Search Results (61)

Background: Idiopathic intracranial hypertension (IIH), also known as primary pseudotumor cerebri, is characterized by increased intracranial pressure (ICP) without an identifiable cause. It can lead to significant morbidity, including permanent vision loss, especially in younger children. The exact cause of IIH is still unclear, but excess adiposity seems to be a key risk factor. Current treatment options are unsatisfactory, but research is exploring novel therapies targeting obesity-related mechanisms. Methods: Narrative review of the literature aimed at summarizing current knowledge regarding the epidemiology, pathophysiology, clinical features, treatment options and long-term outcomes for pediatric IIH, with a particular focus on the link with obesity. Results: The incidence of IIH is rising, mirroring the obesity epidemic. Excess adiposity, predominantly visceral, might cause IIH through several factors such as decreased venous return, hormone dysregulation, inflammation, obstructive sleep apnea, and dysfunction of the glymphatic system. The extent of weight loss required and the most appropriate strategy to achieve it are still uncertain. Given the difficulty in achieving and maintaining weight loss with dietary strategies, bariatric surgery and weight loss medications are emerging as effective options for long-term remission of both obesity and IIH. Conclusions: IIH is a rare and poorly understood disease. At present, weight loss represents the only treatment that addresses the pathophysiology of IIH. The role and potential as standalone or synergistic therapies of weight loss drugs and bariatric surgery for IIH in adolescents require future research. Full article

Background/Objectives: Idiopathic intracranial hypertension (IIH) is an uncommon but clinically important cause of elevated intracranial pressure in children. Conventional MRI findings such as perioptic subarachnoid space (SAS) distension and posterior globe flattening are helpful but may lack sensitivity or specificity in certain cases. Diffusion tensor imaging (DTI), which quantifies white matter microstructure through metrics such as fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (AD), and radial diffusivity (RD), offers additional diagnostic potential, yet its role in pediatric IIH remains insufficiently defined. Methods: This retrospective case–control study included 26 pediatric patients with IIH and 26 age- and sex-matched controls who underwent brain MRI with DTI between 2010 and 2025. DTI parameters were measured in major white matter tracts, and conventional MRI findings associated with raised intracranial pressure were recorded. Associations between DTI metrics and conventional imaging markers were analyzed using standardized statistical tests. Results: Children with IIH demonstrated significantly reduced FA and increased MD and RD values in several key white matter regions, particularly within the optic radiation, splenium of the corpus callosum, and posterior limb of the internal capsule. FA values showed a negative correlation with perioptic SAS width, while RD and MD were positively correlated with posterior globe flattening and empty sella grade. Receiver operating characteristic analysis identified FA in the optic radiation as the strongest discriminator between IIH and controls (AUC = 0.83). Inter-observer reliability for FA measurements was excellent (ICC = 0.91). Conclusions: Pediatric IIH appears to be associated with pressure-related microstructural alterations in white matter, detectable through DTI. Among the diffusion metrics, FA demonstrated the strongest diagnostic potential and may serve as a complementary tool to conventional MRI. Validation in larger, prospective pediatric cohorts is required to establish its clinical utility. Full article

Background: Fulminant idiopathic intracranial hypertension (IIH) is a rare and vision-threatening variant of IIH, characterized by rapid visual deterioration and a high risk of irreversible blindness. Urgent intervention is required to prevent permanent optic nerve damage. Cerebral transverse venous stenting (CTVS) has emerged as an effective treatment for medically refractory IIH, but data on its use in fulminant cases remain limited. Methods: A retrospective consecutive cohort study was conducted at a tertiary center and included all patients with fulminant IIH diagnosed by modified Dandy criteria, with bilateral transverse sinus stenosis > 50% and a trans-stenotic pressure gradient ≥ 8 mmHg on venography. Before stenting, patients received high-dose acetazolamide (up to 3000 mg/day) and IV methylprednisolone (1000 mg/day × 3). Neuro-ophthalmic assessment included BCVA, Ishihara color vision, pupillary exam, disc edema grading, Humphrey visual fields, and optical coherence tomography (OCT). Follow-up occurred at baseline (admission), 1 week, 1 month, 3 months, and 12 months. Results: Five young female patients underwent successful CTVS without peri- or post-procedural complications. Significant improvement in headache and stabilization or recovery of visual function were observed in all patients. OCT revealed early retinal nerve fiber layer thinning within one week, preceding clinical resolution of papilledema. Conclusions: Emergent CTVS appears to be a safe and effective vision-preserving procedure in fulminant IIH, offering rapid intracranial pressure reduction and early neuro-ophthalmologic improvement. OCT may serve as a useful early predictor of treatment success, supporting its role in post-procedural monitoring. Larger prospective studies are warranted. Full article

