Idiopathic Intracranial Hypertension Research Articles

Safety profile of levonorgestrel intrauterine system: Analysis of spontaneous reports submitted to FAERS

The levonorgestrel-releasing intrauterine system (LNG-IUS) is an established, long-acting contraceptive option. With the widespread use of the LNG-IUS, drug-reported adverse events (AEs) have also garnered significant attention. In this study, we conducted a real-world analysis using the FDA's Adverse Event Reporting System (FAERS) database to assess the incidence of AEs associated with LNG-IUS use. Data from FAERS spanning from 2004Q1 to 2024Q1 were reviewed, with a focus on reports in which LNG-IUS was the primary suspected and secondary suspect drug. Signal detection was carried out utilizing Standardized MedDRA Queries (SMQ) and Preferred Terms (PT), with reporting odds ratio (ROR), proportional reporting ratio (PRR), and information component (IC) employed to identify Signals of Disproportionate Reporting (SDR) for AEs. A positive SDR was defined when all three methods indicated significance. Analysis of 13 SMQs revealed notable SDRs in ear and eye disorders, cardiac arrhythmias, and lipodystrophy. Of the 61 suspected SDRs identified at the PT level, nearly half were not previously documented in labeling. Key potential signals of AEs associated with LNG-IUS use included increased heart rate, papilledema, idiopathic intracranial hypertension, cervical dysplasia, ruptured ovarian cyst, and uterine embedment and perforation. The findings underscore the importance of signal detection using FAERS data for identifying safety concerns related to LNG-IUS. Long-term observational studies are warranted to confirm and further elucidate these potential safety signals.

Radiological signs supporting idiopathic intracranial hypertension in symptomatic patients with lumbar puncture opening pressure < 250 mm

Lumbar puncture opening pressure (LPOP) exceeding 250mmH2O is key in diagnosing idiopathic intracranial hypertension (IIH), per revised Friedman’s criteria. Some patients do not meet LPOP criteria (with or without papilledema), despite having IIH-related symptoms and neuroimaging findings. This study aimed to investigate the radiological findings and clinical symptoms in patients suspected of having IIH without meeting the LPOP criteria. We retrospectively evaluated cerebral venous sinus stenosis using the conduit Farb score (CFS) and other radiological findings suggestive of IIH by computed tomography venography and magnetic resonance venography in females ≥ 18 years-old with chronic headaches, suspected IIH, and LPOP < 250 mm. Eighty-eight women (56 with LPOP < 200 mm H2O and 32 with LPOP ranging between 200 and 250mmH2O) were included. Among patients with LPOP 200–250mmH2O, 40% (12) exhibited three or more radiological findings supporting IIH, compared to 17% (8) in the LPOP < 200 mmH2O group (p = 0.048). Cerebral venous stenosis (CFS ≤ 5) was observed in 80% (24) of those with LPOP 200–250 mmH2O, contrasting with 40% (19) of those with LPOP < 200 mmH2O (p < 0.001). Cerebral venous stenosis was significantly more common in patients with LPOP 200–250 mmH2O than < 200 mmH2O, suggesting that they may benefit from IIH treatment.

Transorbital B-mode ultrasound for the assessment of posterior globe flattening in idiopathic intracranial hypertension: a pilot study

BackgroundPosterior globe flattening (PGF) is a specific neuroimaging sign in patients with idiopathic intracranial hypertension (IIH), but its detection is based on subjective qualitative neuroradiological assessment. This study sought to evaluate the utility of transorbital ultrasound to detect and quantify PGF in IIH patients using the Posterior Globe Angle (PGA).MethodsConsecutive IIH patients and healthy controls were enrolled in a prospective case-control study. Transorbital ultrasound was performed to assess the presence of PGF. For quantification of PGF, an angular measurement (PGA) was performed with the vertex centering the optic nerve at a predefined distance from the lamina cribrosa and angle legs tangentially aligned to the borders of the vitreous body. PGA measurements were compared between IIH patients and healthy controls. Additionally, the diagnostic accuracy of PGA measurements in detecting PGF was evaluated using ROC analysis.ResultsThirty-one IIH patients (37.3 ± 12.3 years, 29 female) and 28 controls (33.3 ± 11.8 years, 21 female) were compared. PGF was present in 39% of IIH patients and absent in the control group. PGA3mm measurements significantly differed between IIH and controls (116.5° ± 5.5 vs. 111.7° ± 2.9; p < 0.001). A PGA3mm cutoff of ≥ 118.5° distinguished IIH patients from controls with 100% specificity, while retaining a sensitivity of 37.5%.ConclusionsTransorbital ultrasound may be applied to detect and quantify PGF in IIH patients. Prospective, multicenter studies with extended cohorts and blinded design are needed to validate these preliminary findings and confirm the diagnostic utility of transorbital ultrasound for the assessment of PGF in IIH.

