n=17) or placebo (n=14). At 12 weeks, lumbar puncture pressure was lower in the AZD4017 group (29.7 cmH2O) compared with placebo (31.3 cmH2O), but the difference between groups was not statistically significant (mean difference: -2.8, 95% confidence interval: -7.1-1.5; p=0.2). An exploratory analysis assessing mean change in lumbar puncture pressure within each group found a significant decrease in the AZD4017 group (mean change: -4.3 cmH2O (SD=5.7); p=0.009) but not in the placebo group (mean change: -0.3 cmH2O (SD=5.9); p=0.8). AZD4017 was safe, with no withdrawals related to adverse effects. Nine transient drug-related adverse events were reported. One serious adverse event occurred in the placebo group (deterioration requiring shunt surgery). In vivo biomarkers of 11{beta}-hydroxysteroid dehydrogenase type 1 activity (urinary glucocorticoid metabolites, hepatic prednisolone generation and CSF cortisone to cortisol ratios) demonstrated significant enzyme inhibition. This is the first phase 2 randomized controlled trial in Idiopathic Intracranial Hypertension evaluating a novel therapeutic target. AZD4017 was safe, well-tolerated and inhibited 11{beta}-hydroxysteroid dehydrogenase type 1 activity in vivo. Possible clinical benefits were noted in this small cohort. A longer, larger study would now be of interest.

  • therapeutic lumbar puncture for headache in Idiopathic Intracranial Hypertension minimal gain is it worth the pain
    Cephalalgia, 2019
    Co-Authors: Keira Markey, Hannah Botfield, Andreas Yiangou, James Mitchell, William J Scotton, Peter Nightingale, Ryan S Ottridge
    Abstract:

    BackgroundHeadache is disabling and prevalent in Idiopathic Intracranial Hypertension. Therapeutic lumbar punctures may be considered to manage headache. This study evaluated the acute effect of lu...

  • Idiopathic Intracranial Hypertension hormones and 11β hydroxysteroid dehydrogenases
    Journal of Pain Research, 2016
    Co-Authors: Keira Markey, Hannah Botfield, Rigmor Jensen, Maria Uldall, Liam D Cato, Mohammed A L Miah, Ghaniah Hassansmith, Ana M Gonzalez, Alexandra J Sinclair
    Abstract:

    Idiopathic Intracranial Hypertension (IIH) results in raised Intracranial pressure (ICP) leading to papilledema, visual dysfunction, and headaches. Obese females of reproductive age are predominantly affected, but the underlying pathological mechanisms behind IIH remain unknown. This review provides an overview of pathogenic factors that could result in IIH with particular focus on hormones and the impact of obesity, including its role in neuroendocrine signaling and driving inflammation. Despite occurring almost exclusively in obese women, there have been a few studies evaluating the mechanisms by which hormones and adipokines exert their effects on ICP regulation in IIH. Research involving 11β-hydroxysteroid dehydrogenase type 1, a modulator of glucocorticoids, suggests a potential role in IIH. Improved understanding of the complex interplay between adipose signaling factors such as adipokines, steroid hormones, and ICP regulation may be key to the understanding and future management of IIH.

  • understanding Idiopathic Intracranial Hypertension mechanisms management and future directions
    Lancet Neurology, 2016
    Co-Authors: Keira Markey, Susan P Mollan, Rigmor Jensen, Alexandra J Sinclair
    Abstract:

    Idiopathic Intracranial Hypertension is a disorder characterised by raised Intracranial pressure that predominantly affects young, obese women. Pathogenesis has not been fully elucidated, but several causal factors have been proposed. Symptoms can include headaches, visual loss, pulsatile tinnitus, and back and neck pain, but the clinical presentation is highly variable. Although few studies have been done to support evidence-based management, several recent advances have the potential to enhance understanding of the causes of the disease and to guide treatment decisions. Investigators of the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) reported beneficial effects of acetazolamide in patients with mild visual loss. Studies have also established weight loss as an effective disease-modifying treatment, and further clinical trials to investigate new treatments are underway. The incidence of Idiopathic Intracranial Hypertension is expected to increase as rates of obesity increase; efforts to reduce diagnostic delays and identify new, effective approaches to treatment will be key to meeting the needs of a growing number of patients.

