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To analyze a case of atypical bilateral papilledema in a puerperium woman, and to explore the pathogenic mechanism of pregnancy-related physiological changes, blood hypercoagulable state, immune abnormalities and abnormal structure of intracranial venous sinus, so as to provide reference for early diagnosis and intervention of similar cases. A 28-year-old woman, 3 days post-operative from a cesarean section, presented at the hospital with decreased vision in her right eye. An examination revealed bilateral papilledema. She did not have typical symptoms like dizziness, headache, or pulsatile tinnitus. During pregnancy, she had taken hydroxychloroquine orally for 5 months due to elevated immune indexes. She also received anticoagulant therapy for lower extremity venous thrombosis a month prior and had a history of cerebrospinal fluid leakage repair for intracranial hypotension syndrome a year ago. Fundus photography and OCT showed bilateral papilledema and macular edema in the right eye, with slightly enlarged physiological blind spots in both eyes. Her pre-pregnancy BMI was 16.5, and postpartum BMI was 22. Laboratory tests indicated a D-dimer level exceeding 20 mg/L and abnormal immune indicators. Ophthalmic color Doppler ultrasound demonstrated bilateral optic nerve sheath widening, with measurements of 0.625 cm on the right and 0.590 cm on the left, suggesting potential elevated intracranial pressure. MRV detected stenosis in the right distal sigmoid sinus and proximal transverse sinus, while the left sigmoid sinus and transverse sinus were not visualized. The patient was diagnosed with increased intracranial pressure caused by multiple factors. Treatment with mannitol to reduce intracranial pressure, along with anticoagulation and other supportive and symptomatic treatments, was administered. After 1 week, macular edema in the right eye subsided, vision improved, and bilateral papilledema slowly improved. This case provides multi-dimensional clinical evidence for the differential diagnosis of puerperium papilledema. For patients with low BMI and atypical symptoms of bilateral papilledema during puerperium, it is necessary to be alert to multiple pathogenic factors. It is recommended to preferentially screen intracranial venous sinus lesions and detect immune indicators by imaging. Ocular ultrasound can be used as a non-invasive screening method for intracranial hypertension.

Abstract BACKGROUND The resistivity index (RI) in cerebral venous sinus stenosis (VSS) has not been studied in patients with idiopathic intracranial hypertension (IIH). OBJECTIVE To evaluate the role of RI measured by quantitative magnetic resonance venogram (QMRV) as a noninvasive tool in the diagnosis of venous hypertension associated with VSS in IIH. METHODS Retrospective evaluation of 13 consecutive IIH patients who underwent venous sinus stenting at our institution between 2013 and 2018. Patients’ demographics, clinical presentation, cerebral mean venous sinus pressure (MVP), and RI both pre- and poststenting were recorded. The baseline RI was also compared to a control group. RESULTS Among 13 patients of IIH, 11 had unilateral VSS in dominant sinus, whereas 2 had bilateral VSS. RI was significantly higher in IIH patients compared to the control group in the superior sagittal (SSS) and transverse sinuses (TS) (0.21 vs 0.11, P = .01 and 0.22 vs 0.13, P = .03, respectively). The MVP (in mm Hg) decreased significantly after venous sinus stenting in the SSS (41.9 to 22.5, P < .001) and TS (39.4 to 19.5, P < .001), which was also associated with a significant reduction of the RI (0.22 vs 0.17, P < .01 in SSS and 0.23 vs 0.17, P = .03 in TS) poststenting. CONCLUSION RI calculated using QMRV can serve as a noninvasive tool to aid in the diagnosis of hemodynamically significant VSS. The study had a small sample size, and larger multicenter studies would be required to validate the results further.

Idiopathic Intracranial Hypertension (IIH) is a debilitating disorder characterised by raised intracranial pressure (ICP), papilloedema with the potential risk of permanent visual loss, and headaches that are profoundly disabling and reduce the quality of life. The first consensus guidelines have been published on investigation and management of adult IIH and one key area of uncertainty is the utility of dural venous sinus stenting for the management of headache and visual loss. There are an increasing number of series published and to help understand the successes and complications. During a patient physician priority setting, the understanding of the best type of intervention to treat IIH was assigned to the top 10 of most desired research questions for the disease. Ultimately randomised clinical trials (RCTs) in neurovascular stenting for IIH would be instructive, as the literature to date may suffer from publication bias. Due to the increasing incidence of IIH, there is no better time to systematically investigate interventions that may reverse the disease process and achieve remission. In this review we discuss the pathophysiology of IIH in relation to venous sinus stenosis, the role of venous sinus stenting with a review of the relevant literature, the advantages and disadvantages of stenting compared with other surgical interventions, and the future of stenting in the treatment of IIH.

