-
Clinical & Experimental Ophthalmology Aug 2023Little is known about the presentation and prognosis of asymptomatic idiopathic intracranial hypertension (IIH). Papilloedema can be found incidentally on routine fundus... (Observational Study)
Observational Study
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 present with or without symptoms.
METHODS
Prospective observational cohort study, between 2012 and 2021, 343 people with confirmed IIH diagnosis were enrolled in the IIH:Life database. Outcomes such as vision (LogMAR); Humphrey visual field perimetric mean deviation (PMD) and optical coherence tomography (OCT) and headache were evaluated using LOESS (locally weighted scatterplot smoothing) graphs and regression analysis.
RESULTS
One hundred and twenty-one people had incidentally found papilloedema, with 36 people with completely asymptomatic presentations. Those with asymptomatic IIH at diagnosis had similar visual prognosis compared to those with symptomatic disease. Sixty-six percent of the asymptomatic cohort became symptomatic during follow-up, and of these the predominant symptom was headache (96%). Headache frequency during follow-up was lower in the asymptomatic cohort.
CONCLUSIONS
The prognosis of those with IIH who present with or without symptoms is similar.
Topics: Humans; Pseudotumor Cerebri; Papilledema; Prevalence; Prospective Studies; Prognosis; Headache; Intracranial Hypertension
PubMed: 37243483
DOI: 10.1111/ceo.14256 -
American Journal of Ophthalmology Feb 2015To examine the clinical, demographic, and anthropometric patient characteristics of secondary pseudotumor cerebri syndrome in children and adolescents based on the... (Observational Study)
Observational Study
PURPOSE
To examine the clinical, demographic, and anthropometric patient characteristics of secondary pseudotumor cerebri syndrome in children and adolescents based on the recently revised diagnostic criteria.
DESIGN
Retrospective observational case series.
METHODS
Patients seen at a tertiary children's hospital for pseudotumor cerebri syndrome were classified as having either primary idiopathic (n = 59) or secondary pseudotumor cerebri syndrome (n = 16), as rigorously defined by recently revised diagnostic criteria. Outcomes included body mass index Z-scores (BMI-Z), height and weight Z-scores, demographics, and clinical features at presentation, such as headache, sixth nerve palsy, and cerebrospinal fluid (CSF) opening pressure.
RESULTS
In this cohort, the associated conditions and exposures seen in definite secondary pseudotumor cerebri syndrome included tetracycline-class antibiotics (n = 11), chronic kidney disease (n = 3), withdrawal from chronic glucocorticoids (n = 1), and lithium (n = 1). Other associations observed in the possible secondary pseudotumor cerebri syndrome group included Down syndrome, vitamin A derivatives, and growth hormone. In comparison with primary pseudotumor cerebri syndrome, definite secondary pseudotumor cerebri syndrome patients were on average older (15.0 vs 11.6 years; P = .003, Mann-Whitney test). According to US Centers for Disease Control (CDC) classifications, 79% of children with secondary pseudotumor cerebri syndrome were either overweight or obese (36% overweight [n = 5] and 43% obese [n = 6]), as compared to 32% nationally.
CONCLUSIONS
Even when a potential inciting exposure is identified for pediatric pseudotumor cerebri syndrome, the possible contribution of overweight and obesity should be considered.
Topics: Adolescent; Body Constitution; Body Mass Index; Cerebrospinal Fluid Pressure; Child; Child, Preschool; Female; Hospitals, Pediatric; Humans; Intracranial Pressure; Male; Obesity; Overweight; Pseudotumor Cerebri; Retrospective Studies
PubMed: 25447107
DOI: 10.1016/j.ajo.2014.11.003 -
Hormone Research in Paediatrics 2014Idiopathic intracranial hypertension (IIH), also known as primary pseudotumor cerebri syndrome (PTCS), is a condition of unknown etiology which affects primarily... (Review)
Review
Idiopathic intracranial hypertension (IIH), also known as primary pseudotumor cerebri syndrome (PTCS), is a condition of unknown etiology which affects primarily overweight, reproductive-aged women and causes increased intracranial pressure (ICP). This review discusses the recently revised diagnostic criteria for PTCS for adults and children. Additionally, the role of obesity in the epidemiology, etiology, and management of IIH as well as the current knowledge of obesity profiles and markers in IIH are reviewed. We also highlight the emerging, unifying theory of the neuroendocrine effects on the mineralocorticoid receptor to explain a possible mechanism for the increased cerebrospinal fluid production and ICP in secondary PTCS.