  • a practical approach to diagnosis assessment and management of Idiopathic Intracranial Hypertension
    Practical Neurology, 2014
    Co-Authors: Susan P Mollan, Keira Markey, Tim Matthews, Michael A Burdon, Andrew S Jacks, James D Benzimra, Alexandra J Sinclair
    Abstract:

    Adult patients who present with papilloedema and symptoms of raised Intracranial pressure need urgent multidisciplinary assessment including neuroimaging, to exclude life-threatening causes. Where there is no apparent underlying cause for the raised Intracranial pressure, patients are considered to have Idiopathic Intracranial Hypertension (IIH). The incidence of IIH is increasing in line with the global epidemic of obesity. There are controversial issues in its diagnosis and management. This paper gives a practical approach to assessing patients with papilloedema, its investigation and the subsequent management of patients with IIH.

Michael Wall - One of the best experts on this subject based on the ideXlab platform.

  • update on Idiopathic Intracranial Hypertension
    Neurologic Clinics, 2017
    Co-Authors: Michael Wall
    Abstract:

    Idiopathic Intracranial Hypertension (IIH) is a disorder of elevated Intracranial pressure of unknown cause occurring predominantly in young women of childbearing age. The typical patient symptom profile is the presence of daily headache, pulse synchronous tinnitus, transient visual obscurations, and papilledema with its associated visual loss. Although surgical procedures are performed for those who fail medical therapy, their relative efficacy remains unclear. The main morbidity of IIH is from visual loss. This visual loss is present in most patients and can usually be reversed if recognized early in the patients' course and treated.

  • safety and tolerability of acetazolamide in the Idiopathic Intracranial Hypertension treatment trial
    Journal of Neuro-ophthalmology, 2016
    Co-Authors: Martin Ten Hove, Deborah I. Friedman, Michael Wall, Anil D Patel, Isabella Irrcher, Michael P Mcdermott
    Abstract:

    OBJECTIVE To examine the tolerability and adverse events reported in the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT). METHODS Randomized, double-masked, placebo-controlled clinical trial. Trial participants (n = 165) with mild visual loss concurrently receiving low-sodium weight-reduction diet plus the maximally tolerated dosage of acetazolamide (up to 4 g/d) or placebo for 6 months. MAIN OUTCOMES MEASURES adverse events (AEs), assessment of clinical and laboratory findings at study visits. RESULTS Thirty-eight of 86 participants randomized to the acetazolamide group (44.1%) tolerated the maximum allowed dosage of 4 g/d. The average time to achieve maximum study dosage in the acetazolamide group was 13 weeks (median 12 weeks; range 10-24 weeks). A total of 676 AEs (acetazolamide, n = 480; placebo, n = 196) and 9 serious AEs (acetazolamide, n = 6; placebo, n = 3) were reported. Notably, the percentages of participants reporting at least 1 AE in the nervous, gastrointestinal, metabolic, and renal organ systems were significantly higher in the acetazolamide group (P < 0.05). The odds of paresthesia (OR 9.82; 95% CI 3.87-27.82), dysgeusia (OR ∞; 95% CI 3.99-∞), vomiting and diarrhea (OR 4.11; 95% CI 1.04-23.41), nausea (OR 2.99; 95% CI 1.26-7.49) and fatigue (OR 16.48; 95% CI 2.39-702.40) were higher in the acetazolamide group than in the placebo group. CONCLUSION Acetazolamide appears to have an acceptable safety profile at dosages up to 4 g/d in the treatment of Idiopathic Intracranial Hypertension. The majority of participants in the Idiopathic Intracranial Hypertension Treatment Trial were able to tolerate acetazolamide above 1 g/d for 6 months.

