Navigating the Enigma: A Comprehensive Review of Idiopathic Intracranial Hypertension.

An unidentified source of increased intracranial pressure is a hallmark of idiopathic intracranial hypertension (IIH), also referred to as pseudotumor cerebri. It mainly affects young, obese women, yet it can happen to anyone, regardless of age, gender, or weight. IIH presents with symptoms such as headaches, visual disturbances, and pulsatile tinnitus and can lead to severe complications, including vision loss, if left untreated. Diagnosis involves clinical evaluation, neuroimaging, and lumbar puncture, while management options include medical interventions and surgical procedures. This review provides a comprehensive overview of IIH, including its etiology, clinical presentation, epidemiology, complications, management approaches, and challenges. Increased awareness among healthcare professionals, standardized diagnostic criteria, and further research efforts are essential for improving outcomes and quality of life for individuals with IIH.

Case report: Association between PTEN-gene variant and an aggressive case of multiple dAVFs.

Introduction: Mutations of the phosphatase and tensin homolog (PTEN) gene have been associated with a spectrum of disorders called PTEN hamartoma tumor syndrome, which predisposes the individual to develop various types of tumors and vascular anomalies. Its phenotypic spectrum includes Cowden syndrome (CS), Bannayan-Riley-Ruvalcaba syndrome (BRRS), Proteus syndrome, autism spectrum disorders (ASD), some sporadic cancers, Lhermitte-Duclos disease (LDD), and various types of associated vascular anomalies. Clinical presentation: A previously healthy 27-year-old woman was experiencing visual scintillating scotomas and mild chronic headaches for the past 2 years. The initial computed tomographic (CT) and magnetic resonance imaging (MRI) scans did not reveal any abnormalities, but the possibility of pseudotumor cerebri was considered. Furthermore, a cerebral angiogram showed a posterior fossa dural arteriovenous fistula (dAVF), which was initially treated through embolization. However, in spite of proper treatment, this patient experienced multiple recurrent dAVFs in different locations, requiring multiple embolizations and surgeries. Despite exhibiting altered cerebral perfusion and hemodynamics, the patient did not display any significant symptoms until she experienced a sudden stroke resulting from deep venous thrombosis, which was not associated with any medical procedures or medication use. A comprehensive analysis was performed due to the aggressive nature of the dAVFs. Surprisingly, exome sequencing of a blood sample revealed a PTEN gene variant in chromosome 10, indicative of Cowden syndrome. However, no tumors or other vascular lesions were detected in other systems that would constitute Cowden syndrome. Conclusion: The rapid formation of multiple and complex dAVFs, coupled with not meeting the criteria for any other PTEN-related syndrome, unequivocally leads to the presentation of a novel phenotype of the PTEN germline variant.

Idiopathic Intracranial Hypertension After Abrupt Cessation of Medication: A Case Report of Abrupt Glucagon-Like Peptide-1 (GLP-1) Receptor Agonist Cessation and Review of the Literature.

PURPOSE OF REVIEW: The purpose of this review is two-fold: (1) to discuss a case report of idiopathic intracranial hypertension (IIH) after abrupt cessation of a glucagon-like peptide-1 (GLP-1) receptor agonist with resultant rapid weight gain and (2) to review the literature regarding the potential role of GLP-1 receptor agonists in the treatment of IIH as well as potential pitfalls. RECENT FINDINGS: GLP-1 receptor agonists have become widely used to treat obesity. Obesity is a known risk factor for the development of IIH, though the precise pathophysiology is unclear. GLP-1 receptor agonists may help treat IIH by promoting weight loss, lipolysis of adipose tissue, and potentially decreasing the secretion of CSF, as was seen in rat models. Abrupt cessation of GLP-1 receptor agonists can result in regaining lost weight rapidly. In the case that we present, the patient stopped duraglutide abruptly due to lack of insurance coverage and regained the weight she had lost within a month. She subsequently developed IIH. GLP-1 receptor agonists have the potential to help treat IIH; however, this class of medication needs to be used carefully, as cessation of the medication and resultant rapid weight gain can result in IIH.

The Pseudotumor Cerebri Syndrome.

