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Ventriculoperitoneal (VP) shunt placement, essential for managing hydrocephalus, often risks catheter malpositioning, especially in patients with small ventricles. We present a novel technique combining neuronavigation with intraoperative cone-beam computed tomography using the BrainLab system and Loop-X mobile imaging unit. This approach enables real-time verification of catheter placement by integrating preoperative MRI data with intraoperative CT imaging. In a 12-year-old boy with therapy-refractory idiopathic intracranial hypertension, neuronavigation was guided by the BrainLab Skull Fix and Cushing canula, ensuring precise catheter insertion into the right frontal horn. Post-placement, Loop-X facilitated immediate verification of the catheter's trajectory and positioning, corroborated by postoperative MRI. This technique demonstrated high precision and minimized radiation exposure, emphasizing its utility in reducing revision rates due to suboptimal catheter placement. [ABSTRACT FROM AUTHOR]

Introduction: Neurobrucellosis (NB) can be associated with meningitis and present as a headache with or without meningeal signs. Pseudotumor presentation of NB has been reported to be accompanied by lymphocytic predominant cerebrospinal fluid(CSF) pleocytosis. NB is diagnosed by means of isolation of Brucella from blood or CSF and/or the presence of anti-Brucella antibodies in the CSF. Molecular techniques have been used in chronic or challenging cases of NB. Clinical findings: We report on seven cases of NB presenting with different types of headache and signs of meningeal involvement. In five cases, signs of intracranial hypertension were evident in the form of papilledema, sixth nerve palsy and blurred vision. Diagnosis: MRIs of the brain revealed signs of intracranial hypertension in three patients, basal meningeal enhancement in one patient and white matter lesions in one patient. Brucella serology in the blood and CSF was negative in all patients. It was interesting that four patients had normocellular CSF analysis with normal glucose and protein results. The diagnosis was made by Brucella PCR in all patients. Conclusion: NB should be considered in the differential diagnoses of pseudotumor cerebri syndrome in endemic areas. It is important to employ molecular techniques using sterile CSF samples in the investigation of Brucella. [ABSTRACT FROM AUTHOR]

Background: Idiopathic intracranial hypertension (IIH) is typically characterized by headaches and vision loss. However, neurocognitive deficits are also described. Our study aimed to test the influence of therapeutic lumbar puncture on the latter. Methods: A total of 15 patients with IIH were tested with a battery of neurocognitive tests at baseline and after therapeutic lumbar drainage. Hereby, Logical Memory of the Wechsler Memory Scale—Revised Edition (WMS-R), the California Verbal Learning Test Short Version (CVLT), alertness, selective attention, and word fluency were used. Changes in cognitive functioning in the course of CSF pressure lowering were analysed and compared with age, sex, and education-matched healthy controls. Results: Before intervention, scores of Logical Memory, the RWT, and the HADS-D were significantly lower in IIH patients compared to matched controls. After short-term normalization of CSF pressure, the RWT improved significantly. Additionally, significant positive correlations were found between headache intensity and subjective impairment, as well as between BMI and CSF opening pressure. Conclusions: Our findings confirm lower performance in terms of long-term verbal memory and word fluency compared to controls, as well as depressive symptoms in IIH patients. Significant improvement after short-term normalization of intracranial pressure by means of CSF drainage was seen only for word fluency. This indicates that short-term normalization of CSF pressure is not sufficient to normalize observed neurocognitive deficits. [ABSTRACT FROM AUTHOR]

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. Synopsis: Women with IIH had a higher incidence of obstetrical complications, including preterm deliveries, hypertensive disorders of pregnancy, and congenital anomalies. [ABSTRACT FROM AUTHOR]

Abstract Introduction Neurobrucellosis (NB) can be associated with meningitis and present as a headache with or without meningeal signs. Pseudotumor presentation of NB has been reported to be accompanied by lymphocytic predominant cerebrospinal fluid(CSF) pleocytosis. NB is diagnosed by means of isolation of Brucella from blood or CSF and/or the presence of anti-Brucella antibodies in the CSF. Molecular techniques have been used in chronic or challenging cases of NB. Clinical findings We report on seven cases of NB presenting with different types of headache and signs of meningeal involvement. In five cases, signs of intracranial hypertension were evident in the form of papilledema, sixth nerve palsy and blurred vision. Diagnosis MRIs of the brain revealed signs of intracranial hypertension in three patients, basal meningeal enhancement in one patient and white matter lesions in one patient. Brucella serology in the blood and CSF was negative in all patients. It was interesting that four patients had normocellular CSF analysis with normal glucose and protein results. The diagnosis was made by Brucella PCR in all patients. Conclusion NB should be considered in the differential diagnoses of pseudotumor cerebri syndrome in endemic areas. It is important to employ molecular techniques using sterile CSF samples in the investigation of Brucella.