Background Idiopathic intracranial hypertension (IIH) is a cause of chronic headaches that are probably driven by raised intracranial pressure (ICP). Cerebral venous sinus pressure is thought to play a role in the underlying pathology, but its relation with intracranial pressure requires further investigation. We aimed to evaluate the concordance between lumbar puncture opening pressure (LPOP) as indicator of the ICP and cerebral venous sinus pressure in patients investigated for IIH. Methods In this case-series replication study, all patients with IIH suspicion and who underwent cerebral venous sinus pressure measurement followed immediately by LP opening pressure (LPOP) measurement were retrospectively included. Pearson’s correlation and measurement agreement (Bland-Altman plots) between venous pressure and LPOP were analyzed. Results 52 consecutive patients (46 women; median age, 31 years [IQR = 25–42]) were included. The mean pressure in the superior sagittal sinus (SSS) and in the torcular were 20.9mmHg (SD ± 7.3) and 20.8 mmHg (SD ± 6.8), respectively. The mean LPOP was 22mmHg (SD ± 6.4). Pressure measured in the transverse venous sinus, the torcular, and the SSS correlated with LPOP ( p  < 0.001). Bland-Altman plots showed that torcular pressure strongly agreed with LPOP (mean difference of 1.7mmHg). The limit of agreement (LOA) (mean difference ± 1.96SD) contained 98.1% of the differences between the two methods, confirming the concordance between the two measures. Torcular pressure and LPOP were consistent in patients with a trans-stenotic pressure gradient ≥ or < to 8 mmHg (mean difference: 1mmHg and 2.4mmHg, respectively), and for those with a LP OP ≥ or < to 18mmHg (mean difference: 1.8mmHg and 1.95mmHg, respectively). Conclusions In patients investigated for IIH, the ICP measured at the LP is correlated and concordant with the torcular pressure. These results confirm previous findings and further corroborate the hypothesis that cerebral venous system plays a major role in CSF dynamics and ICP.

In this review, we provide an update on the pathogenesis, diagnosis, and management of adults with idiopathic intracranial hypertension (IIH) and implications of the cerebral venous system, highlighting the progress made during the past decade with regard to mechanisms of the venous outflow pathway and its connection with the cerebral glymphatic and lymphatic network in genesis of IIH. Early diagnosis and treatment are crucial for favorable visual outcomes and to avoid vision loss, but there is also a risk of overdiagnosis and misdiagnosis in many patients with IIH. We also present details about treatment of intracranial hypertension, which is possible in most cases with a combination of weight loss and drug treatments, but also in selected cases with surgical interventions such as optic nerve sheath fenestration, cerebral spinal fluid (CSF) diversion, or dural venous sinus stenting for some patients with cerebral venous sinus stenosis, after careful analysis of mechanisms of intracranial hypertension, patient clinical profile, and method risks.

Internal jugular vein (IJV) stenosis and cerebral venous sinus (CVS) stenosis belong to cerebral venous outflow insufficiency. This study aimed to analyze the similarities and differences between IJV stenosis and CVS stenosis. Patients with either IJV stenosis or CVS stenosis confirmed by contrast-enhanced magnetic resonance venography between October 2017 and July 2018 were enrolled in this retrospective study. The similarities and differences between IJV stenosis and CVS stenosis on the aspects of clinical and imaging features were compared. A total of 82 eligible patients entered into the final analysis. The similarities of the two subsets of cerebral venous outflow insufficiency mainly included headache, head noises or tinnitus, visual disorders, and sleeping disorders, as well as cloud-like white matter hyperintensity in T2WI and FLAIR sequences of MRI. However, there were differences in between, the ratio of patients with higher intracranial pressure (ICP) was common in CVS stenosis (p < 0.001). Namely, higher ratios of papilledema (p = 0.001) and visual damage (p = 0.029), as well as poor Frisen papilledema grade scores were more commonly observed in CVS stenosis (p = 0.004), while abnormal collateral-vessels appeared more frequently in IJV stenosis (100.00%) than CVS stenosis (28.57%). Continuous head noises, tinnitus and cloud-like white matter hyperintensity in MRI are the features of both IJV stenosis and CVS stenosis. Whereas, severe headache, visual damage, papilledema, and intracranial hypertension (IH) were more common in CVS stenosis, and the appearance of collateral-vessels is a key feature of IJV stenosis.

Purpose of ReviewIdiopathic intracranial hypertension (IIH) is a disorder characterized by long-standing elevated intracranial pressure (ICP). As the name applies, no uniform cause has been identified. IIH is typically characterized by headaches, pulsatile tinnitus, and visual deterioration.Recent FindingsAnomalies in cerebrospinal fluid (CSF) absorption are implicated in the pathophysiology of IIH. Non-invasive imaging of the brain parenchyma and the cerebral venous sinus has improved, and research has gained a better understanding of the role of cerebral venous sinus stenosis. Both have led to a better delineation of the role of arachnoid granulations (AG) and the glymphatic system in the development of IIH.SummaryIIH may occur as a result of restrictions of CSF absorption from the venous system, and or the congestion and overflow of the glymphatic system. Elucidating these mechanisms will lead to greater understanding of its underlying pathophysiologic mechanisms.

