Idiopathic Intracranial Hypertension Patients Research Articles

Topiramate is more effective than acetazolamide at lowering intracranial pressure.

BackgroundThe management of idiopathic intracranial hypertension focuses on reducing intracranial pressure to preserve vision and reduce headaches. There is sparse evidence to support the use of some of the drugs commonly used to manage idiopathic intracranial hypertension, therefore we propose to evaluate the efficacy of these drugs at lowering intracranial pressure in healthy rats.MethodsWe measured intracranial pressure in female rats before and after subcutaneous administration of acetazolamide, topiramate, furosemide, amiloride and octreotide at clinical doses (equivalent to a single human dose) and high doses (equivalent to a human daily dose). In addition, we measured intracranial pressure after oral administration of acetazolamide and topiramate.ResultsAt clinical and high doses, subcutaneous administration of topiramate lowered intracranial pressure by 32% (p = 0.0009) and 21% (p = 0.015) respectively. There was no significant reduction in intracranial pressure noted with acetazolamide, furosemide, amiloride or octreotide at any dose. Oral administration of topiramate significantly lowered intracranial pressure by 22% (p = 0.018), compared to 5% reduction with acetazolamide (p = >0.999).ConclusionOur in vivo studies demonstrated that both subcutaneous and oral administration of topiramate significantly lowers intracranial pressure. Other drugs tested, including acetazolamide, did not significantly reduce intracranial pressure. Future clinical trials evaluating the efficacy and side effects of topiramate in idiopathic intracranial hypertension patients would be of interest.

Sonographic assessment of optic nerve and ophthalmic vessels in patients with idiopathic intracranial hypertension

ABSTRACTBackground: Early diagnosis and proper monitoring of intracranial pressure (ICP) in idiopathic intracranial hypertension (IIH) could reduce morbidity.Objectives: The objective was to explore and monitor reflection of raised ICP in IIH on optic nerve sheath diameter (ONSD), papillary height and ophthalmic vessels hemodynamics, using transorbital sonography (TOS).Methods: The study included 24 IIH patients and 30 controls. Patients were compared to controls (phase I) then reassessed twice; 1 week and 4 weeks later (phase II). Both groups underwent clinical evaluation and TOS to measure ONSD, papillary elevation, and color Doppler indices of the ophthalmic vessels. Patients underwent lumbar puncture (LP) to measure cerebrospinal fluid (CSF) pressure.Results: ONSD was significantly higher in patients compared to controls (p < 0.001). The cut-off value was 6.2 mm. Papillary elevation (p = 0.006) and ONSD (p = 0.006) were significantly reduced 4 weeks following LP. Baseline color Doppler indices of the ophthalmic vessels were comparable between both groups and the changes observed during the follow-up visits in the patients were insignificant.Conclusion: Reflected ICP changes on ONSD and papilla, measured by TOS, could be a valuable noninvasive additional tool to diagnose and monitor IIH patients. IIH insignificantly influences ophthalmic vessels hemodynamics.Abbreviation BMI: Body mass index. CSF: Cerebrospinal fluid. EDV: End diastolic velocity. ICP: Intracranial pressure. IH:intracranial hypertension. IIH: Idiopathic intracranial hypertension. LP: Lumbar puncture. MI: Mechanical index. MRI: Magnetic resonance imaging. MRV: Magnetic resonance venography. OA: Ophthalmic artery OND: Optic nerve diameter. ONSD: Optic nerve sheath diameter. OV: Ophthalmic vein. PIs: Pulsatility indices. PSV: Peak systolic velocity. ROC: Receiver operator characteristic. TOS: Trans-orbital sonography.

Contrast enhanced magnetic resonance venography in the follow-up evaluation of idiopathic intracranial hypertension patients with cerebral venous sinus stenting

PurposeRole of contrast-enhanced magnetic resonance venography (CE-MRV) in the follow-up of venous sinus stenting (VSS) among the idiopathic intracranial hypertension (IIH) patients. Materials and methodsProspective evaluation of VSS patients with CE-MRV, DRCV and DSA for follow-up of clinically suspected recurrent stenosis. CE-MRV was evaluated against DRCV and DSA. ResultsTen patients with twelve episodes of recurrent symptoms. Sensitivity, specificity, PPV, NPV and accuracy of the CE-MRV for the detection of recurrent stenosis were: 100%, 33.33%, 81.82%, 100% and 83.3% respectively. ConclusionCE-MRV was a reliable first-line investigation for the detection of recurrent stenosis following VSS.