Abstract Background Childhood 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. Methods A total of 48 children diagnosed with PTC, who were aged

Abstract Background Currently, there is a relative lack of detailed reports regarding clinical presentation and outcome of idiopathic intracranial hypertension in Asians. This study aims to describe the clinical features and treatment outcomes of Korean patients with idiopathic intracranial hypertension. Methods We prospectively recruited patients with idiopathic intracranial hypertension from one hospital and retrospectively analyzed the medical records of 11 hospitals in Korea. We collected data regarding preceding medical conditions or suspected medication exposure, headache phenotypes, other associated symptoms, detailed neuroimaging findings, treatments, and outcomes after 1–2 and 3–6 months of treatment. Results Fifty-nine (83.1% women) patients were included. The mean body mass index was 29.11 (standard deviation, 5.87) kg/m2; only 27 patients (45.8%) had a body mass index of ≥ 30 kg/m2. Fifty-one (86.4%) patients experienced headaches, patterns of which included chronic migraine (15/51 [29.4%]), episodic migraine (8/51 [15.7%]), probable migraine (4/51 [7.8%]), chronic tension-type headache (3/51 [5.9%]), episodic tension-type headache (2/51 [3.9%]), probable tension-type headache (2/51 [3.9%]), and unclassified (17/51 [33.3%]). Medication overuse headache was diagnosed in 4/51 (7.8%) patients. After 3–6 months of treatment, the intracranial pressure normalized in 8/32 (25.0%), improved in 17/32 (53.1%), no changed in 7/32 (21.9%), and worsened in none. Over the same period, headaches remitted or significantly improved by more than 50% in 24/39 patients (61.5%), improved less than 50% in 9/39 (23.1%), and persisted or worsened in 6/39 (15.4%) patients. Conclusion Our findings suggest that the features of Asian patients with idiopathic intracranial hypertension may be atypical (i.e., less likely obese, less female predominance). A wide spectrum of headache phenotypes was observed. Medical treatment resulted in overall favorable short-term outcomes; however, the headaches did not improve in a small proportion of patients.

Background: Childhood 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. Methods: A 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. Results: Of 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). Conclusions: The 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. [ABSTRACT FROM AUTHOR]

Background: 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. Purpose: 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. Material and Methods: 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. Results: 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). Conclusion: 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. [ABSTRACT FROM AUTHOR]

Myelin oligodendrocyte glycoprotein antibody disease (MOGAD) is characterized by multiple phenotypic conditions such as acute disseminated encephalomyelitis, optic neuritis, and myelitis. MOGAD's spectrum is expanding, with potential symptoms of increased intracranial pressure that are similar to idiopathic intracranial hypertension (IIH). We report a boy with new-onset continuous headache and a brain MRI at onset suggesting idiopathic intracranial hypertension (IIH). The patient showed resistance to treatment with acetazolamide and, after one month, developed optic neuritis in the left eye. Laboratory tests documented positive MOG antibodies (anti-MOG) in the serum. The final diagnosis was MOGAD, with the initial symptoms resembling IIH. [ABSTRACT FROM AUTHOR]

Fulminant idiopathic intracranial hypertension (IIH) is a rapid vision-degrading presentation of IIH with limited published studies. This study composed a narrative review of fulminant IIH with the aim of better characterising fulminant IIH presentation and visual outcomes. SCOPUS and PubMed were searched for papers referencing IIH, benign intracranial hypertension, or pseudotumour cerebri. Abstracts were screened for rapid degradation in vision. All studies were required to meet both the modified Dandy and fulminant IIH criteria. Thirty-six studies met the inclusion criteria. Demographics, treatments, and visual outcome data were collected. Case studies made up 69% of the studies and 31% were case series. In total, 72 patients with fulminant IIH were reported, of which 23.6% were paediatric and 96% were female. Surgical intervention occurred in 85% of patients. Anaemia was present in 11% of patients and 85.7% of paediatric patients had a sixth cranial nerve palsy. In conclusion, we propose the following practice guidelines to assist in diagnosing and treating fulminant IIH patients: 1) patients who present with optic disc oedema require urgent visual field testing to evaluate for vision loss; 2) a paediatric patient presenting with a sixth cranial nerve palsy should have a comprehensive eye examination; 3) fulminant IIH can occur in patients with a normal body mass index; and 4) anaemia should be tested for in the setting of fulminant IIH. As little is known about the optimal treatment mechanisms for this presentation, multi-institutional and international collaborations will be a critical step for future research. [ABSTRACT FROM AUTHOR]