Pseudotumor cerebri syndrome is a syndrome of increased cerebrospinal fluid pressure without ventriculomegaly, mass lesion, or meningeal abnormality. It is either primary (idiopathic intracranial hypertension, IIH) or secondary. A secondary cause is unlikely when adhering to the diagnostic criteria. Permanent visual loss occurs if undetected or untreated, and the associated headaches may be debilitating. Fulminant disease may result in blindness despite aggressive treatment. This study addresses the diagnosis and management of IIH including new insights into the pathobiology of IIH, updates in therapeutics and causes of overdiagnosis.

The diagnostic value of MRI findings in pediatric idiopathic intracranial hypertension: a case-control study.

BACKGROUND: Idiopathic intracranial hypertension (IIH) is a rare medical condition in children. Based on the different radiological findings reported in various studies in pediatric IIH, this study was conducted to determine the diagnostic value of MRI findings in diagnosing IIH in children. METHODS: In this retrospective study, the medical records of all children aged 1 to 18 years who visited Ghaem Hospital in Mashhad, Iran, between 2012 and 2022 and were diagnosed with IIH were gathered. Forty-nine cases of children with IIH and 48 control cases of children with the first unprovoked seizure with no indications of increased intracranial pressure for comparison were selected. Patient demographic information and MRI findings were extracted. The comparison between different MRI findings in the case and control groups was conducted using statistical tests. RESULTS: In the case group, the mean diameter of the subarachnoid space expansion around the optic nerve was 5.96 ± 1.21, compared to 4.79 ± 0.33 in the control group, with statistically significant difference (P < 0.001). All the patients with flattening of the posterior globe or transverse sinus stenosis were in the case group, and the frequency of these findings in the case group was significantly higher than in the control group (P < 0.001). The majority of patients (95.5%) classified under category 3 and 4 of empty sella were part of the case group, and the statistical test results indicated a significant difference between the two groups (P < 0.001). The optic nerve sheath diameter cut-off of 5.35 mm, when used for expansion of the subarachnoid space around the optic nerve, with a sensitivity of 82% and a specificity of 100% in diagnosing IIH. CONCLUSION: The most reliable diagnostic indicators for diagnosing IIH in children are perioptic subarachnoid space expansion with high sensitivity, and posterior globe flattening and transverse sinus stenosis with high specificity.

Diagnosis and treatment of disorders of intracranial pressure: consensus statement of the Spanish Society of Neurology's Headache Study Group.

Primary intracranial pressure disorders include idiopathic intracranial hypertension and spontaneous intracranial hypotension. Remarkable advances have been made in the diagnosis and treatment of these 2entities in recent years. Therefore, the Spanish Society of Neurology's Headache Study Group (GECSEN) deemed it necessary to prepare this consensus statement, including diagnostic and therapeutic algorithms to facilitate and improve the management of these disorders in clinical practice. This document was created by a committee of experts belonging to GECSEN, and is based on a systematic review of the literature, incorporating the experience of the participants, and establishes practical recommendations with levels of evidence and grades of recommendation.

Enhancing Diagnostic Accuracy Through Neuroimaging Revisions in Pediatric Pseudotumor Cerebri Syndrome: A Cross-Sectional Study.

BACKGROUND: This cross-sectional study aimed to report all neuroimaging findings suggestive of raised intracranial pressure in children with pseudotumor cerebri syndrome (PTCS), before and after re-review by two neuroradiologists. METHODS: We included 48 children aged <18 years diagnosed with PTCS between 2016 and 2021. Clinical and radiological data were obtained from their medical files. Two neuroradiologists independently re-reviewed all neuroimages, and the average of their assessments was compared with the initial neuroimaging reports; an additional review was done to analyze inter- and intraclass correlation. RESULTS: The initial neuroimaging reports showed under-reporting of findings, with only 26 of 48 (54.1%) patients identified with abnormal reports. After revision, the proportion of the reported findings increased to 44 of 48 (91.6%). Distention of the perioptic space was the most commonly reported finding after revision (36.5 of 48; 76%). Flattening of the posterior globe and empty sella were initially under-reported but improved after revision. Moreover, several findings suggestive of increased intracranial pressure not mandated by Friedman criteria were identified, such as narrowing of the Meckel cave, posterior displacement of the pituitary stalk, and narrowing of the cavernous sinus. Analysis of associations between neuroimaging findings and demographic and clinical characteristics yielded no statistically significant results. The inter- and intraclass correlation results demonstrated a significant agreement between raters and within each rater's assessment (P < 0.05). CONCLUSIONS: This study highlights the impact of image revision in enhancing PTCS diagnosis. Intra- and interclass correlations underscore the reliability of the review process, emphasizing the importance of meticulous image analysis in clinical practice.