The relationship between body mass index and cerebrospinal fluid pressure in children with pseudotumor cerebri

BackgroundChildhood obesity has become a major global health problem. Obesity is associated with major health problems, such as diabetes, hypertension, dyslipidemia, cardiovascular disease. Obesity is also considered a risk factor for Pseudotumor cerebri (PTC). The present study aimed to investigate the relationship between body mass index (BMI), and cerebrospinal fluid (CSF) pressure in patients with pseudotumor cerebri.MethodsA total of 48 children diagnosed with PTC, who were aged < 18 years and followed up in the pediatric clinic were included in the retrospective study. National BMI percentile curves were used for reference. We investigated statistically the relationship between BMI, clinical and laboratory results, and CSF pressure in patients.ResultsOf total patients 27 were female (56.25%) and 21 were male (43.75%). With regard to the BMI percentile, 20 (41.67%) were overweight or obese. CSF pressure was higher in overweight and obese patients compared to children with BMI in normal ranges (p < 0.05). A statistically significant positive correlation was also observed between BMI and CSF pressure values and between monocyte and CSF values (p < 0.05).ConclusionsThe results of the present study indicate a direct relationship between CSF pressure and BMI in children with PTC. Appropriate diet, exercise, and medical treatment in overweight and obese children can make a significant contribution to the treatment of PTC. Additionally, a significant correlation was observed between CSF pressure and monocyte levels.

Non-invasive estimation of cerebrospinal fluid pressure in idiopathic intracranial hypertension: magnetic resonance imaging analysis of optic nerve and eyeball.

Invasive methods such as lumbar puncture and intraventricular catheters are commonly used to measure intracranial pressure (ICP). This study aims to develop quantitative and non-invasive techniques to measure ICP in patients with Idiopathic Intracranial Hypertension (IIH) using magnetic resonance imaging (MRI) findings. MRI data obtained from 50 patients with IIH and 30 age- and sex- matched controls were analyzed and optic nerve sheath diameter (ONSD), eyeball transverse diameter (ETD) and optic nerve diameter (OND) were measured. ONSD, ONSD/ETD and OND/ONSD indexes were calculated according to different ONSD measurement distances. Correlations of MRI findings with ICP were calculated. Sensitivity and specificity of all methods were analyzed. ONSD and ONSD/ETD index at 3mm and 10mm behind the eyeball were significantly higher (p < 0.001) and OND/ONSD index at 3mm behind the eyeball was significantly lower (p < 0.001) in the IIH group. The ONSD/ETD index at 3mm had the highest area under the curve (AUC) value (0.898) with a cut-off of 0.27mm (82% sensitivity and 91.67% specificity) for predicting high cerebrospinal fluid (CSF) pressure, followed by ONSD measurements at 3mm (AUC = 0.886) with a cut-off of 6.17mm (83% sensitivity and 86.67% specificity). The OND/ONSD index at 3mm posterior to the eyeball decreased significantly as ICP increased, and the strength of the relationship was moderate (p < 0.001; r = -0.358). ONSD and ONSD/ETD index measured on MRI sequences are potentially useful in detecting elevated ICP. The OND/ONSD index correlates with CSF pressure and these techniques may be helpful in diagnosing IIH.

An interdisciplinary integrated specialized one-stop outpatient clinic for idiopathic intracranial hypertension-a comprehensive assessment of clinical outcome.

