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PURPOSE Idiopathic intracranial hypertension (IIH) is characterized by raised intracranial pressure (ICP), normal cerebrospinal composition and exclusion of alternative causes to increased ICP. The aim of this study was to evaluate long-term visual outcome in a Danish population of IIH patients. METHODS Retrospective chart review of 41 women diagnosed with IIH between June 2007 and March 2013. Best-corrected visual acuity (BCVA), colour vision, grade and type of visual field (VF) defects and grade of papilloedema according to the Modified Frisen Score were recorded at baseline visit (V0), 2-6 months (V1) and 13 months follow-up visit (V2) from time of diagnosis. RESULTS Best-corrected visual acuity (BCVA) was reduced in 25% of eyes at V0, in 10% at V1 and in 15% at V2. Colour vision was barely affected. Visual field (VF) was affected (>grade 0) in 87% of eyes at V0 and VF defect grade significantly improved by 0.58 at V1 (p-value

Idiopathic intracranial hypertension (IIH) is a challenging disorder with a rapid increasing incidence due to a close relation to obesity. The onset of symptoms is often insidious and patients may see many different specialists before the IIH diagnosis is settled. A summary of diagnosis, symptoms, headache characteristics and course, as well as existing evidence of treatment strategies is presented and strategies for investigations and management are proposed.

Idiopathic intracranial hypertension (IIH) primarily affects young obese females, and potentially causes visual loss and severe headache. The aim of this experiment is to examine relapse rate and long-term outcome in IIH patients. The methods involved in this experiment include a prospective controlled study of 18 newly diagnosed IIH patients followed for a mean observation period of 21.1 (±8.0) months. Treatment regime included diuretics, dietary recommendations and check-up visits at a dietician. Baseline and follow-up included neurological examination, detailed headache history and comprehensive neuro-ophthalmological examination, including fundus photography, Humphrey visual fields, and measurement of the retinal thickness (RT) and retinal nerve fiber layers (RNFL) by optical coherence tomography (OCT). Relapse was defined as recurrence of either: (1) papilledema or (2) symptoms and demonstrated raised ICP. The result of this experiment is that relapse was found in 28%. Visual function improved from baseline to follow-up and was generally favorable. In patients without relapse of papilledema RT and RNFL were significantly thinner than in healthy controls (p = 0.003 and 0.02), although atrophy was clinically detectable in only one patient. Headache was still present in 67% of the patients at follow-up. Headache was heterogenic and unrelated to relapse. After an initial reduction, weight increased again in the relapse group compared to reduced weight in the non-relapse group (p = 0.013). Thus, the conclusions drawn are that headache was persistent, difficult to classify, and equally represented in relapse and non-relapse patients. Headache was thus a poor marker of active disease. Relapse rate was high and clinically undetectable optic disc atrophy was discovered in apparently well treated IIH patients.

Objective To explore the extent and nature of cognitive deficits in patients with idiopathic intracranial hypertension (IIH) at the time of diagnosis and after 3 months of treatment. Design Prospective case–control study. Setting Neurological department, ophthalmological department and a tertiary headache referral clinic at a Danish university hospital. Participants 31 patients with definite IIH referred from June 2011 to February 2013 and included within 1 week of diagnostic intracranial pressure (ICP) measurement. 29 patients completed re-examination at the 3-month follow-up. At the time of testing, none of the patients took medication potentially affecting cognitive function. Controls were 31 healthy age-matched and sex-matched volunteers from the local community. Outcome measures Executive function, working memory, visuospatial memory, processing speed, attention and reaction time assessed by a comprehensive neuropsychological test battery consisting of validated computerised (Cambridge neuropsychological test automated battery) and paper-and-pencil tests. Results Patients with IIH performed significantly worse than controls in four of six cognitive domains (p≤0.02). Deficits were most pronounced in reaction time (1.45 SD below controls 95% CI 2.10 to 0.85) and processing speed (1.48 SD below controls 95% CI 2.08 to 0.81). Despite marked improvement in ICP and headache, re-examination showed persistent cognitive dysfunction 3 months after diagnosis and start of treatment. Conclusions We demonstrate for the first time in a well-defined cohort of patients that IIH may be associated with cognitive dysfunction. This could explain the functional disability of patients with IIH. A focused multidisciplinary approach including neuropsychological rehabilitation, therefore, might be relevant in the treatment of patients with IIH.