Pseudotumor cerebri with status epilepticus in a child: A rare presentation of vitamin D deficiency.

Pseudotumor cerebri (PTC) encompasses a constellation of symptoms caused by elevated intracranial pressure of unclear etiology. Various associations have been described, rarely hypovitaminosis D. Vitamin D deficiency should be considered as a potential etiology of neurological manifestations like PTC and seizures in children. Early diagnosis and correction of vitamin D deficiency is key to preventing morbidity and achieving good outcomes.

Ultrasound-guided initial diagnosis and follow-up of pediatric idiopathic intracranial hypertension.

BACKGROUND: Idiopathic intracranial hypertension in children often presents with non-specific symptoms found in conditions such as hydrocephalus. For definite diagnosis, invasive intracranial pressure measurement is usually required. Ultrasound (US) of the optic nerve sheath diameter provides a non-invasive method to assess intracranial pressure. Transtemporal US allows imaging of the third ventricle and thus assessment for hydrocephalus. OBJECTIVE: To investigate whether the combination of US optic nerve sheath and third ventricle diameter can be used as a screening tool in pediatric idiopathic intracranial hypertension to indicate elevated intracranial pressure and exclude hydrocephalus as an underlying pathology. Further, to analyze whether both parameters can be used to monitor treatment outcome. MATERIALS AND METHODS: We prospectively included 36 children with idiopathic intracranial hypertension and 32 controls. Using a 12-Mhz linear transducer and a 1-4-Mhz phased-array transducer, respectively, optic nerve sheath and third ventricle diameters were determined initially and during the course of treatment. RESULTS: In patients, the mean optic nerve sheath diameter was significantly larger (6.45±0.65 mm, controls: 4.96±0.32 mm) and the mean third ventricle diameter (1.69±0.65 mm, controls: 2.99±1.31 mm) was significantly smaller compared to the control group, P<0.001. Optimal cut-off values were 5.55 mm for the optic nerve sheath and 1.83 mm for the third ventricle diameter. CONCLUSIONS: The combined use of US optic nerve sheath and third ventricle diameter is an ideal non-invasive screening tool in pediatric idiopathic intracranial hypertension to indicate elevated intracranial pressure while ruling out hydrocephalus. Treatment can effectively be monitored by repeated US, which also reliably indicates relapse.

Idiopathic intracranial hypertension and obstetric and neonatal outcomes: A 1:20 matched study from a population database.

OBJECTIVE: Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is a common pathology in reproductive-aged women, although data regarding pregnancy outcomes are scarce. In the present study, we aimed to compare pregnancy and perinatal outcomes between women who suffered from IIH to those who did not. METHODS: A retrospective cohort study using the Healthcare Cost and Utilization Project, Nationwide Inpatient Sample. All pregnant women who delivered or had a maternal death in the US (2004-2014) were included. Women with an ICD-9 diagnosis of IIH before or during pregnancy were matched to controls without IIH according to age, race, insurance type, and income quartile, in a 1:20 ratio. Pregnancy, delivery, and neonatal outcomes were compared between the two groups. RESULTS: Overall, 9 096 788 deliveries were identified. Of these, 1454 women (0.016%) had a diagnosis of IIH (study group) and were compared to 29 080 women without IIH (control group). Women with IIH, compared to those without, were more likely to be obese (body mass index >30 kg/m2) and suffer from pregestational diabetes mellitus and chronic hypertension (P < 0.001, all). After adjusting for confounders, patients in the IIH group, compared to those without, had a higher rate of pregnancy-induced hypertension (aOR 1.82, 95% CI: 1.57-2.1, P < 0.001), pre-eclampsia (aOR 1.98, 95% CI: 1.61-2.45, P < 0.001), preterm delivery (aOR 1.88, 95% CI: 1.59-2.23, P < 0.001), CD (aOR 2.41, 95% CI: 2.12-2.73, P < 0.001), wound complications (aOR 3.2, 95% CI: 1.89-5.42, P < 0.001), and congenital anomalies (aOR 2.18, 95% CI: 1.4-3.4, P < 0.001). CONCLUSION: Women with IIH had a higher incidence of obstetrical complications, including preterm deliveries, hypertensive disorders of pregnancy, and congenital anomalies.