Characterising the patient experience of diagnostic lumbar puncture in idiopathic intracranial hypertension: a cross-sectional online survey

ObjectivesPatients with idiopathic intracranial hypertension (IIH) usually require multiple lumbar punctures (LPs) during the course of their disease, and often report significant morbidity associated with the procedure. The aim of...

Neurophysiologic tests screening cognitive impairment in idiopathic intracranial hypertension patients

BackgroundIdiopathic intracranial hypertension (IIH) is a disorder with increased intracranial pressure of obscure cause. Patients with IIH may suffer from difficulty in thinking or concentrating. This work aimed at highlighting the neurophysiologic suggestions of cognitive impairment in IIH patients.MethodsTwenty patients with IIH—and a similar number of matched control subjects—were examined in this case–control study. The P300 and contingent negative variation (CNV) were performed. Results from both groups were compared.ResultsThere were significant lower means of P300 amplitude and CNV amplitude (early and late response) in patients than in controls. Also, there were significant delayed latencies of P300 and CNV in patients than in normal control subjects. Finally, P300 latency was correlated to mini-mental state examination.ConclusionsWe concluded that cognitive affection in IIH is well appreciated at neurophysiologic levels and is related to clinical inputs. We are providing a suggestion of the significant relation between clinical screening (i.e., mini-mental state examination) and NP screening (i.e., P300) of cognitive functions.

Novel anti-neuronal antibodies in idiopathic intracranial hypertension patients (P3.102)

Novel anti-neuronal antibodies in idiopathic intracranial hypertension patients (P3.102)

Effect of Body Mass Index on Venous Sinus Pressures in Idiopathic Intracranial Hypertension Patients Before and After Endovascular Stenting.

Elevated body mass index (BMI) has been correlated with worse outcomes after treatment for idiopathic intracranial hypertension (IIH). Venous sinus stenting (VSS) has emerged as a safe and effective treatment for a subset of patients with IIH and evidence of venous sinus stenosis. However, the association between BMI and the efficacy of VSS remains poorly characterized. To determine, in a retrospective cohort study, the effect of BMI on preoperative mean intracranial venous pressure (MVP) and post-VSS outcomes. We performed a retrospective evaluation of a prospectively collected database of patients with IIH and intracranial venous sinus stenosis who underwent VSS. Patient demographics and treatment factors, including pre- and postprocedural trans-stenosis pressure gradients, were analyzed to identify the relationship between BMI and outcomes after VSS. Increasing BMI was significantly correlated with higher maximum MVP (P = .013) and higher trans-stenosis pressure gradient (P = .043) prior to treatment. The degrees of improvement in maximum MVP and pressure gradient after VSS were greatest for obese and morbidly obese patients (BMI > 30 kg/m2). Maximum poststent MVP, clinical outcomes, and stent-adjacent stenosis requiring retreatment after VSS were not significantly associated with BMI. We provide direct evidence for a positive correlation between BMI and intracranial venous pressure in patients with IIH. VSS affords a significantly greater amelioration of intracranial venous hypertension and stenosis for IIH patients with higher BMIs. As such, obesity should not be a deterrent for the use of VSS in the management of IIH.

Venous waveform morphological changes associated with treatment of symptomatic venous sinus stenosis

IntroductionVenous outflow obstruction is recognized as a contributing factor in a subset of patients with idiopathic intracranial hypertension (IIH). Little is known about venous sinus waveform morphology or how it...

Intracranial pressure in patients with papilloedema.

Papilloedema is a clinical manifestation of chronically raised intracranial pressure (ICP), often seen in idiopathic intracranial hypertension (IIH). However, the extent of intracranial hypertension required to produce papilloedema is not known. We compare ICP values in IIH patients who developed papilloedema and those who did not. We aim to identify a pathological ICP threshold predictive of the development of papilloedema in IIH patients. Single-centre cohort of IIH patients (2006-2016) who underwent 24-hour ICP monitoring (ICPM) and ophthalmology assessments, prior to intervention. Papilloedema was graded according to the Frisén scale. An unpaired t-test compared 24-hour ICPM between papilloedema and no-papilloedema groups. Fisher's exact test was used to determine predictive value of ICP. Thirty-six patients with IIH (35F: 1M), mean age 32.5±9.49years (mean±SD) were included. Patients with papilloedema had a mean median 24-hour ICP of 10.4±5.32mmHg (n=25), significantly higher than the group without papilloedema 6.31±3.30mmHg (n=11) (P<.05). The papilloedema group were exposed to higher pressures (10mmHg) for 30minutes or more. Using 24-hour median ICP of 10mmHg as a minimum cut-off predictive value gives a specificity=91%, sensitivity=48%, PPV=92% and NPV=44% of detecting papilloedema. A 24-hour ICP of 10mmHg or more is a good predictor for papilloedema and reflects a pathological threshold. The range varied widely suggesting papilloedema can occur at even lower pressures. These results are consistent with emerging evidence suggest that pathologically "high" 24hours ICP is lower than previously quoted.