Topics: Adult; Child; Female; Humans; Male; Obesity; Pseudotumor Cerebri
PubMed: 24642712
DOI: 10.1159/000357730 -
JAMAAcetazolamide is commonly used to treat idiopathic intracranial hypertension (IIH), but there is insufficient information to establish an evidence base for its use. (Randomized Controlled Trial)
Randomized Controlled Trial
Effect of acetazolamide on visual function in patients with idiopathic intracranial hypertension and mild visual loss: the idiopathic intracranial hypertension treatment trial.
IMPORTANCE
Acetazolamide is commonly used to treat idiopathic intracranial hypertension (IIH), but there is insufficient information to establish an evidence base for its use.
OBJECTIVE
To determine whether acetazolamide is beneficial in improving vision when added to a low-sodium weight reduction diet in patients with IIH and mild visual loss.
DESIGN, SETTING, AND PARTICIPANTS
Multicenter, randomized, double-masked, placebo-controlled study of acetazolamide in 165 participants with IIH and mild visual loss who received a low-sodium weight-reduction diet. Participants were enrolled at 38 academic and private practice sites in North America from March 2010 to November 2012 and followed up for 6 months (last visit in June 2013). All participants met the modified Dandy criteria for IIH and had a perimetric mean deviation (PMD) between -2 dB and -7 dB. The mean age was 29 years and all but 4 participants were women.
INTERVENTIONS
Low-sodium weight-reduction diet plus the maximally tolerated dosage of acetazolamide (up to 4 g/d) or matching placebo for 6 months.
MAIN OUTCOMES AND MEASURES
The planned primary outcome variable was the change in PMD from baseline to month 6 in the most affected eye, as measured by Humphrey Field Analyzer. Perimetric mean deviation is a measure of global visual field loss (mean deviation from age-corrected normal values), with a range of 2 to -32 dB; larger negative values indicate greater vision loss. Secondary outcome variables included changes in papilledema grade, quality of life (Visual Function Questionnaire 25 [VFQ-25] and 36-Item Short Form Health Survey), headache disability, and weight at month 6.
RESULTS
The mean improvement in PMD was greater with acetazolamide (1.43 dB, from -3.53 dB at baseline to -2.10 dB at month 6; n = 86) than with placebo (0.71 dB, from -3.53 dB to -2.82 dB; n = 79); the difference was 0.71 dB (95% CI, 0 to 1.43 dB; P = .050). Mean improvements in papilledema grade (acetazolamide: -1.31, from 2.76 to 1.45; placebo: -0.61, from 2.76 to 2.15; treatment effect, -0.70; 95% CI, -0.99 to -0.41; P < .001) and vision-related quality of life as measured by the National Eye Institute VFQ-25 (acetazolamide: 8.33, from 82.97 to 91.30; placebo: 1.98, from 82.97 to 84.95; treatment effect, 6.35; 95% CI, 2.22 to 10.47; P = .003) and its 10-item neuro-ophthalmic supplement (acetazolamide: 9.82, from 75.45 to 85.27; placebo: 1.59, from 75.45 to 77.04; treatment effect, 8.23; 95% CI, 3.89 to 12.56; P < .001) were also observed with acetazolamide. Participants assigned to acetazolamide also experienced a reduction in weight (acetazolamide: -7.50 kg, from 107.72 kg to 100.22 kg; placebo: -3.45 kg, from 107.72 kg to 104.27 kg; treatment effect, -4.05 kg, 95% CI, -6.27 to -1.83 kg; P < .001).
CONCLUSIONS AND RELEVANCE
In patients with IIH and mild visual loss, the use of acetazolamide with a low-sodium weight-reduction diet compared with diet alone resulted in modest improvement in visual field function. The clinical importance of this improvement remains to be determined.
TRIAL REGISTRATION
clinicaltrials.gov Identifier: NCT01003639.
Topics: Acetazolamide; Adult; Carbonic Anhydrase Inhibitors; Combined Modality Therapy; Diet, Sodium-Restricted; Female; Humans; Male; Pseudotumor Cerebri; Quality of Life; Treatment Outcome; Vision Disorders; Weight Loss
PubMed: 24756514
DOI: 10.1001/jama.2014.3312 -
Arquivos de Neuro-psiquiatria May 2022Idiopathic Intracranial Hypertension (IIH) is a secondary headache with a steadily growing incidence. Currently, there is little evidence to guide the treatment of IIH. (Review)
Review
BACKGROUND
Idiopathic Intracranial Hypertension (IIH) is a secondary headache with a steadily growing incidence. Currently, there is little evidence to guide the treatment of IIH.