Management of idiopathic intracranial hypertension (IIH) is complex requiring multiple specialized disciplines. In practice, this creates considerable organizational and communicational challenges for healthcare professionals and patients. Thus, an interdisciplinary integrated outpatient clinic for IIH (comprising neurology, neuroophthalmology, neuroradiology, neurosurgery and endocrinology) was established with central coordination and a one-stop concept. Here, the aim was to evaluate the effects of this one-stop concept on objective clinical outcome. In a retrospective cohort study, the one-stop era with integrated care (IC) (1 July 2021 to 31 December 2022) was compared to a reference group receiving standard care (SC) (1 July 2018 to 31 December 2019) regarding visual impairment/worsening and headache improvement/freedom 6 months after diagnosis. Multivariate binary logistic regression models were used to adjust for confounders. Baseline characteristics of the IC group (n = 85) and SC group (n = 81) were comparable (female 90.6% vs. 90.1%; mean age 33.6 vs. 32.8 years; median body mass index 31.8 vs. 33.0; median cerebrospinal fluid opening pressure 32 vs. 34 cmH2O; at diagnosis, visual impairment was present in 71.8% vs. 69.1% and chronic headache in 55.3% vs. 56.8% in IC vs. SC). IC was associated with a higher likelihood of achieving both headache improvement (odds ratio [OR] 2.24, 95% confidence interval [CI] 1.52-4.33, p < 0.001) and headache freedom (OR 1.75, 95% CI 1.11-3.09, p = 0.031). Regarding the risk of visual impairment and visual worsening IC was superior numerically but not statistically significantly (OR 0.87, 95% CI 0.69-1.16, p = 0.231, and OR 0.67, 95% CI 0.41-1.25, p = 0.354). Interdisciplinary integrated care of IIH is favourably associated with headache outcomes and potentially also visual outcomes.

Evaluation of the audiovestibular system before and after treatment in patients with Idiopathic intracranial hypertension

Background Idiopathic intracranial hypertension (IIH) can affect both hearing and balance due to increased inner ear pressure. Aims/Objectives This study aimed to evaluate the impact of increased inner ear pressure on hearing and balance in patients with IIH using auditory and vestibular tests. Material and Methods Twenty-four IIH patients and 28 healthy controls underwent oVEMP, pure tone audiometry, tympanometry, and acoustic reflex tests pre-lumbar punctures. IIH patients received acetazolamide. Pre- and post-treatment results, tinnitus, and vertigo scores were compared. Post-treatment oVEMP and audiometry results were compared between groups. Results Pre-treatment oVEMP showed a significant left N1 latency difference (p = 0.049). Post-treatment, left ear amplitude (p = 0.035) and both ear amplitude ratios (p = 0.044 and p = 0.047) increased significantly. Audiometry had no significant changes (p < 0.05). Tinnitus and vertigo scores decreased significantly (p ≤ 0.001). Conclusion Prolonged oVEMP latency suggests IIH may impact the brain stem and vestibular nerve, while increased amplitude values indicate peripheral vestibular involvement. IIH affects hearing across all frequencies, especially at 4000 Hz, impacting both hearing and balance. Significance Understanding the effects of IIH on auditory and vestibular functions can guide effective treatments, improving quality of life for patients by addressing both hearing and balance issues.

Outcomes of Pulsatile Tinnitus After Cerebral Venous Sinus Stenting: Systematic Review and Pooled Analysis of 616 Patients

Pulsatile tinnitus (PT) is a debilitating condition with substantial morbidity related to quality of life. Cerebral venous sinus stenosis has recently emerged as a noninfrequent cause of PT, either in the setting of concurrent idiopathic intracranial hypertension (IIH) or due to primary venous stenosis. Venous sinus stenting (VSS) is an endovascular technique that can be used to treat venous stenosis. However, it is unclear if outcomes are different between patients with primary venogenic PT and IIH associated PT. A systematic literature review and pooled analysis was completed to evaluate the clinical outcomes of PT in patients undergoing cerebral VSS. Outcome measures included: Improved PT, complete resolution of PT, and PT recurrence at follow-up. Subgroup analysis between patients with IIH and primary PT was completed. In total, 28 studies were identified with 616 patients. The proportion of improved PT symptoms after VSS had an overall pooled rate of 91.7% (confidence interval [CI]:88.1%-95.2%; I2= 65%) and no difference between subgroups (P= 0.12). Complete resolution after VSS had an overall pooled rate of 88.6% (CI: 84.0%-93.3%; I2= 68%) and no significant difference between subgroups (P= 0.35). Recurrent PT after stenting occurred in 6.5% of cases (CI: 1.7%-11.3%; I2= 62%). Furthermore, subgroup analysis demonstrated that IIH patients had a significantly higher recurrence rate (10.6%; CI: 5.2%-16.1%; I2= 26%) compared to patients treated with venous stenting for PT as the primary indication (2.0%; CI: 0.8%-4.7%; I2= 0%) (P < 0.0001). Venous stenting in patients with PT results in a substantial decrease and often complete resolution of symptoms. PT is more likely to recur in patients with IIH-associated PT.

