Diagnosis Of Idiopathic Intracranial Hypertension Research Articles

Overdiagnosis of idiopathic intracranial hypertension.

To delineate the factors contributing to overdiagnosis of idiopathic intracranial hypertension (IIH) among patients seen in one neuro-ophthalmology service at a tertiary center. We retrospectively reviewed new patients referred with a working diagnosis of IIH over 8 months. The Diagnosis Error Evaluation and Research taxonomy tool was applied to cases referred with a diagnosis of IIH and a discrepant final diagnosis. Of 1,249 patients, 165 (13.2%) were referred either with a preexisting diagnosis of IIH or to rule out IIH. Of the 86/165 patients (52.1%) with a preexisting diagnosis of IIH, 34/86 (39.5%) did not have IIH. The most common diagnostic error was inaccurate ophthalmoscopic examination in headache patients. Of 34 patients misdiagnosed as having IIH, 27 (27/34 [79.4%]; 27/86 [31.4%]) had at least one lumbar puncture, 29 (29/34 [85.3%]; 29/86 [33.7%]) had a brain MRI, and 8 (8/34 [23.5%]; 8/86 [9.3%]) had a magnetic resonance/CT venogram. Twenty-six had received medical treatment, 1 had a lumbar drain, and 4 were referred for surgery. In 8 patients (8/34 [23.5%]; 8/86 [9.3%]), an alternative diagnosis requiring further evaluation was identified. Diagnostic errors resulted in overdiagnosis of IIH in 39.5% of patients referred for presumed IIH, and prompted unnecessary tests, invasive procedures, and missed diagnoses. The most common errors were inaccurate ophthalmoscopic examination in headache patients and thinking biases, reinforcing the need for rapid access to specialists with experience in diagnosing optic nerve disorders. Indeed, the high prevalence of primary benign headaches and obesity in young women often leads to costly and invasive evaluations for presumed IIH.

Role of Lumbopleural Shunt in Management of Idiopathic Intracranial Hypertension

Idiopathic intracranial hypertension (IIH) denotes the condition of increased intracranial pressure without a clear underlying pathologic condition of the brain. The treatment plan should be conducted to save vision. Treatment options include medications, serial lumbar punctures, and surgical intervention. Surgery is indicated once visual loss continues despite optimum medical therapy. This is a prospective study carried out during a period of 2 years. Cases were those who fulfilled the modified Dandy criteria for the diagnosis of IIH. All cases experienced a previously failed lumboperitoneal shunt. This study was approved by The Ethical Committee of Mansoura Faculty of Medicine. The following data were gathered for analysis: age, sex, presenting symptoms, number of shunt failures, apparent causes of failure, cerebrospinal fluid opening pressure on lumbar puncture, visual acuity before surgery, operative time, visual acuity at 3 and 6 months and 1 year, and any procedure-related or device-related complication. Our study included 12 patients with lumboperitoneal shunt failure, all of which were women with mean age of 33 years. The major presenting symptom was headache. The main cause of failure was shunt migration (n= 10, 83.3%). Mean cerebrospinal fluid opening pressures was 37 cmH2O. The mean operative time was 42.5 minutes. The lumbopleural shunt is a potentially effective technique in terms of symptoms control and vision improvement in treatment of IIH. The technique is safe, less time-consuming, and more suitable for morbid obese patients with high body fat percentages.

Sonographic assessment of the optic nerve sheath diameter in the diagnosis of idiopathic intracranial hypertension

Objective: Sonographic assessment of the optic nerve sheath diameter (ONSD) is a useful technique in detecting raised intracranial pressure (ICP) in neurocritical care patients. Its utility in idiopathic intracranial hypertension (IIH) is less known. The aim of this study was to evaluate the diagnostic accuracy of ONSD for detecting IIH.Material and methods: Ultrasound measurement of ONSD was performed in 19 patients with IIH and in 11 patients with different neurological diseases without raised ICP that required undergoing a lumbar puncture. The validity of this technique for diagnosing IIH was established with cerebrospinal fluid manometry values.Results: Patients with IIH showed significantly enlarged ONSD than those without IIH. The best cut-off point for detecting raised ICP was 6.3 mms, with a sensitivity, specificity and positive likelihood ratio of 94.7%, 90.9% and 10.4, respectively. After a therapeutic lumbar puncture an 87% of cases had a partial reduction of ONSD values.Conclusion: Sonographic assessment of ONSD seems to be a useful and reliable technique for detecting raised ICP. While the spinal manometry is not replaced in usual clinical settings, transorbital sonography alternatively allows a suitable and harmless screening of patients with suspected IIH. It would be desirable to perform an internal validation of the technique in each hospital in order to get the optimal cut-off point.