Síndrome de pseudotumor cerebri en la población pediátrica: características clínicas, tratamiento y pronóstico / Pseudotumor cerebri in the paediatric population: clinical features, treatment and prognosis

Introducción El síndrome de pseudotumor cerebri (SPTC) en pacientes prepuberales presenta características que lo diferencian respecto a su presentación en la etapa pospuberal. Nuestro objetivo es describir las características de los pacientes diagnosticados de SPTC pediátrico en nuestro centro y compararlas en función de su estado puberal. Pacientes y métodos Se incluyeron a los pacientes diagnosticados de SPTC en un hospital de tercer nivel entre los años 2006 y 2019 con edades comprendidas entre uno y 18 años que cumplieran los criterios diagnósticos actualizados del SPTC. Se clasificaron en función de su estado puberal y peso corporal. Posteriormente, se analizaron los datos de las punciones lumbares, estudios de neuroimagen, valoraciones oftalmológicas, así como el régimen terapéutico recibido a lo largo de su seguimiento. Resultados Se recogieron 28 pacientes, 22 prepuberales y seis pospuberales, con edad media de 9,04 ± 2,86 años. El 83,3% de los pacientes pospuberales eran varones presentando sobrepeso/obesidad en el 66,7%. Eran varones el 27% de los pacientes prepuberales, de ellos asociaban sobrepeso el 31,8%. La sintomatología más frecuente fue cefalea (89,9%) y visión borrosa (42,9%). Todos los pacientes presentaron papiledema; un 21,4% de los casos presentaron parálisis del VI par. Se identificó un posible desencadenante en un 28,6%. El 19% presentaron recurrencia clínica, siendo todos ellos prepuberales. La resolución clínica completa se produjo en el 55,6% de los pacientes. Conclusión Pacientes con SPTC presentan menor prevalencia de obesidad en la etapa prepuberal, junto con un mayor porcentaje de etiologías secundarias y tasa de recurrencia que los pacientes pospuberales. (AU)

Introduction Pseudotumor cerebri (PC) in prepubertal patients displays certain characteristics that differentiate it from its presentation at the postpubertal stage. The aim of this study is to describe the characteristics of paediatric patients diagnosed with PC at our centre and to compare them according to their pubertal status. Patients and methods We included patients aged between 1 and 18 years who were diagnosed with PC in a tertiary-level hospital between 2006 and 2019 and who met the updated diagnostic criteria for PC. They were classified according to body weight and pubertal status. Subsequently, we analysed results from lumbar punctures, neuroimaging studies, ophthalmological assessments, and treatments received during follow-up. Results We included 28 patients, of whom 22 were of prepubertal age and 6 were of postpubertal age. The mean age (standard deviation) was 9.04 (2.86) years. Among the postpubertal patients, 83.3% were boys, 66.7% of whom presented overweight/obesity. In the group of prepubertal patients, 27% were boys, 31.8% of whom were overweight. The most frequent symptoms were headache (89.9%) and blurred vision (42.9%). All patients presented papilloedema, and 21.4% manifested sixth nerve palsy. Possible triggers were identified in 28.6% of cases. Nineteen percent of patients presented clinical recurrence, all of whom were prepubertal patients. Complete clinical resolution was achieved in 55.6% of patients. Conclusion Prepubertal patients with PC show lower prevalence of obesity, higher prevalence of secondary aetiologies, and higher recurrence rates than postpubertal patients. (AU)

Idiopathic Hypertension with Multiple Cranial Nerve Palsies: A Case Report and Literature Review.

We report a rare case of idiopathic intracranial hypertension (IIH) with multiple cranial nerve palsies involving cranial nerves VI, VII, IX, and X in a 32-year-old female who had no prior comorbidities. Her condition improved rapidly on a ten-day regimen of acetazolamide and tablet topiramate. IIH should be considered in every patient presenting with persistent headache and multiple cranial nerve abnormalities. This paper also includes a literature review of similar cases.

Persistent Elevation of Opening Pressure Despite Treatment and Symptom Resolution in Pediatric Intracranial Hypertension.

