Adult Idiopathic Intracranial Hypertension Research Articles

Clinical profile and ophthalmological manifestations of idiopathic intracranial hypertension in adults at a tertiary care center in India: A cross-sectional study.

Our aim was to describe the clinical profile of patients with idiopathic intracranial hypertension (IIH), assess ophthalmological manifestations, and correlate grade of papilledema with optic nerve sheath diameter (ONSD) and cerebrospinal fluid (CSF) opening pressure. This was a prospective cross-sectional study. Patients between 18 and 60 years, diagnosed with IIH using modified Dandy criteria were included. Demographic details, ocular symptoms and signs were noted and papilledema graded. Ocular investigations such as B-scan ONSD and perimetry findings were noted for analysis. The study included 32 patients of mean age 35.25(±9.57) years with a predominantly female population (96.9%). Mean BMI was 28.12(±5.32) kg/m2. Common presenting complaint was headache (87.5%). The most common gynecological disorder was dysmenorrhea (15.6%). Vitamin D deficiency (46.9%) was a biochemical abnormality seen. Most patients had BCVA 6/6 (62.5%). Lateral rectus palsy was present in 12.5%. Papilledema was present in 81.3% eyes with 31.3% Grade I. There was visual field loss in 53.1%, with 20.3% Grade I. Mean CSF opening pressure was 376.3(±191.51) mmH2O. MRI showed empty sella (34.4%) and tortuous optic nerve (18.8%). MRV showed transverse sinus stenosis (52.4%) as common abnormality. There was significant correlation between grade of papilledema and B-scan ONSD. No correlation was seen between CSF opening pressure and ONSD, grade of papilledema and grade of visual field defect and CSF opening pressure with BMI. Clinical profile of patients with IIH was an overweight female of child-bearing age with headache. Visual field examinations are essential in management and follow-up. B-scan ONSD is useful to quantify raised ICP.

Detailed evaluation of cognitive performance in idiopathic intracranial hyper- tension and relevance of intracranial pressure

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 multiple domains of deficiency in a case-control prospective study of 66 female adult IIH patients. Attention networks (executive function) and sustained attention were impaired compared to a body mass index and age matched control group of 25 healthy female participants. These deficits were not permanent as participants exhibited improvement in several domains over 12 months which were associated with reduction in ICP. Cognition was evaluated before and after a lumbar puncture, with acute reduction in ICP and noted significant improvement in executive function. The impact of comorbidities was explored, with headache severity during cognitive testing, co-morbid depression and markers of obstructive sleep apnoea adversely associated with cognitive performance. Deficits were also significantly associated with impaired reliability to perform visual field tests, the cornerstone of monitoring vision in IIH. Our findings propose that cognitive impairment should be recognised as a clinical manifestation of IIH and treating comorbidities could improve cognitive performance.livgrech@googlemail.com

Cognitive performance in idiopathic intracranial hypertension and relevance of intracranial pressure.

Cognitive impairments have been reported in idiopathic intracranial hypertension; however, evidence supporting these deficits is scarce and contributing factors have not been defined. Using a case-control prospective study, we identified multiple domains of deficiency in a cohort of 66 female adult idiopathic intracranial hypertension patients. We identified significantly impaired attention networks (executive function) and sustained attention compared to a body mass index and age matched control group of 25 healthy female participants. We aimed to investigate how cognitive function changed over time and demonstrated that deficits were not permanent. Participants exhibited improvement in several domains including executive function, sustained attention and verbal short-term memory over 12-month follow-up. Improved cognition over time was associated with reduction in intracranial pressure but not body weight. We then evaluated cognition before and after a lumbar puncture with acute reduction in intracranial pressure and noted significant improvement in sustained attention to response task performance. The impact of comorbidities (headache, depression, adiposity and obstructive sleep apnoea) was also explored. We observed that body mass index and the obesity associated cytokine interleukin-6 (serum and cerebrospinal fluid) were not associated with cognitive performance. Headache severity during cognitive testing, co-morbid depression and markers of obstructive sleep apnoea were adversely associated with cognitive performance. Dysregulation of the cortisol generating enzyme 11β hydroxysteroid dehydrogenase type 1 has been observed in idiopathic intracranial hypertension. Elevated cortisol has been associated with impaired cognition. Here, we utilized liquid chromatography-tandem mass spectrometry for multi-steroid profiling in serum and cerebrospinal fluid in idiopathic intracranial hypertension patients. We noted that reduction in the serum cortisol:cortisone ratio in those undergoing bariatric surgery at 12 months was associated with improving verbal working memory. The clinical relevance of cognitive deficits was noted in their significant association with impaired reliability to perform visual field tests, the cornerstone of monitoring vision in idiopathic intracranial hypertension. Our findings propose that cognitive impairment should be accepted as a clinical manifestation of idiopathic intracranial hypertension and impairs the ability to perform visual field testing reliably. Importantly, cognitive deficits can improve over time and with reduction of intracranial pressure. Treating comorbid depression, obstructive sleep apnoea and headache could improve cognitive performance in idiopathic intracranial hypertension.