  • the Idiopathic Intracranial Hypertension treatment trial design considerations and methods
    Journal of Neuro-ophthalmology, 2014
    Co-Authors: Deborah I. Friedman, Michael P Mcdermott, James J Corbett, Karl Kieburtz, Mark J Kupersmith, Ann Stoutenburg, John L Keltner, Steven E Feldon, Eleanor Schron, Michael Wall
    Abstract:

    Background:The objectives of this study were to present the rationale for the main aspects of the study design and describe the trial methodology for the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT).Methods:Eligible candidates with mild visual field loss (automated perimetric mean de

  • the Idiopathic Intracranial Hypertension treatment trial clinical profile at baseline
    JAMA Neurology, 2014
    Co-Authors: Michael Wall, Deborah I. Friedman, James J Corbett, Karl Kieburtz, Mark J Kupersmith, John L Keltner, Steven E Feldon, Eleanor Schron, David M Katz, Michael P Mcdermott
    Abstract:

    Importance To our knowledge, there are no large prospective cohorts of untreated patients with Idiopathic Intracranial Hypertension (IIH) to characterize the disease. Objective To report the baseline clinical and laboratory features of patients enrolled in the Idiopathic Intracranial Hypertension Treatment Trial. Design, Setting, and Participants We collected data at baseline from questionnaires, examinations, automated perimetry, and fundus photography grading. Patients (n = 165) were enrolled from March 17, 2010, to November 27, 2012, at 38 academic and private practice sites in North America. All participants met the modified Dandy criteria for IIH and had a perimetric mean deviation between −2 dB and −7 dB. All but 4 participants were women. Main Outcomes and Measures Baseline and laboratory characteristics. Results The mean (SD) age of our patients was 29.0 (7.4) years and 4 (2.4%) were men. The average (SD) body mass index (calculated as weight in kilograms divided by height in meters squared) was 39.9 (8.3). Headache was the most common symptom (84%). Transient visual obscurations occurred in 68% of patients, back pain in 53%, and pulse synchronous tinnitus in 52%. Only 32% reported visual loss. The average (SD) perimetric mean deviation in the worst eye was −3.5 (1.1) dB, (range, −2.0 to −6.4 dB) and in the best eye was −2.3 (1.1) dB (range, −5.2 to 0.8 dB). A partial arcuate visual field defect with an enlarged blind spot was the most common perimetric finding. Visual acuity was 85 letters or better (20/20) in 71% of the worst eyes and 77% of the best eyes. Quality of life measures, including the National Eye Institute Visual Function Questionnaire–25 and the Short Form–36 physical and mental health summary scales, were lower compared with population norms. Conclusions and Relevance The Idiopathic Intracranial Hypertension Treatment Trial represents the largest prospectively analyzed cohort of untreated patients with IIH. Our data show that IIH is almost exclusively a disease of obese young women. Patients with IIH with mild visual loss have typical symptoms, may have mild acuity loss, and have visual field defects, with predominantly arcuate loss and enlarged blind spots that require formal perimetry for detection. Trial Registration clinicaltrials.gov Identifier:NCT01003639

  • Epidemiology and risk factors for Idiopathic Intracranial Hypertension.
    International ophthalmology clinics, 2014
    Co-Authors: John J. Chen, Michael Wall
    Abstract:

    Idiopathic Intracranial Hypertension (IIH) is a syndrome characterized by elevated Intracranial pressure that usually occurs in obese women in the childbearing years. The signs and symptoms of Intracranial Hypertension are that the patient maintains an alert and oriented mental state, but has no localizing neurologic findings. There is no evidence of deformity or obstruction of the ventricular system and neurodiagnostic studies are otherwise normal except for increased cerebrospinal fluid pressure.1 Neuroimaging signs of increased Intracranial pressure include empty sella syndrome, smooth-walled venous stenoses, flattened globes and fully unfolded optic nerve sheaths. In addition, no secondary cause of Intracranial Hypertension can be found. This definition comprises the modified Dandy criteria for IIH.2 There is considerable literature on IIH epidemiology, risk factors and secondary causes of Intracranial Hypertension. In this review, we will focus on cases of IIH where the modified Dandy criteria are met.