Background/Objectives: Idiopathic intracranial hypertension (IIH) often features dural venous sinus stenosis; venous sinus stenting (VSS) improves venous outflow and intracranial pressure, but most stents are off-label, and few are engineered for intracranial venous anatomy. The aim was to synthesize animal models relevant to IIH/VSS, catalogue stents used clinically for VSS and summarize corresponding animal data, appraise current preclinical VSS research, and propose a pragmatic preclinical evaluation framework. Methods: We performed a targeted search (PubMed, Web of Science, Scopus; through to May 2025), dual-screened the records in Nested Knowledge, and extracted the model/device characteristics and outcomes as per the predefined criteria. Results: We identified 65 clinical VSS studies; most were retrospective and used off-label carotid/peripheral/biliary stents (Precise, Zilver, and Wallstent were the most frequent). Recent dedicated systems (River, BosStent) have limited animal evidence; VIVA has GLP porcine venous peripheral data demonstrating its patency, structural integrity, and benign healing outcomes. Rodent models reproduce obesity/androgen drivers with modest, sustained ICP elevation; large animal models show the technical feasibility of in sinus implantation, but no chronic focal venous stenosis model fully mirrors the IIH condition. Conclusions: Despite broad clinical uptake, the translational underpinnings of VSS in IIH remain incomplete: most devices lack intracranial venous-specific preclinical validation, and there is no existing animal model that recapitulates both IIH biology and focal sinus stenosis. Full article

Idiopathic intracranial hypertension (IIH) with (IIHWP) and without papilledema (IIHWOP) is characterized by increased cerebrospinal fluid (CSF) pressure and no evident cause, mostly affecting obese women of childbearing age and possibly leading to vision loss. However, in neonates, infants, and toddlers, these conditions remain understudied entities. This review investigates clinical features, risk factors, treatments, and outcomes to inform their care. From 2278 publications found in PubMed, 2974 in Scopus, and 1684 in the Web of Science Core Collection, 104 relevant articles were analyzed. Among 300 cases, 48.3% were male and 26.0% female, with 43.0% meeting the modified Dandy criteria. Typical signs and symptoms, besides papilledema (23.0%) or its absence (49.0%), included bulging fontanelle (67.7%), irritability (34.3%), vomiting (33.0%), and fever (18.3%). The most triggering factors were medications (35.3%) and infections (15.0%). The mean CSF opening pressure was 35.1 cm H₂O, ranging from 9.5 to 77 cm H₂O. Main treatment options were lumbar punctures (72.7%), discontinuation of triggering medications (26.3%), acetazolamide (18.7%), and corticosteroids (7.7%); 3.0% required shunting. Unlike in adults, males were more commonly affected, and papilledema was less frequent. Most cases resolved with conservative treatment. A nosological distinction between IIHWP and IIHWOP seems unlikely. Considering our findings and age-specific CSF pressure limits, new diagnostic criteria are proposed. Full article