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

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. 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), 2-6months (V1) and 13months follow-up visit (V2) from time of diagnosis. 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). Idiopathic intracranial hypertension (IIH) is associated with long-term loss of visual function, and relevant treatment strategies need to be improved.

Inflammatory gene expression signatures in idiopathic intracranial hypertension: possible implications in microgravity-induced ICP elevation

The visual impairment and intracranial pressure (VIIP) syndrome is a neuro–ophthalmologic condition described in astronauts returning from long duration space missions. Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is characterized by a chronic elevation of intracranial pressure (ICP) in the absence of an intracranial mass lesion. Because VIIP and IIH share some neurologic and ophthalmologic manifestations, the latter might be used as a model to study some of the processes underlying VIIP. This work constitutes a preliminary investigation of the molecular pathways associated with the elevation of ICP in IIH. Gene expression signatures were obtained from exosomes collected from CSF and plasma in patients with possible signs of IIH. The gene expression targets focused on inflammatory genes and miRNAs. The results suggest that inflammatory cytokine-driven processes and immune cell migration are activated when ICP is elevated in IIH patients, either as a cause or effect of the ICP increase. Several miRNAs appear to be involved in this response, among which miR-9 and miR-16 are upregulated in CSF and plasma of higher ICP subjects. This study provides evidence in support of neurophysiological alterations and neuro-immunomodulation in this condition. If similar changes are seen in astronauts manifesting with the VIIP syndrome, an underlying pathophysiological basis may be discovered.

Clinical and Neuro-ophthalmologic Predictors of Visual Outcome in Idiopathic Intracranial Hypertension

ABSTRACTDespite the potential of Idiopathic Intracranial Hypertension (IIH) to cause visual morbidity, limited literature is available focussing on predictors of visual outcome in IIH. This study was planned to assess visual morbidity in patients of IIH in terms of clinical and neuro-ophthalmo- logical parameters. In this prospective study of 40 patients of IIH, neuro-ophthalmological parameters were noted in the form of visual acuity, visual field, contrast sensitivity, Retinal Nerve Fibre Layer thickness, and visual evoked potential. Visual outcome was defined in using specific criteria. Final visual outcome of patients was compared with clinical and neuro-ophthalmologic para- meters to determine any correlation. The most common presenting clinical symptoms were headache (85%) and Transient visual obscurations (TVOs) (52.5%). In univariate analysis severity of visual loss, Cerebro Spinal Fluid (CSF) pressures and abnormal Visual evoked potential (VEP) were associated with worse visual outcome or need of aggressive management. When adjusted for severity of visual loss no independent clinical/neuroophthalmic predictor could be established. High CSF opening pressure, worsening vision/papilledema, greater Retinal Nerve Fiber Layer (RNFL) thickness and abnormal VEPs may be some of the alarming signs for physicians, but none of these parameters can be used as an independent predictor for visual outcome in isolation. Visual loss at presentation is probably the most important predictor of the final visual outcome in these patients. This may also suggest that patients presenting in an advanced disease course (with worse visual status) fair badly despite best medical/surgical management. Early diagnosis and prompt management is the cornerstone of management.

Weight reduction for a better visual outcome in idiopathic intracranial hypertension.