Deborah I. Friedman - One of the best experts on this subject based on the ideXlab platform.

  • the Idiopathic Intracranial Hypertension treatment trial a review of the outcomes
    Headache, 2017
    Co-Authors: Stacy V Smith, Deborah I. Friedman
    Abstract:

    Objective The Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) was the first large, randomized study on the use of acetazolamide and weight loss for treatment of Idiopathic Intracranial Hypertension-associated vision loss. The multicenter trial also examined a number of secondary outcomes. This review summarizes all available results of the study published in the literature since 2014. Background Prior to the IIHTT, clinicians managed Idiopathic Intracranial Hypertension based on data from small, unmasked trials, expert opinion, and clinical experience. Due to the lack of empiric evidence, there were no official treatment protocols to guide treatment of the disorder. Methods We performed a PubMed literature search for all articles with data from the IIHTT Study Group. After review of each article and any relevant supporting literature, the results were compiled into a summary of the literature. Results The PubMed search identified 14 articles with primary and/or secondary outcome data from the IIHTT. We summarized the findings for the primary outcome of visual field outcomes in the acetazolamide treatment group compared to the placebo group, as well as secondary outcomes for the safety and tolerability of acetazolamide, cerebrospinal fluid opening pressure, quality of life, fundus photography, and optical coherence tomography. While both groups demonstrated improvement on most outcomes, acetazolamide had a greater effect even when controlling for its effect on weight loss. Conclusions As the first large, randomized, prospective trial, the IIHTT extensively expanded the available data on Idiopathic Intracranial Hypertension treatment. Most importantly, it provided support for the safe use of acetazolamide up to 4 g daily with weight loss for effective treatment of mild vision loss in IIH, with associated improvements in papilledema, increased Intracranial pressure, and quality of life.

  • safety and tolerability of acetazolamide in the Idiopathic Intracranial Hypertension treatment trial
    Journal of Neuro-ophthalmology, 2016
    Co-Authors: Martin Ten Hove, Deborah I. Friedman, Michael Wall, Anil D Patel, Isabella Irrcher, Michael P Mcdermott
    Abstract:

    OBJECTIVE To examine the tolerability and adverse events reported in the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT). METHODS Randomized, double-masked, placebo-controlled clinical trial. Trial participants (n = 165) with mild visual loss concurrently receiving low-sodium weight-reduction diet plus the maximally tolerated dosage of acetazolamide (up to 4 g/d) or placebo for 6 months. MAIN OUTCOMES MEASURES adverse events (AEs), assessment of clinical and laboratory findings at study visits. RESULTS Thirty-eight of 86 participants randomized to the acetazolamide group (44.1%) tolerated the maximum allowed dosage of 4 g/d. The average time to achieve maximum study dosage in the acetazolamide group was 13 weeks (median 12 weeks; range 10-24 weeks). A total of 676 AEs (acetazolamide, n = 480; placebo, n = 196) and 9 serious AEs (acetazolamide, n = 6; placebo, n = 3) were reported. Notably, the percentages of participants reporting at least 1 AE in the nervous, gastrointestinal, metabolic, and renal organ systems were significantly higher in the acetazolamide group (P < 0.05). The odds of paresthesia (OR 9.82; 95% CI 3.87-27.82), dysgeusia (OR ∞; 95% CI 3.99-∞), vomiting and diarrhea (OR 4.11; 95% CI 1.04-23.41), nausea (OR 2.99; 95% CI 1.26-7.49) and fatigue (OR 16.48; 95% CI 2.39-702.40) were higher in the acetazolamide group than in the placebo group. CONCLUSION Acetazolamide appears to have an acceptable safety profile at dosages up to 4 g/d in the treatment of Idiopathic Intracranial Hypertension. The majority of participants in the Idiopathic Intracranial Hypertension Treatment Trial were able to tolerate acetazolamide above 1 g/d for 6 months.