Aim: This study aimed to investigate the possible reciprocal relationship between idiopathic intracranial hypertension (IIH) and obstructive sleep apnea (OSA). Material and Methods: This cross-sectional study was conducted from October 2023 to February 2024. Patients with IIH and age and gender-matched controls without IIH were included. Information on age, gender, comorbidities, smoking, and alcohol consumption was recorded. Body mass index (BMI) was calculated, and a BMI ≥30 was considered obese. Berlin questionnaire and STOP-BANG questionnaire were administered to all participants. High risk for OSA was determined if participants responded affirmatively to at least three out of the eight questions on the STOP-BANG questionnaire, or if two out of the three categories showed positive results on the Berlin questionnaire. Results: Sixty patients with IIH and 120 controls participated. There were no significant differences between groups regarding age (p=0.437) and gender distribution (p=0.716). The percentage of obese subjects was significantly higher in the IIH group (p<0.001). The Berlin and STOP-BANG results showed that the IIH group had higher risks for OSA than the control group. Multivariate logistic regression analysis revealed obesity as the only factor independently associated with high-risk classification with the Berlin questionnaire. In the STOP-BANG survey, higher age, male gender, obesity, and hyperlipidemia were independently related to high-risk classification. Conclusion: Obesity is a common risk factor for both OSA and IIH. The coexistence of OSA and IIH may cause increased morbidity and mortality rates in both diseases. Therefore, we recommend that patients with IIH be screened for OSA risk. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: Currently, there is a relative lack of detailed reports regarding clinical presentation and outcome of idiopathic intracranial hypertension in Asians. This study aims to describe the clinical features and treatment outcomes of Korean patients with idiopathic intracranial hypertension. Methods: We prospectively recruited patients with idiopathic intracranial hypertension from one hospital and retrospectively analyzed the medical records of 11 hospitals in Korea. We collected data regarding preceding medical conditions or suspected medication exposure, headache phenotypes, other associated symptoms, detailed neuroimaging findings, treatments, and outcomes after 1–2 and 3–6 months of treatment. Results: Fifty-nine (83.1% women) patients were included. The mean body mass index was 29.11 (standard deviation, 5.87) kg/m2; only 27 patients (45.8%) had a body mass index of ≥ 30 kg/m2. Fifty-one (86.4%) patients experienced headaches, patterns of which included chronic migraine (15/51 [29.4%]), episodic migraine (8/51 [15.7%]), probable migraine (4/51 [7.8%]), chronic tension-type headache (3/51 [5.9%]), episodic tension-type headache (2/51 [3.9%]), probable tension-type headache (2/51 [3.9%]), and unclassified (17/51 [33.3%]). Medication overuse headache was diagnosed in 4/51 (7.8%) patients. After 3–6 months of treatment, the intracranial pressure normalized in 8/32 (25.0%), improved in 17/32 (53.1%), no changed in 7/32 (21.9%), and worsened in none. Over the same period, headaches remitted or significantly improved by more than 50% in 24/39 patients (61.5%), improved less than 50% in 9/39 (23.1%), and persisted or worsened in 6/39 (15.4%) patients. Conclusion: Our findings suggest that the features of Asian patients with idiopathic intracranial hypertension may be atypical (i.e., less likely obese, less female predominance). A wide spectrum of headache phenotypes was observed. Medical treatment resulted in overall favorable short-term outcomes; however, the headaches did not improve in a small proportion of patients. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. Summary: 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. [ABSTRACT FROM AUTHOR]

Background: Idiopathic intracranial hypertension (IIH) or pseudotumor cerebri is a rare disorder. Regarding the latest diagnostic criteria for this disorder, we present a rare case of IIH. Case presentation: The case was an 11-year-old girl referred to our center with generalized body pain and mild left-eye deviation with no other visual symptoms. She had a severe generalized pain especially in her back and prevertebral area. In the neurological examination, there was a mild left sixth nerve palsy and bilateral papilledema. We treated the patient with acetazolamide and to lumbar puncture, which reduced intracranial pressure. The generalized body pain was reduced gradually in ten days, eye deviation in three weeks, and papilledema in two months after treatment. Conclusion: Based on the serious complications of IIH, a thorough assessment of suspicious cases, including ophthalmoscopic examinations, is mandatory. Furthermore, clinicians should consider even rare and unknown specific symptoms in these patients.