Venous sinus stenting under conscious sedation

BackgroundVenous sinus stenting (VSS) is an increasingly performed procedure for the treatment of idiopathic intracranial hypertension (IIH) refractory to medical treatment. VSS is typically performed under general anesthesia.ObjectiveTo present our...

Idiopathic intracranial hypertension pathogenesis: The jugular hypothesis.

In spite of expanding research, idiopathic intracranial hypertension (IIH) and its spectrum conditions remain challenging to treat. The failure to develop effective treatment strategies is largely due to poor agreement on a coherent disease pathogenesis model. Herein we provide a hypothesis of a unifying model centered around the internal jugular veins (IJV) to explain the development of IIH, which contends the following: (1) the IJV are prone to both physiological and pathological compression throughout their course, including compression near C1 and the styloid process, dynamic muscular/carotid compression from C3 to C6, and lymphatic compression; (2) severe dynamic IJV stenosis with developments of large cervical gradients is common in IIH-spectrum patients and significantly impacts intracranial venous and cerebrospinal fluid (CSF) pressures; (3) pre-existing IJV stenosis may be exacerbated by infectious/inflammatory etiologies that induce retromandibular cervical lymphatic hypertrophy; (4) extra-jugular venous collaterals dilate with chronic use but are insufficient resulting in impaired aggregate cerebral venous outflow; (5) poor IJV outflow initiates, or in conjunction with other factors, contributes to intracranial venous hypertension and congestion leading to higher CSF pressures and intracranial pressure (ICP); (6) glymphatic congestion occurs but is insufficient to compensate and this pathway becomes overwhelmed; and (7) elevated intracranial CSF pressures triggers extramural venous sinus stenosis in susceptible individuals that amplifies ICP elevation producing severe clinical manifestations. Future studies must focus on establishing norms for dynamic cerebral venous outflow and IJV physiology in the absence of disease so that we may better understand and define the diseased state.

Beyond the AJR: Can Evidence-Based MRI Signs Improve the Diagnostic Accuracy of Idiopathic Intracranial Hypertension?

Beyond the AJR: Can Evidence-Based MRI Signs Improve the Diagnostic Accuracy of Idiopathic Intracranial Hypertension?

Progress and recognition of idiopathic intracranial hypertension: A narrative review.

Idiopathic intracranial hypertension (IIH) mainly affects obese young women, causing elevated intracranial pressure, headaches, and papilledema, risking vision loss and severe headaches. Despite weight loss as the primary treatment, the underlying mechanisms remain unclear. Recent research explores novel therapeutic targets. This review aimed to provide a comprehensive understanding of IIH's pathophysiology and clinical features to inform pathogenesis and improve treatment strategies. Recent publications on IIH were searched and summarized using PubMed, Web of Science, and MEDLINE. The review highlights potential pathomechanisms and therapeutic advances in IIH. IIH incidence is rising, with growing evidence linking it to metabolic and hormonal disturbances. Early diagnosis and treatment remain challenging.

Role of neuroimaging markers on predicting of idiopathic intracranial hypertension.

The goals of neuroimaging in idiopathic intracranial hypertension (IIH) are the exclusion of mimickers and effective management of disease. In recent studies, several imaging markers have been identified as potential predictors of IIH. To investigate the predictive roles of novel radiological markers as the Meckel's cave area, alongside classical radiologic markers in identifying IIH such as the empty sella. The patients were classified according to cerebrospinal fluid (CSF) opening pressure as the IIH group and control group. The observational, case-control study included 22 patients with IIH and 22 controls. Groups were compared for presence of empty sella, Meckel's cave area, fat area of posterior neck, fat thickness of scalp, presence of transverse sinus stenosis, and ophthalmic markers, such as increase of optic nerve (ON) sheath diameter. In the IHH group, higher occurrences of increased ON sheath diameter, ON tortuosity, flattening of the scleral surface, and transverse sinus stenosis were observed (P < 0.001, P < 0.001, P = 0.046, and P = 0.021, respectively). Meckel's cave area and fat area of posterior neck were similar in both groups (P = 0.444 and P = 0.794). Ophthalmic markers and transverse sinus stenosis could be utilized as radiologic features supporting early and precise diagnosis of IIH. However, enlargement of Meckel's cave area and measurements of fatty area of posterior neck are not helpful for diagnosis of IIH.

