Idiopathic Intracranial Hypertension With Sustained Normal Opening Pressure: A Case Report.

Idiopathic intracranial hypertension (IIH) is a rare condition characterized by raised intracranial pressure (ICP) without related pathology in either the brain or the composition of cerebrospinal fluid (CSF). Herein, we provide a brief review of the clinical presentation of IIH and the anesthetic considerations in parturients diagnosed with the disorder. We conducted a MEDLINE® literature search for all types of articles published in English with restriction for year of publication, and we used the search terms "idiopathic intracranial hypertension", "pseudotumor cerebri", "benign intracranial hypertension", "pregnancy", "cesarean section", "labour analgesia", "epidural", and "anesthesia". Idiopathic intracranial hypertension affects primarily obese women of childbearing age. The main symptom is headache, and the cardinal sign is papilledema. The main goal of management is to preserve visual function. Treatment lies in the administration of diuretics and corticosteroids, control of excessive weight gain, and surgical management, such as cerebrospinal fluid diversion or optic nerve sheath fenestration for refractory cases. For the parturient with IIH, Cesarean delivery is not necessarily indicated. Neuraxial anesthesia has been used uneventfully for both labour analgesia and for Cesarean delivery. There are reports describing successful use of both spinal and epidural anesthesia, even in IIH patients with CSF diversion devices in situ. Although IIH is rare, there are special considerations for anesthetic management in the parturient. Despite the presence of raised ICP in these patients, there are no specific contraindications to neuraxial techniques, and uncal herniation has not been reported to occur in patients with IIH.

Idiopathic intracranial hypertension (IIH) is characterized by raised intracranial pressure of unknown origin that primarily afflicts obese women of childbearing age. There are several treatment options, but currently there are none that are effective for the entire affected population. The lack of a universally effective treatment is related to an incomplete understanding of the etiology of the condition and the lack of a well-defined pathophysiological mechanism for the disease process. Classically, IIH has been thought of as a diagnosis of exclusion once radiographical imaging has ruled out all other causes of elevated intracranial pressure. Today, we know that imaging does capture subtle changes, and might provide keys to finally understand the pathogenesis of IIH so that a definitive treatment can be discovered or developed. Recently, advancements in radiography, optical coherence tomography, and electroretinography have shown promise for the future of IIH evaluation. A topic within IIH imaging that has recently sparked interest is the possibility that the severity of papilledema may have an association with the size of the optic canal. In this article, we also discuss the recent studies on the relationship between asymmetric papilledema and optic canal size.

The purpose of this review is to shed light into the diagnostic challenges regarding idiopathic intracranial hypertension (IIH), giving more emphasis to the role of optical coherence tomography (OCT) in IIH, while it also presents recent advances in the management of IIH. A literature search in PubMed was performed for studies about the diagnosis and management of IIH published before June 30, 2017. Then, a comprehensive review of the eligible studies and the relevant references was performed. Apart from the updated modified criteria, OCT has been found to be important for the diagnosis of IIH, along with assessment of clinical signs and symptoms, brain imaging, and lumbar puncture. On the other hand, there is no current consensus for the management of IIH, but various interventions have been used, such as dietary therapy (weight loss, bariatric surgery, lifestyle modification, low-salt diet), medications, and surgical treatment. IIH is a challenging and serious disorder, which may lead to permanent visual impairment if there is a delay in its diagnosis. Advances in retinal imaging have led to better understanding of the pathogenesis of the disease and prompt diagnosis even in subtle cases can be done using OCT. Surgical interventions should be minimized and only used in cases refractory to medical treatment and in those with rapid progression.