Background and purpose Our aim was to prospectively describe the course of headache during the first year of idiopathic intracranial hypertension (IIH). Methods Patients with newly diagnosed IIH were consecutively included from December 2010 to June 2013. Treatment according to standard guidelines was initiated. Headache history was obtained by headache diaries and standardized interviews performed at baseline and after 1, 2, 3 and 12 months. Parallel changes in papilledema were assessed by optical coherence tomography (OCT). All patients had comprehensive neuro-ophthalmological examinations including automated perimetry. Results Forty-four patients were included. Thirty-five patients completed the 12-month follow-up. Dramatic improvement in headache occurred within the first weeks after diagnosis. After 1 year, 15 patients reported no or only infrequent headache. However, 15 of the remaining 20 patients reported sustained chronic headache. Early age of onset and high diagnostic intracranial pressure (ICP) were associated with better headache outcome (≤1 headache days/month) after a year. Papilledema decreased rapidly within the first 2 months of diagnosis. After 1 year, OCT measures had normalized. Visual outcome was excellent in most patients. Conclusions Although headache in 43% of patients responded well to ICP management, sustained long-term headache was seen in the remaining patients, despite resolution of papilledema. Headache in IIH may thus be attributed to more complex mechanisms than ICP elevation alone. High ICP and young age were associated with better headache outcome. Early treatment according to standard guidelines seems sufficient to ensure excellent visual outcome in the vast majority of patients.

Aims The aims of this article are to characterize the headache in idiopathic intracranial hypertension (IIH) and to field-test the ICHD diagnostic criteria for headache attributed to IIH. Materials and methods We included 44 patients with new-onset IIH. Thirty-four patients with suspected but unconfirmed IIH served as controls. Headache and other IIH-related symptoms were assessed by a detailed standardized interview. In participants referred before diagnostic lumbar puncture ( n = 67), we recorded headache intensity before and after cerebrospinal fluid (CSF) withdrawal. Results Headache in patients with IIH was daily occurring in 86%, focal in 84% and pulsating in 52%. Aggravation of headache by coughing or straining and relief after CSF withdrawal were significantly more frequent in patients than in controls ( p ≤ 0.002). From the ICHD-2 to the ICHD-3 beta classification the sensitivity increased from 60% to 86% whereas the specificity decreased from 86% to 53%. Based on our data the headache criteria can be revised to increase sensitivity to 95% and specificity to 65%. Conclusion Aggravation of headache by coughing or straining, relief after CSF withdrawal, retrobulbar pain and pulsatile tinnitus may suggest intracranial hypertension. Based on data from a well-defined IIH cohort, we propose a revision of the ICDH-3 beta diagnostic criteria with improved clinical applicability and increased sensitivity and specificity.

Background Idiopathic intracranial hypertension (IIH) is a condition of raised intracranial pressure (ICP) in the absence of space-occupying lesions or other known etiology. It primarily affects young obese females, and potentially causes permanent visual loss due to papilledema and secondary optic atrophy. The aim of this study was to evaluate the diagnostic value of optical coherence tomography (OCT) as a marker for CSF opening pressure in patients with idiopathic intracranial hypertension (IIH).

