Search Results (57)

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

Background/objectives: The aim of this study was to evaluate the long-term outcomes of a cohort of ophthalmologically resolved female idiopathic intracranial hypertension (IIH) patients. Methods: Our cross-sectional study included adult females with at least 6 months of ophthalmologically resolved IIH. Patients with papilledema or who underwent IIH-targeted surgical intervention were excluded. Participants completed a questionnaire consisting of medical information, the Migraine Disability Assessment Scale (MIDAS) and the Headache Impact Test (HIT-6). Electronic medical records and the results of imaging upon diagnosis were retrospectively reviewed. Results: One-hundred-and-four participants (mean age 35.5 ± 11.9 years) were included (7.85 ± 7 years post-IIH diagnosis). Patients with moderate–severe disability according to the MIDAS scale (n = 68, 65.4%) were younger (32.4 ± 8.9 vs. 41.5 ± 14.4 year-old, p < 0.001), had a shorter time interval from IIH diagnosis (5.9 ± 5.3 vs. 11.7 ± 8.5 years, p < 0.001), and had lower FARB scores (indicating a more narrowed transverse-sigmoid junction; 1.28 ± 1.82 vs. 2.47 ± 2.3, p = 0.02) in comparison to patients with low–mild disability scores. In multivariate analysis, a lower FARB score (OR 1.28, 95% CI 0.89–1.75, p = 0.12) and younger age (OR 1.09, 95% CI 0.98–1.19, p = 0.13) showed a trend toward an association with a moderate–severe MIDAS score. Moreover, in the sub-analysis of patients with a moderate–severe MIDAS scale score, the 10 patients with the highest MIDAS scores had a low FARB score (1.6 ± 1.1 vs. 2.7 ± 2.4, p = 0.041). Conclusions: High numbers of patients with ophthalmologically resolved IIH continue to suffer from related symptoms. Symptoms may be associated with the length of time from the diagnosis of IIH and a lower FARB score. Full article

The pathology of idiopathic intracranial hypertension (IIH), a disease characterized by papillary edema and increased intracranial pressure (IICP), is not yet understood; this disease significantly affects quality of life due to symptoms including vision loss, headache, and pulsatile tinnitus. By contrast, superficial siderosis (SS), a disorder in which hemosiderin is deposited on the surface of the cerebral cortex and cerebellum, potentially causes cerebellar ataxia or hearing loss. So far, no cases of IIH with infratentorial and supratentorial cortical SS have been reported. Herein, we report a case of a 31-year-old woman with obesity who developed this condition. The patient suddenly developed headache and dizziness, had difficulty walking, and subsequently became aware of diplopia. Fundus examination revealed bilateral optic nerve congestive papillae and right eye abducens disturbance. Head magnetic resonance imaging (MRI) showed prominent SS on the cerebellar surface and cerebral cortex. Lumbar puncture revealed IICP of 32 cmH₂O, consistent with the diagnostic criteria for IIH, and treatment with oral acetazolamide was started; subsequently, the intracranial pressure decreased to 20 cmH₂O. Her abduction disorder disappeared, and the swelling of the optic papilla improved. She was now able return to her life as a teacher without any sequelae. SS is caused by persistent slight hemorrhage into the subarachnoid space. In this case, both infratentorial and supratentorial cortical superficial SS was observed. Although cases of IIH complicated by SS are rare, it should be kept in mind that a causal relationship between IIH and SS was inferred from our case. Our findings also suggest that cerebrospinal fluid dynamic analysis using MRI is effective in diagnosing IIH and in determining the efficacy of treatment. Full article

(1) Introduction: This pilot study aimed to analyze neurofilament light chain levels in cerebrospinal fluid (cNfL) in a cohort of children with different acute nontraumatic neurological conditions. (2) Methods: This prospective observational cohort study consisted of 35 children aged 3 months to 17 years and was performed from November 2017 to December 2019. Patients’ clinical data were reviewed, and patients were assigned to the following groups: n = 10 (28.6%) meningitis, 5 (14.3%) Bell’s palsy, 7 (20.0%) febrile non-CNS infection, 3 (8.6%) complex febrile seizure, 4 (11.4%) idiopathic intracranial hypertension, and 6 (17.1%) others. cNfL levels were measured using a sensitive single-molecule array assay. (3) Results: The cNfL levels [median (range)] in children with meningitis were 120.5 pg/mL (58.1–205.4), in Bell’s palsy 88.6 pg/mL (48.8–144.5), in febrile non-CNS infection 103.9 pg/mL (60.1–210.8), in complex febrile seizure 56 pg/mL (53.2–58.3), and in idiopathic intracranial hypertension 97.1 pg/mL (60.1–124.6). Within the meningitis group, children with Lyme neuroborreliosis (LNB) had significantly higher cNfL concentrations (median 147.9 pg/mL; range 87.8–205.4 pg/mL) than children with enterovirus meningitis (72.5 pg/mL; 58.1–95.6 pg/mL; p = 0.048) and non-significantly higher cNfL levels when compared to Bell’s palsy (88.6 pg/mL; 48.8–144.5 pg/mL; p = 0.082). There was no correlation between cNfL levels and age. (4) Conclusions: Although the number of patients in this pilot study cohort is limited, higher cNfL levels in children with LNB compared to those with viral meningitis (significant) and Bell’s palsy (trend) may indicate the potential of cNfL as a biomarker in the differential diagnosis of pediatric meningitis and facial palsy. Full article

While the typical patient with idiopathic intracranial hypertension (IIH) is an obese female of childbearing age, there are unique patient populations, such as non-obese females, that have not been well studied. Characterizing this subpopulation may increase awareness our of it, which may prevent underdiagnosis and improve our understanding of IIH’s underlying pathophysiology. We retrospectively reviewed electronic medical records and compared the clinical and radiological characteristics of non-obese (BMI < 30) and obese (BMI > 30) female patients with IIH. Two hundred and forty-six patients (age 32.3 ± 10) met our inclusion criteria. The non-obese patients (n = 59, 24%) were significantly younger than the obese patients (29.4 ± 9.9 vs. 33.2 ± 10.2, p = 0.004) and had higher rates of severe papilledema (Friesen 4–5; 25.4% vs. 11.8%, p = 0.019), scleral flattening (62.7% vs. 36.9%, p = 0.008), and optic nerve dural ectasia (78.0% vs. 55.6%, p = 0.044). Non-obese patients also had a tendency to have a higher lumbar puncture opening pressure (368 ± 92.7 vs. 344 ± 76.4, p = 0.062). Non-obese patients were three times more likely to present with a combination of scleral flattening and optic nerve dural ectasia (OR = 3.00, CI: 1.57–5.72, χ² = 11.63, α < 0.001). Overall, non-obese females with IIH were found to have a more fulminant presentation, typified by higher rates of severe papilledema and radiological findings typical for IIH. Full article