To define the efficacy, complication profile and cost of surgical options for treating idiopathic intracranial hypertension (IIH) with respect to the following endpoints: vision and headache improvement, normal CSF pressure restoration, papilloedema resolution, relapse rate, operative complications, cost of intervention and quality of life. A systematic review of the surgical treatment of IIH was carried out. Cochrane Library, MEDLINE and EMBASE databases were systematically searched from 1985 to 2014 to identify all relevant manuscripts written in English. Additional studies were identified by searching the references of retrieved papers and relative narrative reviews. Forty-one (41) studies were included (36 case series and 5 case reports), totalling 728 patients. Three hundred forty-one patients were treated with optic nerve sheath fenestration (ONSF), 128 patients with lumboperitoneal shunting (LPS), 72 patients with ventriculoperitoneal shunting (VPS), 155 patients with venous sinus stenting and 32 patients with bariatric surgery. ONSF showed considerable efficacy in vision improvement, while CSF shunting had a superior headache response. Venous sinus stenting demonstrated satisfactory results in both vision and headache improvement along with the best complication profile and low relapse rate, but longer follow-up periods are needed. The complication rate of bariatric surgery was high when compared to other interventions and visual outcomes have not been reported adequately. ONSF had the lowest cost. No surgical modality proved to be clearly superior to any other in IIH management. However, in certain contexts, a given approach appears more justified. Therefore, a treatment algorithm has been formulated, based on the extracted evidence of this review. The traditional treatment paradigm may need to be re-examined with sinus stenting as a first-line treatment modality.

Idiopathic intracranial hypertension (IIH), also known as benign intracranial hypertension or pseudotumor cerebri, is characterized by increased intracranial pressure secondary to unknown causes. IIH affects both adults and children but predominately young obese females of child-bearing age. IIH gives rise to headache, visual disturbances, and symptoms of raised intracranial pressure. The pathophysiology of IIH remains elusive; postulations include obesity-related hormonal changes, obstruction of the central venous sinus, and hereditary causes. Diagnosing IIH is based on modified Dandy criteria. Diagnosis in children is challenging as symptoms and presentations vary; atypical and delayed presentations are not uncommon. Treatment for IIH includes oral acetazolamide or lumbar puncture; surgical treatment with shunts and optic nerve sheath fenestration is reserved for complicated and recurring cases. Treatment options for children are limited and associated with considerable risks and side effects. Vision loss is debilitating and occurs in 10% of IIH patients. A thorough and collaborative approach in managing IIH is needed. This study reviews the epidemiology, clinical presentation, diagnosis, and management of IIH in adults and pediatric patients.

Introduction: Idiopathic intracranial hypertension (IIH) is a chronic, debilitating condition that typically affects obese women of childbearing age. Higher BMI has been associated with a higher risk of IIH and of its most disabling complication, permanent vision loss. There is still limited evidence on how higher BMI may impact procedural and clinical outcomes in cerebral venous sinus (CVS) stenting for IIH-induced venous hypertension. Hypothesis: Severe obesity may affect CVS stenting outcomes in patients with IIH refractory to medical management. Methods: We performed a retrospective analysis of a multicentric prospectively-maintained registry of patients with medically refractory IIH who underwent venous manometry and CVS stenting. Patients were divided into two groups according to their body mass index (BMI): those with BMI < 40 kg/m 2 , and those with BMI ≥ 40 kg/m 2 . We compared venous pressure gradient resolution (post-stenting mean pressure gradient of 0-3 mmHg), need for restenting by 12 months, and rates of improvement of papilledema, tinnitus, and headache. Results: Three hundred and fourteen patients with IIH underwent CVS stenting, among whom 107 were severely obese (BMI ≥ 40 kg/m 2 ), and 207 had normal to moderately high BMI. Baseline characteristics were similar across the two groups, except for a lower frequency of contraceptive use (p < 0.05) and a higher prevalence of obstructive sleep apnea (p < 0.001) in severely obese patients. Post-stenting venous pressure gradient resolution and rates of symptom improvement were not significantly different across groups. There was a higher but nonsignificant trend of patients requiring re-stenting among severely obese patients (p = 0.14). Conclusions: Compared to those with BMI < 40, our IIH patients with severe obesity had no significant difference in stenting outcomes. Although re-stenting rates were low in both groups, there was a nonsignificant trend towards the need for re-stenting in severely obese patients. This trend highlights the importance of lifestyle modifications and weight loss after venous stenting. Future analysis of IIH with severe obesity will evaluate BMI change after stenting in a larger cohort.