Brain Imaging in Idiopathic Intracranial Hypertension.

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 the imaging findings reported in IIH and discusses their possible roles in the pathophysiology and the diagnosis of IIH. References were identified by searches of PubMed from 1955 to January 2015, with the terms "idiopathic intracranial hypertension," "pseudotumor cerebri," "intracranial hypertension," "benign intracranial hypertension," "magnetic resonance imaging," "magnetic resonance venography," "computed tomography (CT)," "CT venography," "imaging," and "cerebrospinal fluid (CSF) leak." Additional references were identified by hand search of relevant articles. When possible, we extracted the number of patients and control subjects from each study for each radiological finding. When at least 2 studies used the same criteria to define a radiological finding, all patients from these studies were pooled to obtain a mean sensitivity and specificity with 95% confidence interval. Specific neuroimaging findings may suggest long-standing IIH, including empty sella, flattening of the posterior globes, optic nerve head protrusion, distention of the optic nerve sheaths, tortuosity of the optic nerve, cerebellar tonsillar herniation, meningoceles, CSF leaks, and transverse venous sinus stenosis. Although IIH remains a diagnosis of exclusion, the most recently proposed diagnostic criteria have included neuroimaging findings to suggest IIH when major diagnostic criteria are not fulfilled. However, these findings are not diagnostic of IIH, and their presence is not required for the diagnosis of definite IIH. Their incidental discovery on brain imaging should not prompt invasive procedures, unless other signs of IIH, such as papilledema, are present.

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.

P027. Idiopathic intracranial hypertension without papilledema in refractory chronic daily headache.

Background A diagnosis of idiopathic intracranial hypertension without papilledema (IIHWOP) should be considered in unresponsive chronic daily headache (CDH) patients [1]. A CSF opening pressure (OP) above 200 mm H2O has been detected in chronic migraine patients with conflicting result, ranging from 10% to 86% of patients [1,2]. Moreover, controversies exist regarding the OP cut-off value greater than 200 or 250 mm H2O and the role of transverse sinus stenosis (TSS) [3,4].

Are shunt series and shunt patency studies useful in patients with shunted idiopathic intracranial hypertension in the emergency department?

ObjectivesShunt series and shunt patency studies can be performed in the emergency department (ED) to evaluate for shunt malfunction in patients with idiopathic intracranial hypertension (IIH). Here, we examine the utility of these studies in this specific patient population. MethodsWe retrospectively reviewed the ED visits of all shunted patients diagnosed with IIH from 2003 to 2014. ED visits for symptoms not related to the patient's IIH were excluded from the study. Collected variables included demographics, symptoms, IIH diagnosis and treatment history, imaging findings, and management changes. ResultsTwenty-five (81%) patients had a total of 105 visits involving a shunt series, with four (3.9%) showing problems with the catheter. The majority of shunt series (n=101, 96%) showed no catheter pathology. Based on results of the shunt series alone, in 3 instances, management changes in the form of shunt revision or shunt reprogramming occurred. Of the 105 visits with a shunt series, 17 (16%) resulted in a change in management as compared to 12 out of 66 (18%) visits without a shunt series (p=0.83). Nine patients had a total of 10 visits involving a shunt patency study: five were normal, four were abnormal, and one was inconclusive. Based on findings on the shunt patency study alone, changes in management leading to shunt adjustment or revision occurred in 4 instances. Of the 10 visits with shunt patency studies, 5 resulted in a change in management as compared to 24 out of 161 visits without a shunt patency study (p=0.014). ConclusionsShunt series detected catheter pathology only 3.9% of the time, and there was no difference in the rate of management changes between those patients who underwent a shunt series and those who did not. There was a significant difference in the rate of management changes in patients who received shunt patency studies as compared to those who did not. Shunt series may not be a useful screening tool to be used universally to diagnose shunt malfunction in IIH patients in the ED, and should be utilized when there is concern for impending visual loss. Shunt patency studies should be reserved for patients with inconclusive diagnostic imaging and clinical findings to decide whether to proceed to surgical exploration and revision.