  • evolving evidence in adult Idiopathic Intracranial Hypertension pathophysiology and management
    Journal of Neurology Neurosurgery and Psychiatry, 2016
    Co-Authors: Susan P Mollan, Deborah I. Friedman, Hannah Botfield, Alexandra J Sinclair, Ghaniah Hassansmith, Fizzah Ali
    Abstract:

    Idiopathic Intracranial Hypertension (IIH) is a rare but important disease associated with significant morbidity. There is an expected rise in prevalence in line with the escalating global burden of obesity. Modern revisions in the terminology and diagnostic criteria for IIH help guide clinicians in investigations and researchers in standardising recruitment criteria for clinical trials. The pathophysiology of IIH is incompletely characterised; suggested underpinning mechanisms include the role of cerebrospinal fluid regulation as well as metabolic and endocrinological perspectives. Recent treatment trials are providing insights into the management but debate still surrounds key areas in treatment. This review will provide an up-to-date discussion on the potential pathogenic mechanisms and management of IIH.

  • the Idiopathic Intracranial Hypertension treatment trial design considerations and methods
    Journal of Neuro-ophthalmology, 2014
    Co-Authors: Deborah I. Friedman, Michael P Mcdermott, James J Corbett, Karl Kieburtz, Mark J Kupersmith, Ann Stoutenburg, John L Keltner, Steven E Feldon, Eleanor Schron, Michael Wall
    Abstract:

    Background:The objectives of this study were to present the rationale for the main aspects of the study design and describe the trial methodology for the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT).Methods:Eligible candidates with mild visual field loss (automated perimetric mean de

  • the Idiopathic Intracranial Hypertension treatment trial clinical profile at baseline
    JAMA Neurology, 2014
    Co-Authors: Michael Wall, Deborah I. Friedman, James J Corbett, Karl Kieburtz, Mark J Kupersmith, John L Keltner, Steven E Feldon, Eleanor Schron, David M Katz, Michael P Mcdermott
    Abstract:

    Importance To our knowledge, there are no large prospective cohorts of untreated patients with Idiopathic Intracranial Hypertension (IIH) to characterize the disease. Objective To report the baseline clinical and laboratory features of patients enrolled in the Idiopathic Intracranial Hypertension Treatment Trial. Design, Setting, and Participants We collected data at baseline from questionnaires, examinations, automated perimetry, and fundus photography grading. Patients (n = 165) were enrolled from March 17, 2010, to November 27, 2012, at 38 academic and private practice sites in North America. All participants met the modified Dandy criteria for IIH and had a perimetric mean deviation between −2 dB and −7 dB. All but 4 participants were women. Main Outcomes and Measures Baseline and laboratory characteristics. Results The mean (SD) age of our patients was 29.0 (7.4) years and 4 (2.4%) were men. The average (SD) body mass index (calculated as weight in kilograms divided by height in meters squared) was 39.9 (8.3). Headache was the most common symptom (84%). Transient visual obscurations occurred in 68% of patients, back pain in 53%, and pulse synchronous tinnitus in 52%. Only 32% reported visual loss. The average (SD) perimetric mean deviation in the worst eye was −3.5 (1.1) dB, (range, −2.0 to −6.4 dB) and in the best eye was −2.3 (1.1) dB (range, −5.2 to 0.8 dB). A partial arcuate visual field defect with an enlarged blind spot was the most common perimetric finding. Visual acuity was 85 letters or better (20/20) in 71% of the worst eyes and 77% of the best eyes. Quality of life measures, including the National Eye Institute Visual Function Questionnaire–25 and the Short Form–36 physical and mental health summary scales, were lower compared with population norms. Conclusions and Relevance The Idiopathic Intracranial Hypertension Treatment Trial represents the largest prospectively analyzed cohort of untreated patients with IIH. Our data show that IIH is almost exclusively a disease of obese young women. Patients with IIH with mild visual loss have typical symptoms, may have mild acuity loss, and have visual field defects, with predominantly arcuate loss and enlarged blind spots that require formal perimetry for detection. Trial Registration clinicaltrials.gov Identifier:NCT01003639