We reviewed the medical case report literature to determine the proportion of cases of idiopathic intracranial hypertension (IIH) that were either inappropriately labelled as IIH or prematurely given this diagnosis. We searched OVID MEDLINE from 2012 to 2022 to identify case reports that diagnosed patients with IIH. Case reports were assessed for diagnostic accuracy using Friedman et al.'s revised diagnostic criteria for primary pseudotumor cerebri syndrome. Our primary outcome was the crude prevalence of inappropriate or premature IIH diagnoses. Our secondary outcome was determining if inaccurate IIH diagnoses were associated with variables such as journal subscription model and impact factor, author affiliation, country of origin, and year of publication. A total of 33/185 case reports (17.8%) either incorrectly labelled a patient as having IIH or did not perform all of the investigations necessary to make a diagnosis of IIH. Some of these studies (4.8%) were believed to still represent 'probable' IIH given the clinical presentation, whereas 13.0% of studies were determined to have mislabelled their patients as having IIH. The most common reason that case reports did not meet diagnostic criteria included: a lack of MRV in atypical patient cases (42.4%, n = 14), no papilledema in addition to a lack of characteristic neuroimaging features (33.3%, n = 11), intracranial hypertension being secondary to another documented cause (12.1%, n = 4), normal LP opening pressure in addition to other factors (12.1%,n = 4), no description of neuroimaging (6.1%, n = 2), and abnormal CSF composition (6.1%, n = 2). Case reports that used the term 'IIH' incorrectly had a significantly lower journal impact factor (2.0 vs. 2.6, p = 0.01). There is a high prevalence of premature or inappropriate diagnoses of IIH in the peer-reviewed case report literature. Adherence to published diagnostic criteria is needed when publishing IIH case reports, and authors are expected to report all relevant data in their report to ensure that an accurate diagnosis is made.

In the current coronavirus disease 2019 (COVID-19) pandemic, health systems are struggling to prioritize care for affected patients; however, physicians globally are also attempting to maintain care for other less-threatening medical conditions that may lead to permanent disabilities if untreated. Idiopathic intracranial hypertension (IIH) is a relatively common condition affecting young females that could lead to permanent blindness if not properly treated. In this article, we provide some insight and recommendations regarding the management of IIH during the pandemic. The diagnosis, follow-up, and treatment methods of IIH during the COVID-19 pandemic period are reviewed. COVID-19, as a mimic of IIH, is also discussed. Diagnosis and follow-up of papilledema due to IIH during the COVID-19 pandemic can be facilitated by nonmydriatic fundus photography and optical coherence tomography. COVID-19 may mimic IIH by presenting as cerebral venous sinus thrombosis, papillophlebitis, or meningoencephalitis, so a high index of suspicion is required in these cases. When surgical treatment is indicated, optic nerve sheath fenestration may be the primary procedure of choice during the pandemic period. IIH is a serious vision-threatening condition that could lead to permanent blindness and disability at a relatively young age if left untreated. It could be the first presentation of a COVID-19 infection. Certain precautions during the diagnosis and management of this condition could be taken that may allow appropriate care to be delivered to these patients while minimizing the risk of coronavirus infection.

Idiopathic intracranial hypertension (IIH) presenting with focal neurological deficits is rare and may mimic acute stroke, complicating timely diagnosis. We report the case of a 43-year-old Malay woman with progressive headache, intermittent visual symptoms, and acute-onset, progressive right-sided weakness over two days. Dilated fundus examination revealed bilateral grade 2 papilledema, and neurological examination showed mild hemiparesis. Non-contrast CT and CT angiography of the brain were normal. Lumbar puncture (LP) demonstrated elevated opening pressure with normal CSF findings. She improved significantly following LP and acetazolamide, with complete resolution of hemiparesis within one week. This uncommon presentation highlights the importance of prompt neuroimaging, early ophthalmological assessment, and LP. IIH should be considered in patients with persistent headache and acute hemiparesis once structural and vascular causes have been excluded.