Idiopathic intracranial hypertension. Review of our experience in the last eight years (2016-2023)

Idiopathic intracranial hypertension (IIH) or benign intracranial hypertension is a rare disease in childhood. The clinical presentation in pediatric patients can be very variable, being more unespecific in younger patients. A retrospective descriptive study was carried out on patients diagnosed of IIH in the last eight years (2016-2023) in the neuropediatrics unit of a tertiary hospital. In the present study, the clinical-epidemiological characteristics and the diagnostic-therapeutic procedure carried out in each case were analyzed. We studied 14 patients, 57% were women. The average age at diagnosis was 9 years, headache was the most common reason for consultation. In all patients, papilledema was found in the fundus and neuroimaging didn´t find alterations. Optical coherence tomography has been carried out in 78.5% of the sample, > 80% of patients showed thickening of the retinal nerve fiber layer. All patients had a high cerebrospinal fluid opening pressure (>25 cmH2O). 57% of patients required treatment with acetazolamide, a carbonic anhydrase inhibito. In all patients the resolution was complete, however almost 30% of them have presented recurrences during follow-up. In recent years there has been an increase in the incidence of this entity, making early diagnosis and treatment essential to avoid possible irreversible damage.

An interdisciplinary integrated specialized one-stop outpatient clinic for idiopathic intracranial hypertension – a comprehensive assessment of patient satisfaction

BackgroundManagement of idiopathic intracranial hypertension (IIH) is complex requiring contributions from multiple specialized disciplines. In practice, this creates considerable organizational and communicational challenges. To meet those challenges, we established an interdisciplinary integrated outpatient clinic for IIH with a central coordination and a one-stop concept. Here, we aimed to evaluate effects of this one-stop concept on subjective patient satisfaction and economic outcome in patients with IIH.MethodsIn a retrospective cohort study, we compared the one-stop era with integrated care (IC, 1-JUL-2021 to 31-DEC-2022) to a reference group receiving standard care (SC, 1-JUL-2018 to 31-DEC-2019) regarding subjective patient satisfaction (assessed by the Vienna Patient Inventory). Multivariable binary linear regression models were used to adjust for confounders.ResultsBaseline characteristics of the IC group (n = 85) and SC group (n = 81) were comparable (female: 90.6% vs. 90.1%; mean age: 33.6 vs. 32.8 years, educational level: ≥9 years of education 60.0% vs. 59.3%; located in Vienna 75.3% vs. 76.5%). Compared to SC, management within IC concept was associated with statistically significantly higher subjective patient satisfaction (beta = 0.93; p < 0.001) with the strongest effects observed in satisfaction with treatment accessibility and availability (beta = 2.05; p < 0.001). Subgroup analyses of patients with migration background and language barrier consistently indicated stronger effects of IC in these groups.ConclusionsInterdisciplinary integrated management of IIH statistically significantly and clinically meaningfully improves patient satisfaction – particularly in socioeconomically underprivileged patient groups. Providing structured central coordination to facilitate and improve access to interdisciplinary management provides means to further improve outcome.

Health Coaching Effectiveness in Controlling Idiopathic Intracranial Hypertension

Health Coaching Effectiveness in Controlling Idiopathic Intracranial Hypertension

Yoga Nidra as an Adjunctive Therapy in Idiopathic Intracranial Hypertension: A Case Study

Yoga Nidra as an Adjunctive Therapy in Idiopathic Intracranial Hypertension: A Case Study

Comparing open and closed cell stents in idiopathic intracranial hypertension: A comprehensive meta-analysis of clinical outcomes.