Introduction Idiopathic intracranial hypertension (IIH) is a disorder of increased intracranial fluid pressure (ICP) of unknown etiology. This study aims to investigate osmolality of cerebrospinal fluid (CSF) from patients with IIH. Methods We prospectively collected CSF from individuals referred on suspicion of IIH from 2011–2013. Subjects included as patients fulfilled Friedman and Jacobson’s diagnostic criteria for IIH. Individuals in whom intracranial hypertension was refuted were included as controls. Lumbar puncture with ICP measurement was performed at inclusion and repeated for patients after three months of treatment. Osmolality was measured with a Vapor Pressure Osmometer. Results We collected 90 CSF samples from 38 newly diagnosed patients and 28 controls. At baseline 27 IIH-samples and at 3 months follow-up 35 IIH-samples were collected from patients. We found no significant differences in osmolality between 1) patients at baseline and controls (p = 0. 86), 2) patients at baseline and after 3 months treatment (p = 0.97), and 3) patients with normalized pressure after 3 months and their baseline values (p = 0.79). Osmolality in individuals with normal ICP from 6–25 cmH2O (n = 41) did not differ significantly from patients with moderately elevated ICP from 26–45 cmH2O (n = 21) (p = 0.86) and patients with high ICP from 46–70 cmH2O (n = 4) (p = 0.32), respectively. There was no correlation between osmolality and ICP, BMI, age and body height, respectively. Mean CSF osmolality was 270 mmol/kg (± 1 SE, 95% confidence interval 267–272) for both patients and controls. Conclusions CSF osmolality was normal in patients with IIH, and there was no relation to treatment, ICP, BMI, age and body height. Mean CSF osmolality was 270 mmol/kg and constitutes a reference for future studies. Changes in CSF osmolality are not responsible for development of IIH. Other underlying pathophysiological mechanisms must be searched.

Idiopathic intracranial hypertension (IIH) is a disease of unknown aetiology. IIH typically affects young obese females. The majority of the cases are complicated by papilloedema, visual loss and significant headache. Weight loss is, in addition to medical therapy, the mainstay of IIH management. Medical therapy is currently solely based on clinical experience. Malignant cases with rapid visual deterioration may require immediate surgical treatment in order to prevent irreversible visual loss. Early identification is essential, and increased awareness of IIH is important.

Idiopathic intracranial hypertension (IIH) is a condition of raised intracranial pressure (ICP) in the absence of space-occupying lesions or other known etiology. It primarily affects young obese females, and potentially causes permanent visual loss due to papilledema and secondary optic atrophy. The aim of this study was to evaluate the diagnostic value of optical coherence tomography (OCT) as a marker for CSF opening pressure in patients with idiopathic intracranial hypertension (IIH). We conducted a case-control study of 20 newly diagnosed, 21 long-term IIH patients, and 20 healthy controls. Investigations included measurement of peripapillary retinal nerve fiber layer thickness (RNFLT) and total retinal thickness (RT), automated visual field testing, and measurement of CSF opening pressure. An OCT elevation diagram was developed as a new diagnostic tool. The diagnostic ability of OCT as a marker of increased ICP (> 25 cmH2O) was investigated using multiple regression and receiver operating characteristic (ROC) curves. OCT elevation diagrams showed that in 60 % of patients newly diagnosed with IIH and in 10 % of patients with long-term IIH, 50 % or more of the OCT scans (RT and RNFLT) were above normal. The percentage of abnormal OCT scans was significantly associated with increased ICP (p

Idiopathic intracranial hypertension (IIH) is a condition of yet unknown aetiology affecting predominantly obese females of childbearing age. IIH is a diagnosis of exclusion as raised cerebrospinal fluid pressure may occur secondary to numerous other medical conditions. An atypical phenotype or an atypical disease course should alert the physician to reevaluate a presumed IIH-diagnosis. The authors report a case of a 32-year-old non-obese male with intracranial hypertension, secondary to a syphilitic central nervous system infection, initially misdiagnosed as being idiopathic. Upon relevant antibiotic treatment, signs and symptoms of elevated intracranial pressure resolved completely. Syphilis is a rare, but very important, differential diagnosis that in this case was clinically indistinguishable from IIH.

Idiopathic Intracranial Hypertension (IIH) primarily affects young obese females, and potentially causes visual loss and severe headache.