Background: Venous sinus stenting is a promising treatment for intracranial venous disorders, such as idiopathic intracranial hypertension and pulsatile tinnitus, associated with transverse sinus stenosis. The VIVA Stent System (VSS) is a novel self-expanding braided venous stent designed to navigate tortuous cerebral venous anatomy. This preclinical study assessed the safety, thrombogenicity, and performance of the VSS in a swine model. Methods: Fifteen swine underwent bilateral internal mammary vein stenting with either the VSS (n = 9) or the PRECISE^® PRO RX stent (n = 6, reference). Fluoroscopy and thrombogenicity assessments were conducted on the day of stenting, clinical pathology analysis was carried out throughout the in-life phase, and CT Venography was performed before sacrifice. Animals were sacrificed at 30 ± 3 or 180 ± 11 days post-stenting for necropsy and histological evaluation. Results: Fluoroscopic angiography confirmed the successful VSS deployment with complete venous wall apposition and no vessel damage. The VSS achieved the highest scores on a four-point Likert scale for most performance parameters. No thrombus formation was observed on either delivery system. CT Venography confirmed vessel patency, no stent migration, and complete stent integrity. Histopathology showed a mild, expected foreign body reaction at 30 days, which resolved by 180 days, indicating normal healing progression. Both stents showed increased luminal diameter and decreased wall thickness at 180 days, suggesting vessel recovery. No adverse reactions were observed in non-target organs. Conclusions: The VSS exhibited favorable safety, procedural performance, and thromboresistance in a swine model, supporting its potential clinical use for treating transverse sinus stenosis and related conditions. Full article

Pulsatile tinnitus (PT) is a subtype of tinnitus characterized by a perception of heartbeat-synchronous sound. It represents approximately 5–10% of all tinnitus cases and may have either a vascular or non-vascular etiology. Accurate diagnosis is crucial due to the potentially serious implications this condition can entail. Assessment through anamnesis and physical examination may often suggest a diagnosis of PT, but it is rarely definitive. Therefore, a comprehensive and specific imaging diagnostic protocol is essential when evaluating PT. A lack of consensus has been identified regarding the use of a standardized protocol for both pulsatile and non-pulsatile tinnitus, whether unilateral or bilateral. Consequently, neuroradiologists, otologists, and otoneurologists from a tertiary hospital have developed a new imaging diagnostic protocol for PT. The aim of this article is to present an updated approach to the diagnostic and therapeutic management of PT, aiming to establish a protocol that serves as a guide for clinicians assessing this symptom. In patients with bilateral PT, systemic conditions leading to increased cardiac output should generally be ruled out; in unilateral cases, focused imaging studies should be performed to exclude organic etiologies at the cervical and cranial levels. Full article

Pregnancy orchestrates profound neurological, hormonal, and anatomical transformations in the maternal brain, preparing it for caregiving and infant bonding. Neuroimaging reveals structural changes such as gray matter reductions and white matter reorganization during pregnancy, followed by partial recovery postpartum. These adaptations are modulated by fluctuating levels of estradiol, progesterone, prolactin, and oxytocin, which coordinate neuroplasticity and behavioral readiness. At the molecular and cellular levels, pregnancy hormones drive synaptic remodeling, neurogenesis, and glial activity. Together, these changes support maternal motivation, attachment, and responsiveness, highlighting the maternal brain’s dynamic plasticity across gestation and the postpartum period. Also, pregnancy induces profound physiological changes, particularly in vascular, hormonal, and neurologic systems, to support maternal and fetal health. While these adaptations are essential, they can predispose pregnant individuals to various neurologic and ophthalmic pathologies. This review explores how pregnancy-related changes—including hypercoagulability, pituitary enlargement, hormonal fluctuations, and immunological modulation—contribute to conditions such as stroke, idiopathic intracranial hypertension, preeclampsia-associated visual disturbances, and demyelinating disorders like neuromyelitis optica spectrum disorder and multiple sclerosis. Additionally, ocular manifestations of systemic diseases like diabetic retinopathy and thyroid orbitopathy are discussed. Understanding these complex interactions is critical for prompt recognition, accurate diagnosis, and appropriate management of vision-threatening and neurologically significant complications during pregnancy. Nevertheless, many aspects of physiological and pathological changes during and after pregnancy remain unknown and warrant further investigation. Full article