Abstract Our understanding of primary (idiopathic) intracranial hypertension has evolved in recent years. There have been efforts to rename the disorder as pseudotumor cerebri syndrome or primary intracranial hypertension. Some studies have suggested a higher threshold opening pressure to define intracranial hypertension. The reported annual incidence varies from 0.6 to 0.9 per 100 000 children around the world. Patients are typically divided into prepubertal and pubertal groups, with pubertal patients having the same risk factors as adults. Prepubertal patients do not share these risk factors. They are more likely to be asymptomatic, have equal gender distributions, and are less likely to be obese. Headache is the most common presenting complaint, followed by vision changes and nausea/vomiting. A newer concept of fulminant intracranial hypertension has emerged, defined as acute onset with rapid progression of visual deficits or papilledema. Quick insertion of a temporary lumbar drain as a bridge while medical management reaches effectiveness improves visual outcomes and helps avoid permanent shunt placement. Headache is typically the first symptom to resolve with treatment, and papilledema resolves in five to six months. Recurrence rates in children and adolescents range from 28.5% to 36.4%, with higher rates after puberty.

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. Resumen: Introducción: El Síndrome de Pseudotumor Cerebri (SPTC) en pacientes prepuberales presenta características que lo diferencian respecto a su presentación en la etapa postpuberal. 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 1 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. Resultado: Se recogieron 28 pacientes, 22 prepuberales y 6 postpuberales, con edad media de 9,04 ± 2,86 años. El 83,3% de los pacientes postpuberales 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 postpuberales.

Resumen: 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. Abstract: 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.

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. 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. 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). 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. [ABSTRACT FROM AUTHOR]

Background: Idiopathic intracranial hypertension is a secondary headache disorder potentially causing visual loss. Neurofilament light chain is a candidate, prognostic biomarker, but further studies of neuronal biomarkers are needed. Our objective was to investigate neurofilament light chain in cerebrospinal fluid (cNfL) and plasma (pNfL), amyloid-beta 42 (Aβ-42), total-tau and phosphorylated-tau in cerebrospinal fluid in new-onset idiopathic intracranial hypertension. Methods: Prospective case-control study including new-onset idiopathic intracranial hypertension and age, sex and BMI matched controls. Biomarkers were compared between patients and controls and related to papilledema, visual fields and opening pressure. Results: We included 37 patients and 35 controls. Patients had higher age-adjusted cNfL (1.4 vs. 0.6 pg/mL, p-adjusted < 0.001), pNfL (0.5 vs. 0.3 pg/mL, p-adjusted < 0.001) and total-tau/Aβ-42 (0.12 vs. 0.11, p-adjusted = 0.039). Significant, positive linear correlations were found between cNfL, pNfL, total-tau/Aβ-42 and opening pressure. Patients with severe papilledema had elevated cNfL compared to mild-moderate papilledema (median cNfL: 4.3 pg/mL (3.7) versus 1.0 pg/mL (1.4), p-adjusted = 0.009). cNFL was inversely associated with perimetric mean deviation (r = −0.47, p-adjusted < 0.001). Conclusions: cNfL, pNfL and total-tau/Aβ-42 were elevated in new-onset idiopathic intracranial hypertension. cNfL was associated with severity of papilledema and visual field defects at diagnosis. This indicates early axonal damage. Neurofilament light chain is a candidate biomarker for disease severity. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Idiopathic intracranial hypertension (IIH) predominantly affects reproductive-aged females with obesity. However, the prevalence and impact of a healthy weight body mass index (BMI) at disease presentation is not known. This study aimed to evaluate the visual and headache outcomes stratified by the presenting BMI. This was a longitudinal prospective cohort study (IIH Life) based on a tertiary neuro-ophthalmology IIH service, University Hospitals Birmingham NHS Trust, United Kingdom, recruiting consecutive patients living with IIH between 2012 and 2021. Those with a presenting BMI were included. The outcome measures included visual outcomes of LogMAR visual acuity, Humphrey visual field perimetric mean deviation (PMD), optical coherence tomography (OCT) measurements, and headache outcomes of frequency, severity, and Headache Impact Test-6 score. Three hundred seventy-five people with IIH and a documented baseline BMI. About 3.7% of the entire cohort had a healthy weight BMI at IIH presentation and 15.5% BMI < 30 kg/m2. The baseline PMD was worse in patients without obesity; however, OCT papilloedema measures were similar. The presence of obesity was associated with a small but significant greater worsening in visual acuity but slower macular ganglion cell layer loss. There was no impact on PMD or papilloedema prognosis related to baseline obesity. The headache outcomes showed heterogeneity, with worse baseline headache frequency in patients with obesity. No BMI group was associated with worse headache outcomes. Patients with a healthy weight BMI or those without obesity at baseline make up a small proportion of IIH patients. BMI at presentation does not appear to influence long-term visual or headache outcomes. [ABSTRACT FROM AUTHOR]