OBJECTIVE
To review the pathophysiology of IIH, with focus on the role of obesity as a risk factor, and the implications for new therapeutic perspectives.
METHODS
in this narrative review, we summarized the current knowledge on treatment options highlighting available evidence for managing intracranial hypertension, obesity, and headache.
RESULTS
Clinical Presentation: headache is the most common symptom and a significant cause of quality-of-life impairment. Visual loss is common in the diagnosis. Pathophysiology: there is no unified theory able to explain all symptoms and the evolution of the disease. There is growing data pointing to metabolic changes and obesity with a central role in IIH pathophysiology. Treatment: most published data on IIH treatment is related to pressure control and protection from visual loss. Acetazolamide and cerebrospinal fluid diversion are the best options available. Optic nerve sheath fenestration might be useful to temporally control the pressure over the optic nerve and thus protect from visual deterioration. Recently, venous sinus stenting has proven to be a safe option in selected cases. Finally, bariatric surgery has proven to effectively control elevated intracranial pressure.
CONCLUSION
IIH is a potential cause of high disability. Early recognition is important, and treatment should be tailored to the needs of each case. There is a lack of research on headache management, which might persist after ICP control.
Topics: Acetazolamide; Headache; Humans; Intracranial Hypertension; Obesity; Pseudotumor Cerebri; Vision Disorders
PubMed: 35976300
DOI: 10.1590/0004-282X-ANP-2022-S110 -
Indian Journal of Ophthalmology Oct 2014The aim was to identify Pseudotumor cerebri treatment options and assess their efficacy. (Review)
Review
AIMS
The aim was to identify Pseudotumor cerebri treatment options and assess their efficacy.
SETTING AND DESIGN
Review article.
MATERIALS AND METHODS
Existing literature and the authors' experience were reviewed.
RESULTS
Treatment options range from observation to surgical intervention. Weight loss and medical treatment may be utilized in cases without vision loss or in combination with surgical treatment. Cerebrospinal fluid shunting procedures and/or optic nerve sheath decompression is indicated for severe vision loss or headache unresponsive to medical management. The recent use of endovascular stenting of transverse sinus stenoses has also demonstrated benefit in patients with pseudotumor cerebri.
CONCLUSION
While each treatment form may be successful individually, a multimodal approach is typically utilized with treatments selected on a case-by-case basis.
Topics: Disease Management; Humans; Pseudotumor Cerebri
PubMed: 25449933
DOI: 10.4103/0301-4738.145991 -
Neurologia Mar 2024Pseudotumor cerebri (PC) in prepubertal patients displays certain characteristics that differentiate it from its presentation at the postpubertal stage. The aim of this...
INTRODUCTION
Pseudotumor cerebri (PC) in prepubertal patients displays certain characteristics that differentiate it from its presentation at the postpubertal stage. The aim of this study is to describe the characteristics of paediatric patients diagnosed with PC at our centre and to compare them according to their pubertal status.
PATIENTS AND METHODS
We included patients aged between 1 and 18 years who were diagnosed with PC in a tertiary-level hospital between 2006 and 2019 and who met the updated diagnostic criteria for PC. They were classified according to body weight and pubertal status. Subsequently, we analysed results from lumbar punctures, neuroimaging studies, ophthalmological assessments, and treatments received during follow-up.
RESULTS
We included 28 patients, of whom 22 were of prepubertal age and 6 were of postpubertal age. The mean age (standard deviation) was 9.04 (2.86) years. Among the postpubertal patients, 83.3% were boys, 66.7% of whom presented overweight/obesity. In the group of prepubertal patients, 27% were boys, 31.8% of whom were overweight. The most frequent symptoms were headache (89.9%) and blurred vision (42.9%). All patients presented papilloedema, and 21.4% manifested sixth nerve palsy. Possible triggers were identified in 28.6% of cases. Nineteen percent of patients presented clinical recurrence, all of whom were prepubertal patients. Complete clinical resolution was achieved in 55.6% of patients.
CONCLUSION
Prepubertal patients with PC show lower prevalence of obesity, higher prevalence of secondary aetiologies, and higher recurrence rates than postpubertal patients.