Management of idiopathic intracranial hypertension in children utilizing venous sinus stenting.

Venous sinus stenting (VSS) is an accepted and minimally invasive treatment for adult idiopathic intracranial hypertension (IIH) associated with lateral sinus stenosis (LSS). The efficacy and safety of venous sinus stenting (VSS) in children with IIH has not been established. This is a retrospective analysis of IIH patients 18 years of age or younger with LSS treated with VSS at our institution. Included patients have fulminant disease course or are refractory or intolerant to medical management. Eight patients were identified; 4 males and 4 females. Mean age is 13.4 years (range 4-18). All patients had severe headaches, 5 had blurred vision, 3 had diplopia and 3 had pulsatile tinnitus. Papilledema was present in 4 patients. Three patients had prior surgical procedures. Four patients were intolerant to medical management, 3 were refractory and 1 had fulminant course. Cerebral venography demonstrated severe stenosis of the dominant sinus in 6 patients and of bilateral co-dominant sinuses in 2 patients. Six patients had intrinsic stenosis and 2 had extrinsic stenosis. Venous sinus stenting (VSS) resulted in improvement of symptoms, papilledema and normalization of CSF opening pressure in 7 patients. No immediate complications were observed. Mean follow-up period is 21 months (range 6-42). Two patients required re-stenting; one responded well and the other had persistent symptoms and underwent subsequent surgical procedures of CSF diversion, suboccipital decompression and duraplasty which were also ineffective. VSS may provide a viable option for pediatric IIH patients who are intolerant to medication, have failed conservative management or prior surgical interventions, or present with fulminant disease.

Reviewing the Recent Developments in Idiopathic Intracranial Hypertension.

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.

An update on idiopathic intracranial hypertension in adults: a look at pathophysiology, diagnostic approach and management.

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.

Exploring The Current Management Idiopathic Intracranial Hypertension, And Understanding The Role Of Dural Venous Sinus Stenting.

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.

Influence of optic nerve appearance on final visual outcome in pediatric patients with idiopathic intracranial hypertension (IIH)

In adult IIH patients, Frisén grade, but not optic disc hemorrhages (ODH) or cotton wool spots (CWS), was an independent risk factor for worse visual outcomes, allowing for early intervention. We investigated these variables in pediatric IIH (PIIH) patients.

Clinical features of pediatric idiopathic intracranial hypertension.

