Central Venous Reflux, a Rare Cause of Neurological Manifestations in Hemodialysis Patients: A Case Report and Literature Review

Central venous disease (CVD) is a serious complication in hemodialysis patients. Neurological manifestations are rare. We describe a female with end-stage renal disease with throbbing headache accompanied by paresthesia, weakness, and abnormal posture of her right hand during dialysis sessions. Motor symptoms completely resolved after each dialysis session, although the headaches persisted for several hours. No neurological deficit was evidenced on physical examination. Digital subtraction angiography identified an incomplete thrombosis of the left brachiocephalic vein with retrograde flow in the internal jugular vein, sigmoid sinus, and transverse sinus on the left side. This case illustrates that cerebral venous congestion due to CVD can produce neurological symptoms. Furthermore, we systematically review the literature to identify the characteristics of the cases described so far. This allows clinicians to know the entity and have a high index of suspicion in a hemodialysis patient who develops neurological symptoms.

Clinical Manifestations, Neuroimaging Findings, and Treatment of Idiopathic Intracranial Hypertension in a Nepalese Tertiary Centre

Introduction: Idiopathic Intracranial Hypertension is characterized by headache, visual impairment, papilledema, and increased cerebrospinal fluid opening pressure. We aim to evaluate clinical manifestations, neuroimaging findings, and treatment of Idiopathic Intracranial Hypertension in a Nepalese tertiary center. Materials and Methods: We retrospectively included patients with the diagnosis of Idiopathic Intracranial Hypertension who were admitted to the Neurology department of Tribhuvan University Teaching Hospital from 2019 June to 2021 May and presented to the Neuro-ophthalmology outpatient clinic of the hospital for follow-up. Results: Out of 16 Idiopathic Intracranial Hypertension patients, 12(75%) patients had either headache or ocular pain. Reduced visual acuity and progressive visual loss were found in 44% of patients. Normal CSF opening pressure was found in 19%, 44% had CSF opening pressure at a range of 20-30 cm H20 and > 30 cm H20 in 37.5%. 15 Idiopathic Intracranial Hypertension patients (93.7%) were subtyped as Typical and 1 patient was diagnosed as Fulminant Idiopathic Intracranial Hypertension. Acetazolamide was used for treatment in 15 patients and 1 patient needed ventriculoperitoneal shunting. 56% Seven patients (44%) had some abnormal findings: partial empty sella (44%), and tortuous optic nerve (31%), flattened posterior eyeball (31%), and hypoplastic transverse sinus (19%). Conclusions: Idiopathic Intracranial Hypertension is an uncommon diagnosis but should be suspected in patients with chronic headaches with visual impairment. In low-resource settings, proper history along with neurological and ophthalmological examinations can even detect the early features and timely referral can save the vision and disability of Idiopathic Intracranial Hypertension patients.

Prevalence of Tinnitus in Patients Diagnosed with Cerebral Arteriovenous Fistula Treated with Endovascular Technique

Introduction Tinnitus is characterized as the conscious and involuntary perception of sound, and it affects ∼ 30% of the population. Despite careful physical examination, the etiology of tinnitus can be established for only 30% of patients. Tinnitus is a common symptom of cerebral arteriovenous fistulas and results from increased blood flow through the dural venous sinuses, leading to turbulent arterial flow, mainly related to sigmoid and transverse sinus lesions. Objectives To analyze the frequency of tinnitus, patient profile, and endovascular treatment characteristics in individuals diagnosed with cerebral arteriovenous fistulas. Methods A retrospective and observational study based on reviewed data from medical records on the PHILIPS Tasy system (Philips Healthcare, Cambridge, MA, USA) at the neurosurgery and interventional neuroradiology service of Hospital Santa Isabel in Blumenau–state of Santa Catarina, Brazil. Results The profile of 68 individuals diagnosed with cerebral arteriovenous fistula who underwent endovascular treatment were analyzed. Most patients were female, aged 31 to 60. Tinnitus affected 18 individuals. Dural fistulas were the most prevalent in the sample, and computed tomography alone was the most used diagnostic method for initial investigation. Conclusion The prevalence of this symptom in patients diagnosed with cerebral arteriovenous fistula was found in 26.5% of this sample, mainly in women with associated comorbidities. Tinnitus remission was observed in all patients who underwent endovascular treatment to correct cerebral fistula.