Background: Idiopathic intracranial hypertension (IIH) is, by definition, of unknown cause. Davson’s equation indicates that the increased intracranial pressure (ICP) found in IIH could be due to an increase in the CSF formation rate (CSF_fr), the CSF outflow resistance (R_out) or the venous sinus pressure. Studies simultaneously measuring the ICP and sagittal sinus pressures in IIH suggest that there is either a reduction in the R_out and/or the CSF_fr. The latter suggests that the increased venous pressure can be the only variable causing this disease process. A study maintaining the ICP at zero showed a significantly elevated CSF_fr in this disease. The purpose of the current study is to define the most feasible explanation for these findings and to suggest a viable pathophysiology for IIH. Methods: A lumped parameter vascular model, originally developed to study normal pressure hydrocephalus, was extended to investigate IIH. The model used the simultaneously obtained ICP and sagittal sinus pressure measurements from five experiments published in the literature to estimate the CSF_fr and the capillary transmural pressure (TMP). The assumptions made during this study were those of a normal mean arterial pressure, a normal total R_out and a normal blood flow rate. Results: When the CSF formation rates were plotted against the estimated capillary transmural pressures, a straight line was returned, suggesting that the CSF_fr and capillary TMP are related. Conclusions: The novel findings of this study suggest that the CSF_fr in IIH varies with the capillary TMP. A reduced capillary TMP in IIH can moderate the ICP if there is net CSF absorption across the capillaries. This would require the blood–brain barrier (BBB) to be disrupted. The model suggests that drugs which stabilise the BBB may trigger IIH by blocking CSF absorption across the capillaries, increasing the apparent CSF formation rate back toward normal and increasing the ICP. Anaemia will promote IIH by increasing the cerebral blood flow, the capillary TMP and the CSF_fr. Full article

Background and Objectives: Primary pseudotumor cerebri syndrome, another name for idiopathic intracranial hypertension (IIH), is a neurological condition marked by elevated intracranial pressure (ICP) that can result in papilledema without a known etiology. The purpose of this study is to compare the efficacy of topiramate and acetazolamide as medical treatments for IIH and to evaluate the long-term outcomes of both medications. Materials and Methods: This systematic review and meta-analysis followed the PRISMA guidelines and was approved by the International Prospective Register for Systematic Reviews (PROSPERO). This study included randomized clinical trials, retrospective and prospective cohort studies, and patients with idiopathic intracranial hypertension (IIH). Data extraction was performed using the Rayyan application, and the risk of bias was assessed using the Critical Appraisal Skills Program (CASP). Results: The findings revealed a statistically significant 67% increase in the likelihood of improvement at 6 months compared to the baseline with the administration of acetazolamide and topiramate. After six months of the drug administration, there was a 3.6 times decrease in visual obscuration compared to the baseline. A significant advantage of topiramate in IIH is the added benefit of weight loss, since obesity is a modifiable risk factor. However, acetazolamide remains the conventional treatment. Conclusions: This study found that acetazolamide and topiramate are both effective therapies for idiopathic intracranial hypertension (IIH), improving visual metrics and decreasing cerebrospinal fluid pressure. Topiramate aids in weight reduction, while acetazolamide is recommended for its ability to lower CSF pressure and alleviate visual changes. A combination treatment of topiramate and acetazolamide is recommended for better results. Full article

