Idiopathic intracranial hypertension: Clinical nosography and field-testing of the ICHD diagnostic criteria. A case-control study.

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.

Long-term visual outcome in a Danish population of patients with idiopathic intracranial hypertension.

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 Frisén 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 <0.0001) and by 0.55 at V2 (p-value <0.001). The most common type of VF defect at V0 was a nerve fibre layer defect (56.4%), and the second most common type was an enlarged blind spot (20.5%). There was no correlation between BCVA and VF defect type. Mean grade of papilloedema decreased from 2.2 at V0 to 0.5 at V2. The grade of papilloedema at V2 was not significantly related to the severity of papilloedema at V0 (p-values 0.65 and 0.48). CONCLUSION: Idiopathic intracranial hypertension (IIH) is associated with long-term loss of visual function, and relevant treatment strategies need to be improved.

Osmolality of Cerebrospinal Fluid from 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 is a serious condition in rapid growth]. / Idiopatisk intrakraniel hypertension er en alvorlig tilstand i hastig stigning.

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 is not benign: a long-term outcome study.

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.