Neuroimaging is increasingly used to supplement the clinical diagnosis of dementia with Lewy bodies (DLB) by showing reduced occipital metabolism and perfusion and reduced striatal dopaminergic innervation. We aimed to optimize the interpretation of (18)F-FDG PET images for differentiating DLB from Alzheimer disease (AD) and to compare the results with dopamine transporter imaging using (123)I-beta-carbomethoxy-3ss-(4-iodophenyl)tropane ((123)I-beta-CIT) SPECT. Fourteen subjects with a clinical diagnosis of DLB and 10 with AD underwent both (18)F-FDG PET and (123)I-beta-CIT SPECT. Four DLB and 1 AD diagnoses were subsequently confirmed at autopsy. Diagnostic accuracy was calculated for visual interpretation by 3 readers of standard 3-plane and stereotactic surface projection (18)F-FDG PET images, receiver-operating-characteristic analysis of regional (18)F-FDG uptake, and a cutoff value for the striatal-to-occipital binding ratio of beta-CIT defined by receiver-operating-characteristic analysis. Visual interpretation of 3-plane (18)F-FDG PET images had a sensitivity of 83% and specificity of 93% for DLB, slightly higher than the results with the stereotactic surface projection images. Regionally, hypometabolism in the lateral occipital cortex had the highest sensitivity (88%), but relative preservation of the mid or posterior cingulate gyrus (cingulate island sign) had the highest specificity (100%). Region-of-interest analysis revealed that occipital hypometabolism and relative preservation of the posterior cingulate both had a sensitivity of 77% and specificity of 80%. beta-CIT achieved 100% accuracy and greater effect size than did (18)F-FDG PET (Cohen d = 4.1 vs. 1.9). Both (18)F-FDG PET and (123)I-beta-CIT SPECT appear useful for the diagnosis of DLB, although the latter provides more robust results. The cingulate island sign may enhance the specificity of (18)F-FDG PET.

Idiopathic Intracranial Hypertension (IIH) is a disorder characterized by elevated intracranial pressure without an identifiable cause, commonly affecting young obese women. Current treatment strategies, including weight loss, acetazolamide, and surgical interventions, have limitations due to side effects, adherence challenges, and potential complications. Bromocriptine, a dopamine D2 receptor agonist, has emerged as a potential novel therapy due to its metabolic effects. This study aims to evaluate the safety and efficacy of bromocriptine in IIH management through a retrospective cohort analysis. A retrospective analysis was conducted, focusing on patients with IIH. Propensity score matching was applied to balance baseline characteristics, including age, sex, race, and BMI, between the bromocriptine and control groups. Key outcome measures, papilledema, headache severity, refractory IIH status, and acetazolamide dose dependency, were assessed at multiple follow-up intervals. The bromocriptine group demonstrated significant improvement in papilledema and headache severity over 24 months, with early effects observed at one month. There was a marked reduction in refractory IIH (30.66% lower incidence at 24 months, p<0.0001) and reduced dependency on acetazolamide from three months onward (p=0.0246). The safety profile was favorable, with comparable adverse event rates to controls, although allergic skin reactions were noted in the bromocriptine group. Bromocriptine shows promise as an effective and safe therapeutic option for IIH, with sustained improvement in clinical parameters and reduced reliance on conventional treatment. Future randomized controlled trials are needed to confirm these findings and explore optimal dosing strategies.