Background/Objectives: Cerebrospinal fluid (CSF) rhinorrhoea carries a significant risk of life-threatening intracranial complications. This review provides a contemporary overview of current management strategies for CSF rhinorrhoea. Methods: We conducted a literature review, examining studies from Medline, Embase, and Google Scholar published within the last 20 years. This narrative synthesis summarises the current and future trends in the management of CSF rhinorrhoea. Results: The management of CSF leaks requires a multidisciplinary approach, encompassing a thorough clinical assessment, targeted diagnostic testing, and a spectrum of surgical and non-surgical interventions. Endoscopic techniques, particularly the use of vascularised flaps such as the nasoseptal flap, has become central to anterior skull base reconstruction. Numerous graft and flap choices provide tailored solutions based on defect size and CSF flow characteristics, with reported success rates exceeding 90%. Conclusions: Endoscopic repair of CSF rhinorrhoea continues to evolve, with modern techniques significantly enhancing success rates and reducing morbidity. Further understanding of underlying aetiologies, advances in technology, and refinement in surgical technique are areas for future innovation in CSF rhinorrhoea management. Full article

Objective: The course of relapsing–remitting multiple sclerosis (RRMS) is highly variable and there is a lack of effective prognostic biomarkers. This study aimed to assess the potential prognostic value of the chemokines B lymphocyte chemoattractant molecule (CXCL13), eotaxin-1 (CCL11), and macrophage inflammatory protein 3-alpha (CCL20) in RRMS. Methods: Forty-two patients with MS were enrolled, along with 22 controls, 12 of the controls were idiopathic intracranial hypertension (IIH) patients, and 10 of the controls were other neurologic diseases (OND). Chemokine levels were measured using enzyme-linked immunosorbent assay (ELISA) in serum and cerebrospinal fluid (CSF) samples. Results: No significant differences were observed among the groups in serum levels of CXCL13, CCL11, and CCL20 (p = 0.509, p = 0.979, p = 0.169, respectively). CSF CXCL13 levels were significantly higher in the OND group (p = 0.016). A PATH analysis showed CSF CXCL13 was significantly associated with new T2 hyperintense lesions on brain magnetic resonance imaging (p < 0.001), and baseline serum CCL11 levels were associated with EDSS (p = 0.030), implying its potential role in indicating neurodegenerative processes and possible progression risk. Serum CCL20 correlated with EDSS (p = 0.002) and lesion burden (p < 0.001), reflecting disease severity. Conclusions: These findings suggest that CSF CXCL13 could serve as a useful biomarker for predicting active disease in RRMS, while follow-up serum CCL11 may assist in identifying progression. Although these chemokines are not specific to MS, higher levels may signal disease activity, severity, and transition to more progressive stages. Full article

Objective: Patients with chronic idiopathic intracranial hypertension (IIH) commonly experience a high level of disability and low satisfaction with medical treatment. We aim to evaluate long-term functional improvement and patient satisfaction in IIH patients with similar symptoms by comparing venous sinus stenting (VSS) to standard medical therapy. Methods: We conducted a cross-sectional questionnaire study of 111 IIH patients, comparing 37 adult female patients who underwent venous sinus stenting with 74 patients treated medically. Propensity score matching was used to balance age and presence of papilledema at presentation between groups. Headache-related disability was evaluated using the Migraine Disability Assessment Scale (MIDAS), while general function and treatment satisfaction were assessed using custom questionnaires. Electronic medical records and the results of imaging upon diagnosis were reviewed retrospectively. Results: The stented group reported significantly better outcomes in physical well-being (median 4.0 vs. 1.0, p < 0.001), task completion (4.0 vs. 1.0, p < 0.001), work/school persistence (5.0 vs. 1.0, p < 0.001), and mental well-being (4.0 vs. 1.0, p < 0.001). Additionally, the stented group had a lower proportion of patients with severe MIDAS (MIDAS > 4, 24.3% vs. 47.9%, p = 0.017). Logistic regression suggested venous stenting as a protective factor against severe MIDAS scores (OR = 0.174, p = 0.004). Conclusion: Cerebral venous stenting in patients with IIH is associated with lower disability and higher patient satisfaction from medical treatment compared to those treated with medications only. These findings suggest that venous sinus stenting may be a valuable treatment option for selected IIH patients. However, larger prospective studies are needed to further validate our results. Full article

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. Full article

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. Full article