Introduction: Adult idiopathic intracranial hypertension (IIH) is often linked to obesity, however, causes of IIH in children are not well understood. This project identifies potential risk factors and features of pediatric IIH.Methods: This study consisted of a retrospective chart review of patients ages 5–17 years who were seen at a tertiary care children’s hospital and diagnosed with IIH. Diagnostic criteria included the presence of papilledema, normal neurological exam, normal neuroimaging, normal cerebrospinal fluid composition, and an opening pressure of a lumbar puncture >28 mmHg.Results: Of the 26 cases of IIH, 19 met all diagnostic criteria for this study, while seven patients were probable IIH, as they lacked papilledema. Intracranial pressure ranged from 28 to 66 mmHg, with a mean of 40.23 mmHg (±10.74). Overall, 50.0% (95% CI: 29.9–70.1%) of IIH patients were obese, with patients 12 years of age and younger exhibiting an overall obesity rate of 30.7% and patients 13 years of age and older having an obesity rate of 69.2%. The overall allergy rate in this IIH patient population was 46.2% (95% CI: 26.6–66.6%).Conclusion: Obesity appears to have no association with IIH in younger cases, but it is a more common feature in older children. An autoimmune component may play a role in pediatric IIH, given the high rate of atopy observed in this pediatric IIH patient cohort. Because a diagnosis of IIH can have an absence of optic nerve edema, taking a detailed history and performing a thorough examination are keys to diagnosing IIH in the pediatric population.

Novel advances in monitoring and therapeutic approaches in idiopathic intracranial hypertension.

Purpose of reviewThe current article appraises the recent developments in idiopathic intracranial hypertension (IIH), with particular attention to novel therapeutic avenues and advanced clinical assessment and monitoring with optical coherence tomography and telemetric intracranial pressure devices.Recent findingsThe incidence of IIH is increasing. The first consensus guidelines for IIH have been published detailing investigation and management algorithms for adult IIH. Improved understanding, clinical assessment and monitoring are emerging with the use of optical coherence tomography. Intracranial pressure telemetry is providing unique insights into the physiology of raised intracranial pressure in IIH. There are now an increasing number of ongoing clinical trials evaluating weight loss methods and novel targeted therapies, such as 11ß-HSD1 inhibition and Glucagon-like peptide 1 (GLP-1) receptor agonists.SummarySeveral studies are evaluating new therapies for IIH. Monitoring techniques are advancing, aiding diagnosis and allowing the clinician to accurately evaluate changes in papilloedema and intracranial pressure.

Idiopathic intracranial hypertension: adult versus pediatric patients

Idiopathic intracranial hypertension (IIH), also known as benign intracranial hypertension or pseudotumor cerebri, is characterized by increased intracranial pressure secondary to unknown causes. IIH affects both adults and children but predominately young obese females of child-bearing age. IIH gives rise to headache, visual disturbances, and symptoms of raised intracranial pressure. The pathophysiology of IIH remains elusive; postulations include obesity-related hormonal changes, obstruction of the central venous sinus, and hereditary causes. Diagnosing IIH is based on modified Dandy criteria. Diagnosis in children is challenging as symptoms and presentations vary; atypical and delayed presentations are not uncommon. Treatment for IIH includes oral acetazolamide or lumbar puncture; surgical treatment with shunts and optic nerve sheath fenestration is reserved for complicated and recurring cases. Treatment options for children are limited and associated with considerable risks and side effects. Vision loss is debilitating and occurs in 10% of IIH patients. A thorough and collaborative approach in managing IIH is needed. This study reviews the epidemiology, clinical presentation, diagnosis, and management of IIH in adults and pediatric patients.

Perspectives on diagnosis and management of adult idiopathic intracranial hypertension.

The purpose of this review is to shed light into the diagnostic challenges regarding idiopathic intracranial hypertension (IIH), giving more emphasis to the role of optical coherence tomography (OCT) in IIH, while it also presents recent advances in the management of IIH. A literature search in PubMed was performed for studies about the diagnosis and management of IIH published before June 30, 2017. Then, a comprehensive review of the eligible studies and the relevant references was performed. Apart from the updated modified criteria, OCT has been found to be important for the diagnosis of IIH, along with assessment of clinical signs and symptoms, brain imaging, and lumbar puncture. On the other hand, there is no current consensus for the management of IIH, but various interventions have been used, such as dietary therapy (weight loss, bariatric surgery, lifestyle modification, low-salt diet), medications, and surgical treatment. IIH is a challenging and serious disorder, which may lead to permanent visual impairment if there is a delay in its diagnosis. Advances in retinal imaging have led to better understanding of the pathogenesis of the disease and prompt diagnosis even in subtle cases can be done using OCT. Surgical interventions should be minimized and only used in cases refractory to medical treatment and in those with rapid progression.