Cerebral encephaloceles are being recognized as an etiology of drug-resistant epilepsy (DRE). Neuroimaging suggests a link between idiopathic intracranial hypertension (IIH), dural sinus stenosis (DSS), and encephaloceles. One hypothesis is that pulsatile CSF forces, secondary to elevated intracranial pressure, contribute to forming prominent arachnoid villi, which create pockets of CSF and develop encephaloceles. The impact of DSS and IIH management with stent placement for seizure control in patients with DRE and encephaloceles remains poorly understood. In this study, the authors explored the clinical outcomes in this patient population. This single-center case series retrospectively analyzed seizure frequency in 12 patients with DRE due to encephaloceles associated with IIH who underwent cerebral venous sinus stent placement between 2018 and 2024 at the University of Kansas Medical Center. Seizure burden was assessed after stent placement. Twelve patients with encephaloceles and DRE met the inclusion criteria. The mean age was 49 years, and 9 patients were female. The mean BMI was 36.7 kg/m2. The mean age of seizure onset was 42.1 years. All patients had signs of IIH on MRI of the brain: empty sella (n = 8), transverse sinus stenosis (n = 12), and enlarged arachnoid granulations (n = 5). Seven patients underwent lumbar puncture with a mean opening pressure of 27.25 cm H2O. All patients had unilateral temporal lobe epilepsy and encephaloceles involving a sphenoid wing. One patient (8.3%) had an additional lesion in the basal temporal lobe, and another patient (8.3%) had bilateral cerebellar encephaloceles. The mean pre-stent venous pressure gradient was 14.25 mm Hg, and the mean post-stent gradient was 1.25 mm Hg; 50% of patients had a post-stent pressure gradient of 0 mm Hg. Following venous sinus stent insertion and additional surgical interventions, the mean seizure frequency decreased to 0.9 seizures per month. Eleven patients (91.7%) experienced > 50% reduction in seizure frequency postprocedure. The impact of endovascular stent placement for DSS and IIH on seizure burden was investigated. Seizure frequency reduction was observed in all patients. This study demonstrates a prominent role of endovascular stent insertion in patients with encephaloceles and DRE that warrants further exploration.

We aim to provide a comprehensive and updated review on idiopathic intracranial hypertension (IIH), including the most current studies and treatment options. Special focus will be put on recent theories about the pathophysiology, and on newer prospective studies on treatment modalities. The Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) provided evidence supporting acetazolamide as a well tolerated first-line therapy in IIH patients with mild vision loss. Recent studies have shown venous sinus stenting as a well tolerated and effective surgical alternative for patients with refractory IIH. Idiopathic intracranial hypertension is a vision-threatening disorder that predominantly affects obese women of childbearing age. This disorder is becoming more prevalent as the obesity epidemic continues to increase. As our understanding of this disorder continues to evolve, diagnosis and management approaches have changed over time. However, the pathogenesis for IIH remains unclear. Several theories have been proposed, including abnormalities in cerebrospinal dynamics, metabolic causes and genetics. The diagnostic criteria are based on the revised Dandy criteria. Traditionally, treatment was based on clinical experiences and retrospective studies. However, a new, prospective, randomized, controlled trial, the IIHTT, provided evidence-based data to help guide medical therapy. Additionally new, prospective studies are underway for the different surgical alternatives to treat IIH.

Idiopathic Intracranial Hypertension Presenting With Psychiatric Symptoms

Background: Idiopathic intracranial hypertension (IIH) is a rare condition marked by increased intracranial pressure, which, if untreated, can lead to optic nerve damage, higher cerebral blood volume, and increased cerebrospinal fluid (CSF) production. This study aimed to identify the epidemiology, risk factors, follow-up, and management of IIH in Sudanese patients treated at Wadmadani Teaching Hospital between 2022 and 2023. Methods: This cross-sectional study at Wad Madani Teaching Hospital (2022-2023) involved 100 IIH patients. Data on demographics, BMI, hemoglobin levels, and risk factors were collected from medical records and patient interviews. Treatment and follow-up were documented, focusing on Acetazolamide and Topiramate. Statistical analyses included descriptive and bivariate methods to assess relationships between risk factors, treatment responses, and surgical needs. Results: Of the participants, 95% were female and 5% male, with the majority aged 26-30 years (average age: 27.95). Most participants had a BMI in the 26-30 range. Hemoglobin levels above 11 g/dL were recorded in 71% of the patients. The most common risk factors identified were steroid use and elevated BMI. Acetazolamide and Topiramate were the frequently prescribed medications. Surgical intervention was needed in 23% of patients, mainly due to poor response to medical therapy, worsening visual function, and progressive papilledema. There were significant associations between gender, age, risk factors, and the need for surgery. Conclusion: This study sheds light on the demographic and clinical characteristics of IIH, along with common risk factors and treatments, particularly highlighting gender-based differences in surgical interventions. Further research is essential to better understand the gender disparities in IIH and to develop more tailored treatment approaches. Future studies should focus on uncovering the mechanisms of IIH, customizing treatment plans, and improving management practices through collaboration between healthcare providers, researchers, and patients.