Abstract Tinnitus is an abnormal perception of a sound without external stimulation. Venous pulsatile tinnitus (VPT) is a specific form of tinnitus characterized by an objective and often subjective bruit that occurs as a result of localized venous abnormalities. Clinical evaluation relies on sound quality, duration, and precipitating factors. Idiopathic intracranial hypertension (IIH) and transverse sinus stenosis (TSS) are among the most common causes of VPT. Other causes include sigmoid sinus wall abnormalities (SSWAs), jugular vein anomalies (JVAs), and emissary veins anomalies. These anomalies can be detected on magnetic resonance imaging, magnetic resonance angiography/magnetic resonance venography, and high-resolution temporal bone computed tomography. The pathogenesis behind the VPT includes turbulent blood flow as a result of luminal stenosis or abnormal dilation, amplification of internal sound due to temporal bone defects, and abnormal position of the venous sinus system structures adjacent to the bony structures of the auditive apparatus. Based on these theories, different interventional treatment modalities can be applied to treat the underlying causes. Endovascular treatments have shown high efficacy and safety among those treatments which include stenting of the lateral sinus stenosis in IIH and TSS, coiling of the SSWA and JVA, and embolization of emissary veins anomalies. Further studies are needed to understand the natural history of these anomalies and the efficacy of treatments of VPT, which—unlike other types of tinnitus—can be cured with proper treatment.

Background The differential diagnosis of bilateral optic disc edema with painless vision loss poses significant challenges between idiopathic intracranial hypertension (IIH) and optic neuritis (ON). While IIH classically presents with elevated intracranial pressure (ICP), headaches, and papilledema, ON typically manifests as inflammatory optic nerve demyelination with characteristic pain on eye movement. This case illustrates the diagnostic complexity arising from overlapping features of both conditions in a patient with concurrent venous sinus stenosis, underscoring the critical role of multimodal clinical-radiological correlation and therapeutic response analysis. Case presentation A 42-year-old female with a BMI of 29.1 kg/m² presented with sudden bilateral vision loss over one week. The patient had a recent cold, a history of hypertension, and had been menopausal for six months. Ophthalmic examination revealed bilateral optic disc swelling with blurred margins, and visual field tests showed diffuse damage. MRI indicated bilateral optic nerve thickening and fluid accumulation, along with a mildly elevated ICP of 270 mmH 2 O. Initially, optic neuritis was suspected; however, IIH was also considered due to her elevated BMI and increased ICP. Given the rapid progression of her symptoms, the patient was started on methylprednisolone pulse therapy, leading to significant visual improvement. However, subsequent MRV and DSA revealed severe stenosis of the right transverse sinus, suggesting venous sinus stenosis as a contributing factor. The patient refused surgery and continued with corticosteroid treatment, which led to further improvement of her vision. Follow-up showed normal ICP, complete resolution of optic disc edema, and sustained visual acuity after two weeks. Conclusion This case highlights the challenge of diagnosing bilateral optic neuritis with co-existing elevated ICP and venous sinus stenosis. The rapid progression of symptoms and the patient’s response to high-dose corticosteroids suggests optic neuritis as the primary cause of the visual loss and optic disc swelling. Despite the presence of venous sinus stenosis, the elevated ICP likely exacerbated the stenosis rather than being its direct cause. This case underscores the need for careful differentiation between IIH and optic neuritis, especially in patients with atypical presentations, and emphasizes the importance of individualized treatment plans. The patient’s recovery after corticosteroid therapy and the resolution of optic disc edema supports the diagnosis of optic neuritis, with the potential role of venous sinus stenosis in complicating the condition.

Purpose of Review The purpose of this review is to provide an updated approach to the evaluation and management of pulsatile tinnitus (PT), an uncommon but often treatable subtype of tinnitus. Recent Findings Secondary PT can be due to either vascular or non-vascular etiologies, including, but not limited to: neoplasm, arteriovenous malformation or fistula, idiopathic intracranial hypertension, dural venous sinus stenosis, otoacoustic etiologies (e.g., otosclerosis, patulous eustachian tube) and bony defects (e.g., superior semicircular canal dehiscence). Computed tomography (CT) and magnetic resonance imaging (MRI) imaging have comparable diagnostic yield, though each may be more sensitive to specific etiologies. If initial vascular imaging is negative and a vascular etiology is strongly suspected, digital subtraction angiography (DSA) may further aid in the diagnosis. Many vascular etiologies of PT can be managed endovascularly, often leading to PT improvement or resolution. Notably, venous sinus stenting is an emerging therapy for PT secondary to idiopathic intracranial hypertension with venous sinus stenosis. Summary Careful history and physical exam can help establish the differential diagnosis for PT and guide subsequent evaluation and management. Additional studies on the efficacy and long-term outcome of venous sinus stenting for venous stenosis are warranted.