Current concepts and strategies in the diagnosis and management of idiopathic intracranial hypertension in adults.

Since obesity has become an epidemic in industrialized nations, idiopathic intracranial hypertension (IIH) is now a more common neuro-ophthalmic disorder that causes visual loss and headaches. This review highlights the new diagnostic criteria for IIH and the new insights into the pathophysiologic mechanisms of IIH. Key diagnostic and monitoring techniques for papilledema include not only neuroimaging and the measurement of cerebrospinal fluid (CSF) pressure, but also perimetry, optical coherence tomography, and ocular sonography. The main findings of the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) support acetazolamide as the mainstay for medical therapy. CSF diversion procedures, endovascular venous sinus stenting, and optic nerve sheath fenestration are all surgical options when IIH is refractory to medical treatment or when it presents fulminantly. Future clinical trials comparing these procedures will help develop better paradigms in the surgical management of IIH.

Idiopathic intracranial hypertension: ongoing clinical challenges and future prospects.

Idiopathic intracranial hypertension (IIH) is an uncommon disorder characterized by increased intracranial pressure without radiological or laboratory evidence of intracranial pathology except empty sella turcica, optic nerve sheath with filled out cerebrospinal fluid spaces, and smooth-walled nonflow-related venous sinus stenosis or collapse. This condition typically affects obese women. The incidence of IIH is increasing with the rising prevalence of obesity. Persistent headache is the most common symptom. Visual impairment is a serious complication that may not be recognized by the patients. This paper reviews clinical manifestations, diagnostic challenges, and current treatments of IIH in adults. Various imaging modalities have been studied on their validity for detection of IIH and papilledema. This review also includes new studies on medical, surgical, and interventional management of this condition. Acetazolamide and topiramate are the only two medications that have been studied in randomized controlled trials about their efficacy in treatment of IIH. In patients who have severe visual impairment or progressive visual deterioration despite medical management, surgical or interventional treatment may be considered. The efficacy and complications of cerebrospinal fluid diversion, optic nerve sheath fenestration, and endovascular venous stenting reported in the last 3 decades have been summarized in this review. Finally, the prospective aspects of biomarkers and treatments are proposed for future research.

Surgically managed idiopathic intracranial hypertension in adults: a single centre experience.

Idiopathic intracranial hypertension (IIH) is a rare condition that is often managed conservatively. In patients with aggressive progression of the disease surgical options are considered. There are few data on the outcomes of these patients when surgically managed. We describe our experience of surgically managed IIH and the outcomes of these patients, in particular the surgical revision rate and interventions required for resolution of symptoms. A retrospective review of all patient files coded with benign intracranial hypertension, idiopathic intracranial hypertension or pseudotumour cerebri was undertaken. Files were searched with the date of diagnosis and the date these patients were referred for surgical intervention. The surgical interventions and complications were then documented and note was made of the number of inpatient admissions and days spent in hospital. From 2000-2013, 79 patients were identified as patients with IIH that had required surgical intervention; 52 % required further surgical intervention. The average number of surgical interventions was 5.6. For patients requiring further intervention the average number of surgical interventions was 8.6. On average patients with IIH also spent 42 inpatient days in neurosurgical beds, whilst those patients who required further intervention spent 63 days on average in neurosurgical beds. The length of the average individual admission was longer for patients requiring repeated surgical interventions. Based on our experience, patients that require surgical management of IIH frequently require further surgical interventions to control symptoms and manage complications of CSF diversion surgery. Those that require such further intervention on average will have six further operations and spend significantly longer in hospital. Lumboperitoneal (LP) shunting is an effective first line surgical intervention for 52 % of our patient cohort. This sub-group of patients therefore requires specialist neurosurgical input for this long-term and challenging pathological process.