Idiopathic Intracranial Hypertension (IIH) is a condition in which patients have elevated intracranial pressure which does not have an apparent cause. To diagnose IIH, evaluation excluding other causes of elevated pressure must be performed. This typically includes magnetic resonance imaging (MRI) of the brain and venous sinuses. Despite there being known radiographic signs suggestive of IIH on MRI, there currently are no established correlations between radiographic findings and visual outcomes. Previous work revealed diffusion weighted imaging (DWI), a qualitative measurement on MRI, correlated with clinical findings (i.e presence and grade of papilloedema), but not visual outcomes. We hypothesized that the apparent diffusion coefficient (ADC), a quantitative value obtained during clinical MRI, may correlate with visual outcomes. We conducted a retrospective chart review to correlate findings on the ADC sequence on routine brain MRIs in patients with papilloedema with visual outcomes. In 49 patients with IIH, this study shows the ADC in the retrobulbar optic nerve to be 1,487 ± 559 × 10−6 mm2 /s, 15% lower than reported value of 1744 ± 413 in healthy controls. This suggests that there is true restricted diffusion in patients with IIH and papilloedema, as previously reported visually by MRI. However, there was no significant correlation with clinical outcomes of papilloedema grade, mean deviation on standard perimetry, and retinal nerve fibre layer (RNFL) on optical coherence tomography (OCT). We discuss reasons why the ADC measurement may be confounded by motion and partial volume and propose methods that may reduce these confounders for future studies. [ABSTRACT FROM AUTHOR]

Currently no guidelines for repeating a lumbar puncture to guide management in primary intracranial hypertension (PIH) exist. 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. 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 H 2 O, 30.7 cm H 2 O, and 31.1 cm H 2 O, 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 H 2 O (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). 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. [ABSTRACT FROM AUTHOR]

Background: Idiopathic intracranial hypertension (IIH) or pseudotumor cerebri is a rare disorder. Regarding the latest diagnostic criteria for this disorder, we present a rare case of IIH. Case presentation: The case was an 11-year-old girl referred to our center with generalized body pain and mild left-eye deviation with no other visual symptoms. She had a severe generalized pain especially in her back and prevertebral area. In the neurological examination, there was a mild left sixth nerve palsy and bilateral papilledema. We treated the patient with acetazolamide and to lumbar puncture, which reduced intracranial pressure. The generalized body pain was reduced gradually in ten days, eye deviation in three weeks, and papilledema in two months after treatment. Conclusion: Based on the serious complications of IIH, a thorough assessment of suspicious cases, including ophthalmoscopic examinations, is mandatory. Furthermore, clinicians should consider even rare and unknown specific symptoms in these patients. [ABSTRACT FROM AUTHOR]

Key Clinical Message: 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. [ABSTRACT FROM AUTHOR]

Introduction: Pseudotumor cerebri is one of the complex diseases in the field of neurology and neurosurgery. Although it’s classical form manifests with headache and blurred vision, and papilledema is seen in the ophthalmological examination, but its clinical manifestations can be without blurred vision and papilledema, and even without headache.Here, we present a diagnostically challenging pseudotumor cerebri case who presented only with refractory tinnitus and had increased intracranial pressure, and the tinnitus was completely resolved with transverse sinus stenting. Conclusion: This case showed that pseudotumor cerebri can have atypical manifestations such as resistant tinnitus without papilledema, and in these cases, intracranial pressure should be measured and its causes, especially cerebral venous sinus stenosis should be investigated and treated.