Idiopathic Intracranial Hypertension in the Northwest of Northern Ireland: Epidemiology and Clinical Management

Background: There is limited literature on the epidemiology of idiopathic intracranial hypertension (IIH). The diagnosis and management of IIH require a multidisciplinary approach. We sought to study the incidence as well as prevalence of IIH and to evaluate the current management of IIH in the northwest of Northern Ireland. Methods: Medical records of patients diagnosed with IIH between 2007 and 2014 in a general hospital in Northern Ireland were reviewed. Clinical and outcome data were retrieved. Results: There were 45 patients with IIH, 44 women: 1 man. The mean age at presentation was 29.4 (SD 9.8) years and mean body mass index (BMI) 39.8 (SD 9.5) kg/ m². All patients had neuroimaging, 44 (98%) had CT/MR venogram and 41 (91%) had visual perimetry. The crude incidence of IIH was 2.36 per 100,000 (95% CI 1.65-3.37). For women, the incidence was 4.65 per 100,000/year (95% CI 3.25-6.66). The prevalence was 14.3 per 100,000 overall (95% CI 9.72-20.9) but 28.1 per 100,000 in women (95% CI 19.2-41.2). Visual field defects were identified in 25 of 41 (61%); 4 patients (9%) required shunting procedures. At follow-up, the mean BMI decreased by 1.6 kg/m² (p = 0.024). Conclusions: The incidence of IIH in the northwest of Northern Ireland is among the highest ever reported and probably reflects the known increase in obesity.

The Photopic Negative Response in Idiopathic Intracranial Hypertension.

To evaluate the photopic negative response (PhNR) as an index of retinal ganglion cell (RGC) function in idiopathic intracranial hypertension (IIH). Amplitude and implicit time of the PhNR, as elicited by full-field, brief-luminance flashes, was measured in IIH (n = 10) and visually normal control (n = 15) subjects. Visual function was assessed in IIH subjects using standard automated perimetry mean deviation (SAP-MD) scores. Optic nerve structure was evaluated using the Frisén papilledema grading scale (FPG). Macula ganglion cell complex volume (GCCV) was extracted from optical coherence tomography images to assess RGC loss. Median PhNR amplitude was significantly lower in IIH subjects compared with control subjects (P = 0.015, Mann-Whitney Rank Sum [MW]), but implicit time was similar (P = 0.54, MW). In IIH subjects, PhNR amplitude and SAP-MD were correlated (Pearson's r = 0.78, P = 0.008). Ganglion cell complex volume was correlated with both SAP-MD (r = 0.72, P = 0.019) and PhNR amplitude (r = 0.77, P = 0.009). Multivariate linear regression models demonstrated that the correlation between GCCV and PhNR amplitude was improved by accounting for FPG in the model (r = 0.94, P < 0.0001), but the correlation between GCCV and SAP-MD was not (r = 0.74, P = 0.009). Photopic negative response amplitude, which can be decreased in IIH subjects, correlates well with a clinical measure of visual function (SAP-MD). In multivariate models, it correlated with both an imaging measure of chronic ganglion cell injury (GCCV) and a clinical measure of acute optic nerve head pathology (FPG). Further studies are needed to determine the clinical utility of PhNR as a marker for diagnosis and monitoring of IIH.

Outcomes and Experience with Lumbopleural Shunts in the Management of Idiopathic Intracranial Hypertension

To report five patients who underwent lumbopleural (LPl) shunting for the treatment of idiopathic intracranial hypertension (IIH) and to describe the considerations, complications, and outcomes related to this rarely described procedure. The clinical data of five patients treated with LPl shunting over a 23-year period were retrospectively analyzed. Factors including the age at diagnosis of IIH, age at time of LPl shunting, body mass index (BMI), reason for LPl shunt placement, number of revisions before LPl shunt placement, valve type, time to first revision, presence of overdrainage and its management, complications and their management, survival time of LPl shunt, and clinical course at last follow-up were analyzed. All patients were morbidly obese females with an average of 4.6 shunt revisions before an LPl shunt. The average overall survival time of the LPl shunt was 48 months. Two patients experienced failure of their LPl shunts with subsequent replacement within the first year. Four patients experienced complications related to shunt overdrainage, requiring placement of an antisiphon device (ASD) or additional valve. One patient developed a symptomatic pleural effusion, and one patient developed a small pneumothorax, which was managed conservatively. LPl shunting, though rarely used, is a viable option in the treatment of IIH refractory to standard peritoneal shunting. When pursuing this treatment, a valve and ASD are recommended to mitigate the risks of overdrainage and pleural effusion. Chest imaging should be obtained if the patient becomes symptomatic but can be deferred if the patient remains asymptomatic and is doing well.