Background: Open cell stents (OC) and closed cell stents (CC) each offer unique advantages and potential drawbacks in the context of idiopathic intracranial hypertension (IIH) treatment. We aim to investigate the safety and efficacy of using OC and CC for IIH.Methods: We conducted a systematic review in PubMed, Embase, and Cochrane Library databases following the PRISMA guidelines. Eligible studies included ≥4 patients with IIH treated by OC or CC. Primary outcomes were headache, visual acuity, and papilledema status before and after the procedure. Additionally, failure rate, minor complications, major complications, and total complications were assessed. Pooled analysis of the OC group and CC group were done separately and then compared.Results: Twenty-four studies were included. Of these, 20 reported on OC and 6 reported on CC. Pooled analysis of failure rate was 8% (4%-12%) in OC and 5% (0%-11%) in CC. For headache improvement rate: 78% (70%-86%) in OC and 81% (66%-69%) in CC. For visual acuity improvement: 78% (65%-92%) in OC and 76% (29%-100%) in CC. For papilledema improvement: 88% (77%-98%) in OC and 82% (67%-98%) in CC. For minor complications: 0% (0%-1%) in OC and 0% (0%-2%) in CC. For major complications: 0% (0%-1%) in OC and 2% (0%-6%) in CC. Total complications: 0% (0%-1%) in OC and 2% (0%-6%) in CC.Conclusion: Low failure and complication rates were found in both OC and CC, with no significant difference between them in effectiveness. The CC showed a slight but significant increase in major and total complications compared to the OC. Additionally, a subtle yet significantly lower failure rate was identified in the CC.

Infantile Idiopathic Intracranial Hypertension: Case Report of an 8-Month-Old Child

AbstractIdiopathic intracranial hypertension (IIH), a clinical disorder also known as pseudotumor cerebri, is characterized by increased intracranial pressure of unknown causes. Although it is most commonly observed in young obese women, IIH can also occur in the pediatric population; however, it is extremely rare in infants. Infants do not typically exhibit characteristic signs and symptoms of IIH, making it challenging to diagnose. Additionally, treatment modalities for this disease remain uncertain. We report an 8-month-old female child admitted to the pediatric emergency department with IIH. Our patient presented initially with fever, vomiting, and diarrhea, and then she developed inability to abduct the left eye. Papilledema was not detected in our patient. Magnetic resonance imaging and lumbar puncture contributed to diagnosis of the patient. Dexamethasone (0.5 mg/kg/d, twice a day) and acetazolamide (5 mg/kg/d) were administered; dexamethasone was stopped at the 48th hour after esotropia had resolved. The patient developed left ptosis in the first month after discharge. Acetazolamide was administered for a duration of 5 months and was discontinued once the patient's eye findings improved. With a sudden onset of neurological findings in a healthy infant with normal neuroimaging, IIH should be considered in the differential diagnosis and cerebrospinal fluid pressure should be measured. The principles of treatment for this disease are based on adult guidelines, but the efficacy of acetazolamide has also been reported in the pediatric population, like in our case.

Growth hormone therapy does not impact the development of intracranial hypertension in children with Chiari malformation.

Patients with Chiari malformation (CM) are prone to a variety of neurological sequelae, including benign intracranial hypertension (BIH). In these patients, BIH is attributed to impaired cerebrospinal fluid (CSF) flow due to anatomical abnormalities of the posterior fossa. Occasionally, patients with CM may require growth hormone therapy (GHT), which can increase the production of CSF. It is thought that patients with CM who undergo GHT are at high risk of BIH-associated symptoms (BIHAS). We describe the incidence of neurological symptoms in 34 patients with CM before and during GHT. The database of a pediatric endocrinology center was queried for patients with CM who received GHT from2010-22. Records were reviewed for adverse events. Demographic and radiological data were collected and analyzed. Patients with neoplastic disease, active inflammation, or acute trauma were excluded. CM diagnoses were independently assigned by a neuroradiology department. Patients were grouped based on the presence and nature ofsymptoms before and during GHT. Relationships between starting dose/BMI and occurrence of BIHAS/all GHT-associated symptoms were evaluated. GHT was not associated with new-onset or worsening of preexisting BIHAS in 33 out of 34 patients with CM. Five complex patients continued to have preexisting BIHAS, which did not worsen. Of the four patients who developed new-onset BIHAS during GHT, three patients' symptoms were attributed to other medical conditions. No patient permanently discontinued GHT due to BIHAS. Growth hormone therapy is likely a safe treatment in patients with Chiari malformation and is unlikely to cause BIHAS.