BACKGROUND: Currently no guidelines for repeating a lumbar puncture to guide management in primary intracranial hypertension (PIH) exist. METHODS: An institutional database of patients 18 years and younger followed in the institution's pediatric intracranial hypertension clinic was examined for opening pressure changes in PIH at diagnosis, before medication wean, and following medication wean, as well as to examine whether measurements at the time of diagnosis differed between those with and without disease recurrence. RESULTS: Forty-two patients were included in this study; 36% were male and the mean age at diagnosis was 11.01 years. Treatment duration averaged 9.68 months in those without recurrence and 8.5 months in those with recurrence. Average body mass index percentile of patients with disease recurrence was 83.7 and 72.1 in those without recurrence (P = 0.16). Average opening pressure values of all patients at diagnosis, prewean, and postwean was 36.53 cm H2O, 30.7 cm H2O, and 31.1 cm H2O, respectively. There was no statistically significant difference in opening pressures across these time points (P = 0.14). The change in opening pressure from diagnosis to postwean was statistically significant with a reduction of 5.18 cm H2O (P = 0.04). There was no statistical difference between change in opening pressure at diagnosis versus postwean between those with and without recurrence (P = 0.17). CONCLUSIONS: This clinical observational study suggests that mean opening pressure measurements in patients with PIH remain elevated both before and after medication wean despite papilledema resolution and patient-reported PIH symptoms. Clinically, this suggests that other features such as signs of optic disc edema and symptoms should be used to inform a clinical determination of disease recurrence and treatment course.

National trends in the endovascular and surgical treatment of idiopathic intracranial hypertension.

BACKGROUND: The pattern of surgical treatments for Idiopathic Intracranial Hypertension (IIH) in the United States is not well-studied, specifically the trend of utilizing endovascular venous stenting (EVS) as an emerging technique. METHODS: In this cross-sectional study, we aimed to explore the national trend of utilizing different procedures for the treatment of IIH including EVS, Optic Nerve Sheath Fenestration (ONSF), and CSF Shunting, with a focus on novel endovascular procedures. Moreover, we explored rates of 90-day readmission and length of hospital stay following different procedures, besides the effects of sociodemographic and clinical parameters on procedure choice. RESULTS: 36,437 IIH patients were identified from records between 2010 and 2018. Those in the EVS group were 29 years old on average, and 93.4 % were female. Large academic hospital setting was independently associated with higher EVS utilization, while other factors were not predictive of procedure choice. The proportion of EVS use in IIH hospitalizations increased significantly from 2010 to 2018 (P < 0.001), while there was a relative decline in the frequency of shunting procedures (P = 0.001), with ONSF utilization remaining stable (P = 0.39). The rate of 90-day readmission and length of hospital stay were considerably lower following EVS compared to other procedures (Ps < 0.001). CONCLUSION: This study presents novel population-level data on national trends in the frequency and outcome of EVS for IIH therapy. EVS was associated with shorter length of hospital stays and fewer readmission rates. In addition, a continuous increase in venous stenting compared to other procedures suggests an increasing role for endovascular therapies in IIH.

Pseudotumor cerebri in the paediatric population: clinical features, treatment and prognosis.

INTRODUCTION: Pseudotumor cerebri (PC) in prepubertal patients displays certain characteristics that differentiate it from its presentation at the postpubertal stage. The aim of this study is to describe the characteristics of paediatric patients diagnosed with PC at our centre and to compare them according to their pubertal status. PATIENTS AND METHODS: We included patients aged between 1 and 18 years who were diagnosed with PC in a tertiary-level hospital between 2006 and 2019 and who met the updated diagnostic criteria for PC. They were classified according to body weight and pubertal status. Subsequently, we analysed results from lumbar punctures, neuroimaging studies, ophthalmological assessments, and treatments received during follow-up. RESULTS: We included 28 patients, of whom 22 were of prepubertal age and 6 were of postpubertal age. The mean age (standard deviation) was 9.04 (2.86) years. Among the postpubertal patients, 83.3% were boys, 66.7% of whom presented overweight/obesity. In the group of prepubertal patients, 27% were boys, 31.8% of whom were overweight. The most frequent symptoms were headache (89.9%) and blurred vision (42.9%). All patients presented papilloedema, and 21.4% manifested sixth nerve palsy. Possible triggers were identified in 28.6% of cases. Nineteen percent of patients presented clinical recurrence, all of whom were prepubertal patients. Complete clinical resolution was achieved in 55.6% of patients. CONCLUSION: Prepubertal patients with PC show lower prevalence of obesity, higher prevalence of secondary aetiologies, and higher recurrence rates than postpubertal patients.