Uncommon presentation of idiopathic intracranial hypertension

A 51-year-old gentleman, a known hypertensive on medications presented with transient visual disturbance in his left eye. Examination revealed the presence of unilateral optic disc edema. A lumbar puncture revealed elevated opening pressure with normal cerebrospinal fluid biochemical parameters. Neuroimaging showed no evidence for an alternative cause for intracranial hypertension thus confirming the diagnosis of idiopathic intracranial hypertension (IIH). IIH can rarely present with a unilateral papilledema. This emphasizes the need to meticulously examine both optic fundi in all patients with suspected intracranial hypertension. Early diagnosis of this condition can help in the prevention of permanent visual loss. We hereby report the second case of IIH presenting with a unilateral papilledema in Indian literature.

Elevated Intracranial Pressure Diagnosis with Emergency Department Bedside Ocular Ultrasound.

Bedside sonographic measurement of optic nerve sheath diameter can aid in the diagnosis of elevated intracranial pressure in the emergency department. This case report describes a 21-year-old female presenting with 4 months of mild headache and 2 weeks of recurrent, transient binocular vision loss. Though limited by patient discomfort, fundoscopic examination suggested the presence of blurred optic disc margins. Bedside ocular ultrasound (BOUS) revealed wide optic nerve sheath diameters and bulging optic discs bilaterally. Lumbar puncture demonstrated a cerebrospinal fluid (CSF) opening pressure of 54 cm H2O supporting the suspected diagnosis of idiopathic intracranial hypertension. Accurate fundoscopy can be vital to the appropriate diagnosis and treatment of patients with suspected elevated intracranial pressure, but it is often technically difficult or poorly tolerated by the photophobic patient. BOUS is a quick and easily learned tool to supplement the emergency physician's fundoscopic examination and help identify patients with elevated intracranial pressure.

Clinical Consequences of the Revised Diagnostic Criteria for the Diagnosis of Idiopathic Intracranial Hypertension

Clinical Consequences of the Revised Diagnostic Criteria for the Diagnosis of Idiopathic Intracranial Hypertension

Medication overuse headache – An under-diagnosed problem in shunted idiopathic intracranial hypertension patients

Purpose. Management of headache in a subgroup of shunted idiopathic intracranial hypertension (IIH) patients is a well- described and difficult task. We present our series of shunted IIH patients with medication overuse headache (MOH) and discuss the role of careful pain management in this group of patients. Materials and methods. A retrospective review of shunted IIH patients with headache, who had their shunt function assessed by monitoring their intracranial pressure and were subsequently diagnosed with MOH. Results. A total of 15 patients were identified. The mean time between the diagnosis of IIH and the diagnosis of medication overuse headache was 6 years (standard deviation 4.9, range 2–18 years). The majority of patients in this group (12/15) had undergone multiple shunt revisions. Conclusions. Medication overuse headache in shunted IIH patients can lead to the requirement of hospital admission, investigations and procedures. We suggest that opiates and non-steroidal anti-inflammatory analgesics (NSAIDs) are used for a specified duration post-operatively and reviewed regularly. We suggest that patients are counselled regarding medication overuse headache in a multidisciplinary setting.

What is the best imaging modality in evaluating patients with unilateral pulsatile tinnitus?

What is the best imaging modality in evaluating patients with unilateral pulsatile tinnitus?

The incidence of idiopathic intracranial hypertension in Israel from 2005 to 2007: results of a nationwide survey

Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is a disorder related to increased intracranial pressure without clinical, laboratory or radiological evidence of an intracranial space occupying lesion or cerebral sinus vein thrombosis, predominantly affecting obese women of childbearing age. Our aim was to determine the incidence and clinical features of IIH in Israel. In a cross-sectional study, all medical records of patients discharged from the hospital with a primary diagnosis of IIH during 2005-2007 were reviewed. Four hundred and twenty-eight patients with a new onset of IIH were diagnosed. The average annual incidence rate was 2.02 per 100,000 with an incidence of 3.17 per 100,000 for women and 0.85 per 100,000 for men. The incidence rate in females of childbearing age (18-45) was 5.49 per 100,000. The female to male ratio for >17 years old was 6.1:1 (252 females and 41 males) and 2.1:1 (60 females and 28 males) for ages 11-17. Obesity was documented in 83.4% of patients. Body mass index (BMI) data were available for 159 (37.1%) patients; of these, 59.1% had a BMI ≥ 30. The incidence of IIH in Israel has increased during the last decade. This finding could be related to the increasing rates of obesity. The association of IIH and obesity should be further explored especially with regard to the effect of weight reduction for primary prevention.