Update on the Diagnosis and Treatment of Idiopathic Intracranial Hypertension

Idiopathic intracranial hypertension (IIH) is a rare disorder occurring more frequently in obese women of childbearing age, resulting in increased intracranial pressure (ICP) from an unknown cause. Recent advances in epidemiology, imaging, and treatment have provided a better understanding of IIH in recent years, with better identification of visual risk factors and atypical forms of IIH, including fulminant IIH and spontaneous cerebrospinal fluid leaks, and a randomized clinical trial providing the rationale for the use of acetazolamide. In addition, a revised version of the modified Dandy criteria for the diagnosis of IIH was suggested in 2013, with better definition of IIH in adults and children; however, controversy regarding nomenclature has precluded its acceptance among IIH experts. Finally, questions regarding the best surgical strategy, the indications for venous sinus stenting, and the diagnostic role of the radiologic findings commonly seen in IIH have remained unanswered.

EHMTI-0304. Headache determines quality of life in idiopathic intracranial hypertension

No previous studies have assessed quality of life (QoL) in idiopathic intracranial hypertension (IIH) associated with a therapeutic weight loss. Our previously published prospective cohort study confirmed that weight loss significantly reduced intracranial pressure (ICP) and treated chronic active adult IIH.

An Association between External Hydrocephalus in Infants and Reversible Collapse of the Venous Sinuses

The etiology of external hydrocephalus is usually ascribed to either a delay in maturation or obstruction of the arachnoid granulations, but the arachnoid granulations are absent in neonates. Venous outflow stenoses, similar to those seen in idiopathic intracranial hypertension (IIH), have been described in external hydrocephalus. A reversible collapse of the sinuses is known to operate in IIH, but collapsible sinuses have not been previously described in infants with external hydrocephalus. Three infants with external hydrocephalus had magnetic resonance venography at differing time points during their illness. The venous sinuses varied in size depending on the cerebrospinal fluid pressure similar to IIH in adults. External hydrocephalus may be analogous to IIH in adults.

Reply

Salpietro et al raise an interesting point about whether recent weight gain in addition to or in lieu of obesity per se is a risk factor for pediatric idiopathic intracranial hypertension. We were not able to examine this question in the current dataset. We are collecting prospective body mass index trajectories in our large pediatric cohort and plan to examine this question in future studies. Pediatric idiopathic intracranial hypertension and extreme childhood obesity: a role for weight gainThe Journal of PediatricsVol. 162Issue 5PreviewWe read with interest the report by Brara et al,1 who, in line with some findings of previous investigators on adult idiopathic intracranial hypertension (IIH), correlated extreme childhood obesity with the risk of developing pediatric IIH and the severity of symptoms, especially in postpubertal females. We wish to address an additional issue, however: the correlation between the timing and duration of obesity and the risk of developing IIH. Full-Text PDF

Hyperemic hydrocephalus: a new form of childhood hydrocephalus analogous to hyperemic intracranial hypertension in adults

In the majority of adults with idiopathic intracranial hypertension (IIH), there is an elevation in venous pressure associated with a venous outflow stenosis. In about 15% of IIH patients the elevated venous pressure is associated with an elevation in blood flow but little or no evidence of a stenosis. Venostenotic IIH and idiopathic hydrocephalus in children with a normal blood inflow have been shown to be equivalent. The aim of this study was to test whether children with hydrocephalus and an elevated arterial inflow have a vascular pathophysiology that is analogous to the hyperemic form of IIH in adults. Nine children with idiopathic hydrocephalus underwent MR imaging with flow quantification and were found to have arterial inflows 2 SDs above the mean for normal controls. Measurements of the head circumference, ventricular enlargement, total blood inflow, superior sagittal sinus (SSS)/straight sinus (SS) outflow, and the degree of collateral venous flow were performed. The results were compared with findings in 14 age-matched controls. In hyperemic hydrocephalus the cerebral blood inflow was elevated but the SSS and SS outflows were in the normal range. The sinus outflow as a percentage of the inflow was reduced by 8 percentage points in the SSS territory and 5 percentage points in the SS territory compared with findings in the controls (p = 0.04, p = 0.003, respectively), suggesting blood was returning via collateral channels. Similar to patients with hyperemic IIH, children with hyperemic hydrocephalus show a significant elevation in collateral venous flow, indicating that the same venous pathophysiology may be operating in both conditions.