Surgical management of pseudotumor cerebri syndrome: A single center experience with endoscopic optic nerve decompression and CSF diversion procedures.

OBJECTIVES: Pseudotumor cerebri syndrome (PTC) is a chronic disorder, which is initially treated by conservative measures, yet surgery is inevitable in case of progressive worsening of vision or headache despite medical treatment. The surgical management is controversial including CSF diversion procedures and optic decompression. The purpose of this study was to evaluate the efficacy of different surgeries in PTC and to present surgical outcomes in a single center. METHODS: This retrospective study included the patients with PTC who were operated by endoscopic optic nerve decompression (EOND), ventriculoperitoneal (VP) and lumboperitoneal (LP) shunting. Surgical outcomes, i.e. visual acuity, visual field, papilledema and headache were compared according to type of surgery. Surgical complications were noted. RESULTS: Seventeen of 36 patients were treated with shunting, 14 with EOND and 5 patients with both EOND and shunting. No statistical significance was observed between CSF diversion procedures and EOND concerning clinical outcomes. The improvement rate of papilledema was higher with VPS (p = 0574) while more patients benefitted from LPS regarding visual field and acuity (p = 0471 and p = 0718, respectively). The best treatment response for headache was in shunt implemented patients (VPS and LPS) with a rate of 88.2% followed by EOND (78.6%) and both surgeries (60%)(p = 0.294). Gender and BMI were significant predictors of improved papilledema (p = 0.034). CONCLUSIONS: Our study demonstrated comparable results between shunting and EOND, regarding the efficacy on surgical outcomes. EOND is quite effective for headache besides its advantage on treatment-refractory visual loss. Shunting may offer sustained relief of symptoms when compared with EOND.

Spontaneous Cerebrospinal Fluid Leak in a Patient with Asymptomatic Idiopathic Intracranial Hypertension.

BACKGROUND: Spontaneous cerebrospinal fluid (CSF) leaks occur when there is a tear in the dura mater. Spontaneous CSF leaks are rare, and often associated with conditions like intracranial hypertension, connective tissue disorders, or congenital defects in the dura mater. CASE REPORT: The patient was a 66-year-old woman who presented to the Emergency Department with clear, positional nasal discharge from her left nostril for 1 week. She had a history of chronic headaches, which seemed to have been relieved around the time of onset of her rhinorrhea. Diagnostic imaging, including computed tomography and magnetic resonance imaging scans, confirmed the presence of a CSF leak and a left temporal meningoencephalocele. The patient subsequently underwent surgical repair of the leak and ventriculoperitoneal shunt placement, and was discharged home in stable condition. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Early detection of CSF leaks require a thorough history and physical examination, and is crucial in preventing potentially life-threatening complications such as meningitis and intracranial abscesses.

Interrelation Between Cerebrospinal Fluid Pressure, Intracranial Morphology and Venous Hemodynamics Studied by 4D Flow MRI.

PURPOSE: To quantify the effects of CSF pressure alterations on intracranial venous morphology and hemodynamics in idiopathic intracranial hypertension (IIH) and spontaneous intracranial hypotension (SIH) and assess reversibility when the underlying cause is resolved. METHODS: We prospectively examined venous volume, intracranial venous blood flow and velocity, including optic nerve sheath diameter (ONSD) as a noninvasive surrogate of CSF pressure changes in 11 patients with IIH, 11 age-matched and sex-matched healthy controls and 9 SIH patients, before and after neurosurgical closure of spinal dural leaks. We applied multiparametric MRI including 4D flow MRI, time-of-flight (TOF) and T2-weighted half-Fourier acquisition single-shot turbo-spin echo (HASTE). RESULTS: Sinus volume overlapped between groups at baseline but decreased after treatment of intracranial hypotension (p = 0.067) along with a significant increase of ONSD (p = 0.003). Blood flow in the middle and dorsal superior sagittal sinus was remarkably lower in patients with higher CSF pressure (i.e., IIH versus controls and SIH after CSF leak closure) but blood flow velocity was comparable cross-sectionally between groups and longitudinally in SIH. CONCLUSION: We were able to demonstrate the interaction of CSF pressure, venous volumetry, venous hemodynamics and ONSD using multiparametric brain MRI. Closure of CSF leaks in SIH patients resulted in symptoms suggestive of increased intracranial pressure and caused a subsequent decrease of intracranial venous volume and of blood flow within the superior sagittal sinus while ONSD increased. In contrast, blood flow parameters from 4D flow MRI did not discriminate IIH, SIH and controls as hemodynamics at baseline overlapped at most vessel cross-sections.