Idiopathic intracranial hypertension in pregnancy

Key content Idiopathic intracranial hypertension is a rare condition that usually affects overweight women. It is a diagnosis of exclusion in a pregnant woman presenting with headache. It is important to understand the medical and surgical treatment options in pregnancy. The mode of delivery is usually decided by obstetric factors. The risk of visual impairment is the same in pregnant and non‐pregnant women with idiopathic intracranial hypertension. Learning objectives To identify how idiopathic intracranial hypertension presents in pregnancy. To know how to monitor and manage women with this condition during pregnancy. To understand the intrapartum, postpartum and long‐term implications of this condition. Ethical issues What is the extent of investigation before reaching the diagnosis of idiopathic intracranial hypertension? Do we need a high index of suspicion in all pregnant women presenting with headache? What is the preferred mode of delivery in idiopathic intracranial hypertension – do they all need a caesarean section?

Pseudopapilledema and association with idiopathic intracranial hypertension

Diagnosing idiopathic intracranial hypertension (IIH), or pseudotumor cerebri, can be challenging in children. Diagnosis is based on lumbar puncture, opening pressures, and appearance of the optic disk. Misdiagnosis of papilledema, a typical finding, may lead to unnecessary treatments and procedures. We report 52 children over a 6-year period to better identify the true incidence of pseudopapilledema and other factors that may confound the diagnosis of IIH. A retrospective chart review approved by the Institutional Review Board was performed. Fifty-two children under the age of 21 referred to us based on suspected IIH or papilledema from 2007 to 2013 are included in this study. Patients were assessed by a pediatric ophthalmologist and a neurosurgeon. Fifty-two children were initially diagnosed with IIH and/or papilledema; 26 diagnoses were revised to pseudopapilledema after pediatric ophthalmological review. Out of those 26 patients with pseudopapilledema, 14 had undergone lumbar punctures, 19 had MRIs, 9 had CTs, and 12 were taking medications-these medications were discontinued upon revision of the diagnoses. The difference in the CSF opening pressure between children diagnosed with true IIH (32.7cm H2O) and children diagnosed with pseudopapilledema (24.7cm H2O) was statistically significant. IIH diagnosis is heavily reliant on the appearance of the optic disk. Pediatric ophthalmological assessment is essential to carefully examine the optic disk and prevent further unnecessary investigation and treatments. Close communication between pediatricians, ophthalmologists, and neurosurgeons can avoid invasive procedures for children who do have pseudopapilledema, and not IIH or associated papilledema.

Idiopathic Intracranial Hypertension: Pseudotumor Cerebri

Idiopathic intracranial hypertension (IIH) is most often diagnosed in young obese females of childbearing years. The diagnosis is made based on the modified Dandy criteria and the exclusion of alternate causes of raised intracranial pressure. The focus of this review is to provide an overview of the diagnosis and treatment options for patients with IIH. There are long-term consequences for patients experiencing IIH, with visual loss being the most serious. We conclude that the diagnosis of IIH is not usually difficult. An ophthalmologic examination is essential in patients with IIH to monitor visual function. A neurologist or neurosurgeon may be needed at some point for medical and/or surgical intervention.

Continuous 24-Hour Intraocular Pressure Pattern Monitoring in Medically Treated Normal Tension Glaucoma

The typical presenting ocular signs of idiopathic intracranial hypertension (IIH) include bilateral papilledema, enlarged blind spots, and abducens nerve palsy. Unilateral papilledema with choroid folds is an atypical presenting sign of IIH. The authors report the case of a 55-year-old Chinese gentleman, of medium-built, presenting with right eye transient visual obscuration associated with tinnitus for 2 weeks. Physical examination revealed ipsilateral choroidal folds and disc swelling. Computerized tomography of the brain and orbit with contrast revealed no intracranial space occupying lesions. Lumber puncture revealed an elevated opening cerebrospinal fluid pressure (30 cmH₂O) with normal biochemistry. Subsequent contrast Magnetic Resonance Imaging of the brain and orbit with venogram revealed features of empty sella, compatible with the diagnosis of IIH. He was subsequently treated with oral acetazolamide for 6 months and the symptoms resolved. Unilateral papilloedema with choroidal folds is an uncommon presentation of IIH, a lumbar puncture with opening pressure measurement and proper neuro-imaging should be considered when there is suspicion of IIH