To evaluate the correlation between weight reduction and visual outcome in overweight patients with idiopathic intracranial hypertension. Thirty-nine newly diagnosed, overweight (body mass index >25 kg/m2) patients with idiopathic intracranial hypertension were studied retrospectively. All patients underwent medical treatment with acetazolamide, and a weight reduction program was also offered. Patients were grouped according to their compliance with this weight reduction program into the diet-success (Group 1) and diet-failure groups (Group 2). Body mass index, papilledema, visual acuity, and perimetric mean deviation were compared at the end of the 6-month study period. Groups 1 and 2 did not differ regarding the baseline mean body mass index (32.63 ± 5.61, 32.35 ± 5.06 kg/m2), visual acuity (0.080 ± 0.13, 0.130 ± 0.24 logMAR), perimetric mean deviation (-9.978 ± 0.68, -12.86 ± 8.91), or papilledema grade (2.94 ± 0.22, 2.90 ± 0.30), respectively (p>0.05). During the 6 months' follow-up, Group 1 patients, who complied with both medical and diet therapy, improved significantly in all parameters, including body mass index (p<0.001), visual acuity (p=0.001), perimetric mean deviation (p=0.016), and papilledema grade (p<0.001). Conversely, Group 2 patients, who only underwent medical therapy, improved only in papilledema grade (p<0.001). However, coincident development of optic disc pallor was observed in three patients. Further, they also had significant loss in visual acuity (p=0.047) during the study period. Weight reduction combined with medical treatment is associated with significantly better improvement in visual acuity, visual field, and papilledema in idiopathic intracranial hypertension patients. Compliance with an efficient diet program should be encouraged in overweight patients with idiopathic intracranial hypertension.

The effect of topiramate as an adjunct therapy to acetazolamide in Idiopathic intracranial hypertension patients.

The effect of topiramate as an adjunct therapy to acetazolamide in Idiopathic intracranial hypertension patients.

Is cerebral edema effective in idiopathic intracranial hypertension pathogenesis?: Diffusion weighted MR imaging study

The aim of this study is to research whether cerebral edema is effective in the pathogenesis of patients with idiopathic intracranial hypertension (IIH) by using diffusion weighted imaging (DWI) and apparent diffusion coefficient (ADC) measurements. Was changed into thirty-six IHH patients who received DWI and 36 age- and sex-matched healthy control group were assessed retrospectively. ADCmin, ADCmean, and ADCmax values were measured from different regions of the brain for both IHH patients and the control group. The StudentÂ’s t-test was used to compare the ADCmin, ADCmax, ADCmean values acquired from distinct parts of the brain parenchyma of IIH patients with the values of control group. No significant difference was found between ADCmin, ADCmax, and ADCmean values of IIH and control group in bilateral frontal, parietal temporal and occipital lobe cortical and subcortical white matter, caudate nucleus head, putamen, thalamus, corpus callosum splenium and genu (P>0.05). This study showed that cerebral edema cannot be a significant mechanism in the pathogenesis of IIH.

The role of optical coherence tomography in neuro-ophthalmology

Optical coherence tomography (OCT) is an ocular imaging technique that can complement the neuro-ophthalmic assessment, and inform our understanding regarding functional consequences of neuroaxonal injury in the afferent visual pathway. Indeed, OCT has emerged as a surrogate end-point in the diagnosis and follow up of several demyelinating syndromes of the central nervous system (CNS), including optic neuritis (ON) associated with: multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), and anti-myelin oligodendrocyte glycoprotein (MOG) antibodies. Recent advancements in enhanced depth imaging (EDI) OCT have distinguished this technique as a new gold standard in the diagnosis of optic disc drusen (ODD). Moreover, OCT may enhance our ability to distinguish cases of papilledema from pseudopapilledema caused by ODD. In the setting of idiopathic intracranial hypertension (IIH), OCT has shown benefit in tracking responses to treatment, with respect to reduced retinal nerve fiber layer (RNFL) measures and morphological changes in the angling of Bruch’s membrane. Longitudinal follow up of OCT measured ganglion cell-inner plexiform layer thickness may be of particular value in managing IIH patients who have secondary optic atrophy. Causes of compressive optic neuropathies may be readily diagnosed with OCT, even in the absence of overt visual field defects. Furthermore, OCT values may offer some prognostic value in predicting post-operative outcomes in these patients. Finally, OCT can be indispensable in differentiating optic neuropathies from retinal diseases in patients presenting with vision loss, and an unrevealing fundus examination. In this review, our over-arching goal is to highlight the potential role of OCT, as an ancillary investigation, in the diagnosis and management of various optic nerve disorders.