The growth and expansion of meningeal lymphatic networks are affected in craniosynostosis

Skull malformations are associated with vascular anomalies that can impair fluid balance in the central nervous system. We previously reported that humans with craniosynostosis and mutations in TWIST1 have dural venous sinus malformations. It is still unknown whether meningeal lymphatic networks, which are patterned alongside the venous sinuses, are also affected. We now show that the growth and expansion of meningeal lymphatics are perturbed in Twist1 craniosynostosis models. Changes to the local meningeal environment, including hypoplastic dura and venous malformations, affect the ability of lymphatic networks to sprout and remodel. Dorsal networks along the transverse sinus are hypoplastic with reduced branching. By contrast, basal networks closer to the skull base are more variably affected, showing exuberant growth in some animals suggesting they are compensating for vessel loss in dorsal networks. Injecting a molecular tracer into cerebrospinal fluid reveals significantly less drainage to the deep cervical lymph nodes, indicative of impaired lymphatic function. Collectively, our results show that meningeal lymphatic networks are affected in craniosynostosis, suggesting the clearance of beta-amyloid and waste from the central nervous system may be impeded.

Transverse Sinus Stenosis in Venous Pulsatile Tinnitus Patients May Lead to Brain Perfusion and White Matter Changes

Objective: Transverse sinus stenosis (TSS) is associated with various symptoms, but whether it can lead to pathological brain changes is unclear. This study aimed to investigate brain changes in venous pulsatile tinnitus (PT) patients with TSS.Materials and Methods: In this study, fifty-five consecutive venous PT patients and fifty age- and gender-matched healthy controls (HCs) were investigated. In CT venography, the combined conduit score (CCS) was used to assess the degree of TSS in venous PT patients. Magnetic resonance venography was used to assess TSS in HCs. All the participants had undergone arterial spin labeling and structural MRI scans.Results: Two patients without TSS and ten HCs with TSS were excluded. Fifty-three venous PT patients with TSS and 40 HCs without TSS were included in this study. All the patients had unilateral cases: 16 on the left and 37 on the right. Based on the CCS, the patients were divided into high-degree TSS (a score of 1–2) (n = 30) and low-degree TSS groups (a score of 3–4) (n = 23). In the whole brain and gray matter, the patients with high-degree TSS showed decreased cerebral blood flow (CBF) compared with patients with low-degree TSS as well as HCs (P < 0.05), and no significant difference in CBF was found in patients with low-degree TSS and HCs (P > 0.05). In white matter (WM) regions, the patients with high-degree TSS exhibited decreased CBF relative to the HCs (P < 0.05). The incidence of cloud-like WM hyperintensity was significantly higher in the above two patient groups than in the HC group (P < 0.05).Conclusion: TSS in venous PT patients may lead to decreased CBF and cloud-like WM hyperintensity. These neuroimaging findings may provide new insights into pathological TSS in venous PT.

Study on the correlation between hemodynamic status of the transverse sinus-sigmoid sinus and the clinical efficacy of sigmoid sinus wall reconstruction

Background and Purpose For patients with pulsatile tinnitus who have both transverse sinus stenosis and sigmoid sinus wall anomalies, sigmoid sinus wall reconstruction surgery is the first-choice treatment when the trans-stenotic pressure gradient less than 10 mmHg. However, not all patients are cured by surgery. We hypothesized the abnormal hemodynamics caused by transverse sinus stenosis is associated with the clinical efficacy of surgery. Methods Eight pulsatile tinnitus patients treated with surgery were retrospectively reviewed (4 rehabilitated, 4 nonrehabilitated). All patients had radiologically diagnosed transverse sinus stenosis and sigmoid sinus wall anomalies. A numerical simulation of the hemodynamics of the transverse sinus-sigmoid sinus was performed using computational fluid dynamics technology. Changes in the blood flow patterns before and after surgery were observed. The blood flow velocity at the stenosis, vorticity of blood flow in the sigmoid sinus and wall pressure distribution in the sigmoid sinus wall anomalies area were compared. Results The blood flow velocity in the stenosis (preoperative P = 0.04, postoperative P = 0.004) and vorticity in the sigmoid sinus (preoperative P = 0.02, postoperative P = 0.007) pre- and post-surgery were significantly higher in the non-rehabilitation group than in the rehabilitation group. No significant difference was found in the wall pressure distribution in the sigmoid sinus wall anomalies area (preoperative P = 0.12, postoperative P = 0.24). Conclusions There is a clear correlation between the abnormal hemodynamic status caused by transverse sinus stenosis and the clinical efficacy of surgery. The blood flow velocity at the stenosis and vorticity of blood flow in the sigmoid sinus are factors influencing the clinical efficacy of surgery.

Complicated Trichosporon asahii mastoiditis in immunocompetent child

Background Trichosporon asahii is an opportunistic fungus that causes infections in immunosuppressed patients. It is rarely seen in children and immunocompetent hosts. The mortality rates are still high despite early treatment with proper antifungal drugs. Trichosporon asahii mastoiditis in an immunocompetent child makes this case challenging. Case presentation This report presents a case of Trichosporon asahii mastoiditis which was complicated by transverse sinus thrombosis, in an otherwise healthy 21-month-old girl, and successfully treated with voriconazole. Trichosporon asahii was isolated, in three different occasions, from ear discharge of an immunocompetent healthy child, who presented with prolonged history of fever and received appropriate dosages of multiple types of antimicrobials as an outpatient but without improvement. After 48 h of starting the Voriconzole; post auricular swelling and ear discharge improved significantly. Conclusion A high index of clinical and microbiological suspicion is needed for optimal diagnosis of Trichosporon infection. Trichosporon asahii can also cause infection in immunocompetent individual even without previous history of hospitalization or intervention. We emphasize the importance of early pediatric infectious evaluation and intervention.