Assessment of glymphatic dysfunction in pediatric idiopathic intracranial hypertension: insights from quantitative diffusivity and perivascular spaces analysis-a case-control study.

Background: The impaired drainage of cerebrospinal fluid through the glymphatic system is thought to play a role in the idiopathic intracranial hypertension (IIH) pathophysiology. Limited data exist regarding the glymphatic system's involvement in pediatric patients with IIH. Therefore, the study's objective was to quantitatively evaluate alterations in parenchymal diffusivity and magnetic resonance imaging (MRI)-visible dilated perivascular spaces (PVS) as imaging indicators of glymphatic dysfunction in pediatric patients with IIH. Methods: Patients diagnosed with IIH in 2017-2022 in a single tertiary center (Sheba Medical Center, Israel) were retrospectively reviewed. Twenty-four pediatric patients were enrolled. All patients underwent clinical 3-T brain MRI. The control group included 24 age- and gender-matched healthy subjects with a normal-appearing brain on imaging. We used automatic atlas-based diffusion-weighted imaging analysis to determine regional diffusivity of the thalamus, caudate, putamen, globus pallidus, hippocampus, amygdala, and brain stem. PVS were evaluated using a semi-quantitative rating scale on T2-weighted images. Variables were compared using the Mann-Whitney test. Multivariate analysis of covariance was used to test for differences between controls and IIH patients. Results: No significant differences in regional brain diffusivity were observed between individuals with IIH and healthy controls (P=0.14-0.91 for various brain regions). The number of visible PVS was comparable between patients with IIH and the control group across all evaluated sites (P=0.12-0.74 for various brain regions). Conclusions: Pediatric IIH patients exhibited similar patterns of parenchymal diffusivity and PVS compared to age-matched controls. These findings do not support the hypothesis that the glymphatic system may play a role in the pathophysiology of pediatric IIH, although previously postulated. However, employing more sophisticated magnetic resonance (MR) techniques could enhance the sensitivity in uncovering underlying glymphatic dysfunction. Further research is warranted to validate and explore this association in larger cohorts and investigate the underlying mechanisms involved in IIH.

A systematic review on idiopathic intracranial hypertension comorbid with polycystic ovarian syndrome and its consequences.

BACKGROUND: A few publications have examined the frequency and medical implications of individuals with idiopathic intracranial hypertension (IIH) and polycystic ovarian syndrome (PCOS), but the findings have been inconclusive. IIH and PCOS both mainly affect obese women of reproductive age and have an impact on women's health at various levels. The aim of this systematic review was to compare the prevalence and association between comorbid IIH and PCOS, and their effect on such aspects as metabolism, abnormalities in hormone levels, and reproduction. METHODS: The criterion for inclusion was a research study of patients suffering from both syndromes. We excluded review articles, case reports, and papers with an inappropriate study design, patient population or outcomes. Electronic databases PubMed, Scopus, Web of Science and gray literature were searched to retrieve studies published from inception to June 10, 2023. The risk of bias assessment was conducted utilizing Covidence software and by discussion between co-authors. RESULTS: After applying our inclusion/exclusion criteria, we consolidated the initial pool to a final selection of 9 articles, and 2185 patients with comorbidity of these two conditions. The prevalence of PCOS among patients with IIH was observed, with incidence rates ranging from 15.5% to 57%, which is up to 8 times greater, than the 4-10% prevalence of PCOS in the general population. These data may be valuable in clinical practice for both neurologists and gynecologists. DISCUSSION: PCOS associated with obesity facilitates concurrence of IIH. The diagnosis of concurrence of IIH and PCOS may have significant clinical implications for patients due to the accompanying hormonal disorders, obesity-related consequences, and fertility issues. Other No systematic review was found. We have registered the study in PROSPERO (International prospective register of systematic reviews), and the registration number is CRD42023437485.