Segmentation error in spectral domain optical coherence tomography measures of the retinal nerve fibre layer thickness in idiopathic intracranial hypertension

BackgroundOptical Coherence Tomography (OCT) imaging is being increasingly used in clinical practice for the monitoring of papilloedema. The aim is to characterise the extent and location of the Retinal Nerve Fibre Layer (RNFL) Thickness automated segmentation error (SegE) by manual refinement, in a cohort of Idiopathic Intracranial Hypertension (IIH) patients with papilloedema and compare this to controls.MethodsBaseline Spectral Domain OCT (SDOCT) scans from patients with IIH, and controls with no retinal or optic nerve pathology, were examined. The internal limiting membrane and RNFL thickness of the most severely affected eye was examined for SegE and re-segmented. Using ImageJ, the total area of the RNFL thickness was calculated pre and post re-segmentation and the percentage change was determined. The distribution of RNFL thickness error was qualitatively assessed.ResultsSignificantly greater SegE (p = 0.009) was present in RNFL thickness total area, assessed using ImageJ, in IIH patients (n = 46, 5% ± 0–58%) compared to controls (n = 14, 1% ± 0–6%). This was particularly evident in moderate to severe optic disc swelling (n = 23, 10% ± 0–58%, p < 0.001). RNFL thickness was unable to be quantified using SDOCT in patients with severe papilloedema.ConclusionsSegE remain a concern for clinicians using SDOCT to monitor papilloedema in IIH, particularly in the assessment of eyes with moderate to severe oedema. Systematic assessment and manual refinement of SegE is therefore important to ensure the accuracy in longitudinal monitoring of patients.

Improving identification of idiopathic intracranial hypertension patients in Swedish patient register.

Idiopathic intracranial hypertension (IIH) is often misdiagnosed. This can cause problems if conducting register-based studies. The study purpose was to produce algorithms that better identify patients with correct diagnosis of IIH in the Swedish National Patient Register (NPR). Patients with ICD-10 code G93.2 for IIH registered in the NPR (2006-2013, Stockholm County) were included and diagnosis validated by medical record reviews. Patients were randomized into two groups: one used to produce the algorithm (n=105) and one for validation (n=102). We tested variables possible to extract from registries and used forward stepwise logistic regression which provided a predicted probability of correct diagnosis for each patient. We included 207 patients of which 135 had confirmed IIH. This gave a positive predictive value of 65.2% (CI: 58.4-71.4). The algorithm produced with variables extracted from registries, that is, age, number of times with diagnosis code G93.2 recorded (>2 times), and acetazolamide treatment, predicted the diagnosis correctly 88.2% (CI: 80.3-93.3) of the time. Excluding treatment data from the algorithm did not change the prediction notably, 86.3% (CI: 78.1-91.7). We produced two algorithms that with improved accuracy predict whether an IIH diagnosis in the NPR is correct. This can be a useful tool when performing register-based studies.

Migraine headache in patients with idiopathic intracranial hypertension

Migraine is a neurological disorder that afflicts many people in the world and can cause severe disability during the attacks. The pathophysiology of migraine is complex and not fully understood. It seems that migraine is common in idiopathic intracranial hypertension (IIH). However, the association between migraine headache and IIH is still unclear. The present study was conducted to assess the prevalence of migraine headache and associated factors in IIH patients. In this cross-sectional study, a total of 68 patients diagnosed with IIH underwent a medical history interview and a neurological examination. The diagnosis of migraine was based on the four diagnostic criteria of the International Classification of Headache Disorders 3rd edition. Forty-five patients (63.2%) met the diagnostic criteria of migraine headache. There was no significant difference between patients with and without migraine headache in respect of their age, gender, body mass. This study revealed high prevalence of migraine headache in IIH patients; appropriate treatment can reduce their headache and prevent unnecessary treatments for IIH.

Migraine headache in patients with idiopathic intracranial hypertension.

Migraine is a neurological disorder that afflicts many people in the world and can cause severe disability during the attacks. The pathophysiology of migraine is complex and not fully understood. It seems that migraine is common in idiopathic intracranial hypertension (IIH). However, the association between migraine headache and IIH is still unclear. The present study was conducted to assess the prevalence of migraine headache and associated factors in IIH patients. In this cross-sectional study, a total of 68 patients diagnosed with IIH underwent a medical history interview and a neurological examination. The diagnosis of migraine was based on the four diagnostic criteria of the International Classification of Headache Disorders 3rd edition. Forty-five patients (63.2%) met the diagnostic criteria of migraine headache. There was no significant difference between patients with and without migraine headache in respect of their age, gender, body mass. This study revealed high prevalence of migraine headache in IIH patients; appropriate treatment can reduce their headache and prevent unnecessary treatments for IIH.