Anatomic Variation of the Lateral Sinus in Patients With Idiopathic Intracranial Hypertension: Delineation With Black-Blood Contrast-Enhanced MRI

Objectives: The purpose of this study was to describe the peculiar anatomic variations in the lateral sinus and analyze the patterns of cerebrospinal fluid (CSF) drainage by using high-resolution (HR) black-blood (BB) contrast-enhanced magnetic resonance imaging (MRI) in patients with idiopathic intracranial hypertension (IIH).Methods: Total 33 IIH patients who were found cerebral venous sinus stenosis (CVSS) by MR venography (MRV) were enrolled in this study. HR-BB contrast-enhanced MRI was used to assess the features of anatomical variations in transverse sinus and sigmoid sinus. The development of bilateral sinuses was firstly evaluated, including unilateral hypoplasia with contralateral dominance or bilateral balanced development. Then, four kinds of anatomical variations were eventually recorded, including circumscribed stenosis, arachnoid granulation (AG), fibrous septum (FS), and brain herniation (BH) into dural venous sinus (DVS).Results: Bilateral venous drainage dysfunction was found in 30(90.9%) patients, whereas only 3(9.1%) patients presented unilateral venous drainage dysfunction. There was no difference in clinical symptoms between the two groups. The most common case is hypoplasia in unilateral sinus combined with anatomic variation in the contralateral dominant transverse sinus such as AG and BH into DVS. Total of 52 anatomic variations were finally found in bilateral sinuses in 33 enrolled patients, including 19(36.5%)AGs, 12(23.1%)FS, 7(13.5%) BH into DVS and 14(26.9%) circumscribed stenoses. Moreover, 41(62.1%) lateral sinuses showed enhancement in T1-weight-enhanced MRI.Conclusions: Patients with CVSS almost had CSF outflow disorders, whatever bilateral equalization or unilateral hypoplasia with contralateral dominance. Four types of main anatomic variations, including circumscribed stenosis, AG, FS, and BH into DVS, caused venous reflux obstruction by elevating the intracranial press (ICP).

iant extradural dermoid cyst in the right parietal-occipital region with obliteration of the transverse sinus

The study objective – to report a case of giant extradural dermoid cyst in a 48‑year-old woman; the cyst caused extensive erosion of the occipital bone and lower portions of the right parietal bone, which is quite rare.Case report. A 48‑year-old female patient was admitted to the Department of Neurosurgery, A. Ya. Kozhevnikov Clinic of Nervous System Diseases, University Clinical Hospital No. 3, I. M . Sechenov First Moscow State Medical University on 28.07.2020. In 2002, the patient noted the emergence of a 1‑cm subcutaneous formation in the right occipital region. The formation was slowly growing. On 17.12.2015, she underwent magnetic resonance imaging of the brain. It revealed a 2,5 × 4,4 × 4,0 cm extradural formation with clear contours and erosion of the occipital and right parietal bones. On 12.03.2020, she had contrast-enhanced magnetic resonance imaging, which demonstrated that the tumor was growing (its size was 3,0 × 6,4 × 5,5 cm). Magnetic resonance imaging scans also showed extensive erosion (6,4 × 5,4 cm) of the occipital and right parietal bones and obliteration of the right transverse sinus. On 28.07.2020, the patient underwent elective surgery, namely excision of the extradural dermoid cyst in the right parietooccipital region with simultaneous reconstruction of the calvarial bones using a titanium plate. The cyst capsule and its contents were completely removed. The capsule was tightly attached to the dura mater, but did not invade it. To remove small fragments of the capsule that could potentially invade the bone, we performed small marginal resection of the calvarial bones at the depth of 2–3 mm from the defect margin. After the removal of cyst capsule, well-defined furrow could be visualized on the dura mater in the projection of the obliterated transverse sinus. Five days postoperatively, the patient was discharged from hospital in satisfactory condition.Conclusion. Surgical treatment of extradural dermoid cysts implies radical removal of the tumor. Since the cyst capsule gets tightly attached to the edges of the bone defect along with bone erosion, the removal of such cysts as a single block is almost impossible. The risk of severe erosion of the skull bones caused by a dermoid cyst with extradural (and sometimes intradural) growth increases with age. Therefore, it is recommended to remove such tumors as early as possible. Patients with skull bone defects also require cranioplasty.