Topics: Male; Humans; Child; Infant; Child, Preschool; Adolescent; Female; Pseudotumor Cerebri; Overweight; Retrospective Studies; Prognosis; Obesity
PubMed: 38272258
DOI: 10.1016/j.nrleng.2024.01.004 -
BMJ (Clinical Research Ed.) Jul 2010
Topics: Adult; Diet, Reducing; Female; Humans; Intracranial Pressure; Papilledema; Prospective Studies; Pseudotumor Cerebri
PubMed: 20610513
DOI: 10.1136/bmj.c2836 -
Journal of Neuro-ophthalmology : the... Mar 2022Pediatric pseudotumor cerebri syndrome (PTCS) is a vision-threatening condition that is associated with female sex and obesity in pubertal and postpubertal children. It...
BACKGROUND
Pediatric pseudotumor cerebri syndrome (PTCS) is a vision-threatening condition that is associated with female sex and obesity in pubertal and postpubertal children. It is unknown whether the increase in childhood obesity during the COVID-19 pandemic has affected the rates and characteristics of pediatric PTCS.
METHODS
We conducted a retrospective study of children evaluated for PTCS (inpatient or emergency department) at our children's hospital before (March 19, 2015 to March 19, 2020) and during (March 20, 2020 to February 20, 2021) the pandemic. We compared the monthly number of inpatient and emergency department encounters for pediatric PTCS before and during the pandemic. In addition, anthropometric and ophthalmologic characteristics of children evaluated for pediatric PTCS before and during the pandemic were compared.
RESULTS
A total of 36 encounters in the 5 years before the pandemic and 26 encounters in the 11 months during the pandemic were identified. The median monthly number of encounters for pediatric PTCS was significantly higher during the pandemic compared with the 5 years before the pandemic (2 vs 0, P = 0.0021). Compared with prepandemic patients, children evaluated during the pandemic were older (median age 16 vs 14 years, P = 0.02), with higher rates of obesity (85% vs 66%, P = 0.05) and lower likelihood of reporting Caucasian race (4% vs 31%, P = 0.02). Pandemic patients had worse presenting visual acuity (median logMAR 0.14 vs 0.05, P = 0.05) and were more likely to have fulminant presentation (23% vs 6%, P = 0.04) and require surgical intervention (23% vs 6%, P = 0.04).
CONCLUSIONS
At our children's hospital, the rate of inpatient admissions and emergency department visits for pediatric PTCS increased during the pandemic. The severity of disease and frequency of surgical treatment also increased. Racial and ethnic minorities seem to be disproportionately affected. These changes may be related to increasing rates of childhood obesity during the pandemic.
Topics: Adolescent; COVID-19; Child; Emergency Service, Hospital; Female; Humans; Inpatients; Pandemics; Pediatric Obesity; Pseudotumor Cerebri; Retrospective Studies
PubMed: 34803141
DOI: 10.1097/WNO.0000000000001480 -
Journal of the Neurological Sciences Aug 2000The purpose of this theoretical study is to determine whether the absence of ventricular enlargement in pseudotumor cerebri (PTC) is consistent with the theory that PTC... (Review)
Review
The purpose of this theoretical study is to determine whether the absence of ventricular enlargement in pseudotumor cerebri (PTC) is consistent with the theory that PTC is caused by reduced absorption of cerebrospinal fluid (CSF), either from increased outflow resistance at the arachnoid villi or from obstruction of the dural venous sinuses. We model the brain as a thick spherical shell of parenchyma, enclosing a CSF-filled ventricular system, and surrounded by a thin cerebral subarachnoid space (CSAS). We treat the parenchyma as a porous solid matrix, filled with interstitial fluid and blood vessels. We subject the model to a uniform increase in CSF pressure (CSFP) and solve the equations of poroelasticity for the resulting displacements of parenchymal tissue. The effect of a rise in CSFP on ventricular size depends on the response of the cerebral blood vessels and the degree to which the pia is tethered to the dura. If the cerebral vessels decrease in caliber with increasing CSFP, a rise in CSFP causes the ventricles to contract and the CSAS to expand if the pial surface is free to move inward, but causes slight ventricular enlargement if the pia is tethered to the dura. If, instead, the vessels dilate, the ventricles contract and the CSAS becomes effaced. Small, normal, or slightly enlarged ventricles in PTC are consistent with the theory of reduced CSF absorption.
Topics: Brain Neoplasms; Cerebral Ventricles; Humans; Pseudotumor Cerebri
PubMed: 10980304
DOI: 10.1016/s0022-510x(00)00348-8