In recent years, with the current access in techniques, studies have significantly advanced the knowledge on meningeal immunity, revealing that the central nervous system (CNS) border acts as an immune landscape. The latest concept of meningeal immune system is a tertiary structure, which is a comprehensive overview of the meningeal immune system from macro to micro. We comprehensively reviewed recent advances in meningeal immunity, particularly the new understanding of the dural sinus and meningeal lymphatics. Moreover, based on the clues from the meningeal immunity, new insights were proposed into the dural arteriovenous fistula (DAVF) pathology, aiming to provide novel ideas for DAVF understanding.

Background and purpose A major challenge in spinal dural arteriovenous fistula (SDAVF) is timely diagnosis, but no specific predictive biomarkers are known. Methods In the discovery cohort (case, n = 8 vs. control, n = 8), we used cerebrospinal fluid (CSF) and paired plasma samples to identify differentially expressed proteins by label-free quantitative proteomics. Further bioinformatics enrichment analyses were performed to screen target proteins. Finally, it was validated by ELISA in two of the new cohorts (case, n = 17 vs. control, n = 9), and univariate analysis, simple linear regression, and receiver operator characteristic (ROC) curve analysis were performed to evaluate the diagnostic potential. Results In the discovery cohort, the most overexpressed proteins were APOB and C4BPA in CSF samples of patients. The GO/KEGG enrichment analysis indicated that the upregulated proteins were mainly involved in the acute inflammatory response and complement activation. Hub-gene analysis revealed that APP might be the key protein in the molecular interaction network. In the validation cohort, C4BPA and C1QA were significantly overexpressed in the CSF of patients, averaging 3046.9 ng/ml and 2167.2 ng/ml, respectively. Simple linear regression demonstrated that levels of C1QA and C4 were positively correlated with total protein in CSF (R2 = 0.8021, p = 0.0005; R2 = 0.7447, p = 0.0013). The areas under the ROC curves of C4BPA and C1QA were 0.86 and 1.00, respectively. Conclusions This study was the first to identify C4BPA and C1QA as potential biomarkers for the diagnosis of SDAVF and revealed that complement pathway activation might be one of the molecular mechanisms for venous hypertension myelopathy.

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ObjectiveArteriovenous fistulas (AVFs) in the craniocervical junction (CCJ) region are a rare occurrence with special clinical manifestations. This study retrospectively reviewed patients with CCJ AVFs treated at our neurosurgical center, aiming to enhance the understanding of CCJ AVFs.MethodsA total of 113 patients with CCJ AVFs treated at our neurosurgical center between January 2013 and December 2020 were enrolled. They were grouped as patients with CCJ AVFs with spinal arterial feeders (n = 20) and patients with CCJ AVF without spinal arterial feeders (n = 93). Clinical presentation, angiographic characteristics, intraoperative findings, and treatment outcomes were analyzed.ResultsThe patients’ median age was 55 years (IQR 47.5–62 years). The proportion of males in the group without spinal arterial feeders was significantly higher (p = 0.001). Subarachnoid hemorrhage (SAH) was the most common clinical presentation, especially in the group with spinal arterial feeders (p < 0.001). There were significant differences in AVF type, fistula location, and direction of the venous drainage between the two groups (p < 0.001). Intervention embolization combined with microsurgery was more common in treating AVFs with spinal arterial feeders (p = 0.006). Spinal arterial feeders did not affect the outcome (p = 0.275).ConclusionsSAH was the most common presentation of CCJ AVFs in this study. Microsurgery and interventional embolization were optional treatment strategies. The angioarchitecture of CCJ AVFs was essential for selecting treatment strategies.

BACKGROUND: Arteriovenous fistulas (AVFs) at the craniocervical junction (CCJ) region with spinal arterial feeders are rare. There are few reports on such diseases. This study retrospectively reviewed 20 consecutive cases with CCJ AVFs with spinal arterial feeders from our neurosurgical center, which can enrich the experience of diagnosis and treatment of such arteriovenous fistulas. OBJECTIVE: To further explain the clinical manifestations, angiographic characteristics, and treatment strategies of CCJ AVFs with spinal arterial feeders and share the treatment experience of our neurosurgical center. METHODS: A total of 113 patients with CCJ AVFs treated at our institution between January 2013 and December 2020 were enrolled. After analyzing their imaging data, 20 patients with CCJ AVFs with spinal arterial feeders were included. Clinical presentation, angiographic characteristics, intraoperative findings, and treatment outcomes were analyzed. RESULTS: The median age was 52 years (IQR 45-58 years). Twenty-four fistulas were included in this study and were classified based on angiographic characteristics: dural AVF (1 [4.2%] of 24), radicular AVF (15 [62.5%] of 24), epidural AVF (2 [8.3%] of 24), and perimedullary AVF (6 [25.0%] of 24). Subarachnoid hemorrhage occurred in 19 cases (95.0%) and was the main clinical manifestation. Thirteen patients (65.0%) were treated with microsurgery only, 6 (30.0%) with embolization and microsurgery, and 1 (5.0%) with interventional electrocoagulation. Nineteen patients (95.0%) had favorable outcomes. CONCLUSION: Subarachnoid hemorrhage was the most common presentation of CCJ AVFs with spinal arterial feeders. Different treatment strategies should be selected based on different angioarchitecture.

OBJECTIVE Current knowledge about venous hypertensive myelopathy (VHM) is incomplete. This study was performed with the aim of clarifying the clinical features and outcomes of craniocervical VHM. METHODS This retrospective, single-center cohort study included 65 patients with craniocervical junction arteriovenous fistulas resulting in VHM treated in Xuanwu Hospital from January 1, 2002, to December 30, 2020. All patients underwent microsurgery or endovascular treatment. The primary outcome was neurological function assessment using the Japanese Orthopaedic Association (JOA) scale, modified Aminoff-Logue Scale (mALS), and Venous Hypertensive Myelopathy Scale (VHMS). The secondary outcomes were recurrences and postoperative adverse events. Pearson linear regression and receiver operating characteristic curves were used to evaluate the relationships among the three scales. Kaplan-Meier and multivariate logistic regression analyses were performed to predict outcomes. RESULTS The mean patient age was 57.4 ± 11.4 years, and 88% of patients were male. The 1-year follow-up rate was 83.1%, and the 5-year follow-up rate was 50.8%. The VHMS was correlated with the JOA (R2 = 0.6722) and mALS (R2 = 0.7399) and increased the assessment accuracy by approximately 20% when compared with the other two scales. Overall, 25.9% of patients experienced delayed neurological decline beyond the 1-year follow-up. Further logistic regression suggested that age > 65 years was an independent predictor (OR 7.831, 95% CI 1.090–56.266; p = 0.041). Embolic recanalization and new bilateral symmetry feeders were the major reasons for recurrence. Recurrence increased the risk of adverse events after the second surgery (OR 20.455, 95% CI 1.170–357.320; p = 0.039). CONCLUSIONS CCJ AVFs resulting in VHM are a rare but deadly complication, and providers should be cautious of age-related delayed neurological decline and strive for a one-time anatomical cure.

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