Background and Purpose: Paravertebral arteriovenous shunts (PVAVSs) are rare. Whether the intradural venous system is involved in drainage may lead to differences in clinical characteristics through specific pathophysiological mechanisms. This study aims to comprehensively evaluate the natural history and clinical outcomes of PVAVSs with or without intradural drainage. Methods: Sixty-four consecutive patients with PVAVSs from 2 institutes were retrospectively reviewed. Lesions were classified as type A (n=28) if the intradural veins were involved in drainage; otherwise, they were classified as type B (n=36). The clinical course from initial presentation to the last follow-up was analyzed. Results: The patients with type A shunts were older at presentation (52.5 versus 35.5 years, P <0.0001) and more likely to have lower spinal segments affected than patients with type B PVAVSs (67.8% versus 13.9%, P =0.00006). After presentation, the deterioration rates related to gait and sphincter dysfunction were significantly higher in patients with type A than type B shunts (gait dysfunction: 71.8%/y versus 17.0%/y, P =0.0006; sphincter dysfunction: 63.7%/y versus 11.3%/y, P =0.0002). According to the angiogram at the end of the latest treatment, 79% of type A and 75% of type B PVAVSs were completely obliterated. If the lesions were partially obliterated, a significantly higher clinical deterioration rate was observed in patients with type A shunts than those with type B shunts (69.9%/y versus 3.2%/y, P =0.0253). Conclusions: Type A PVAVSs feature rapid progressive neurological deficits; therefore, early clinical intervention is necessary. For complex lesions that cannot be completely obliterated, surgical disconnection of all refluxed radicular veins is suggested.
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.
BACKGROUND: Two of three patients who were consecutively treated within the past 7 years using traditional methods in our center presented with recurrence and even aggravation during follow-up. Therefore, a new approach was developed to treat a recent patient to provide for complete decompression of the spinal cord. OBJECTIVE: To illustrate a new treatment modality for patients with cervical spinal cord compression resulting from aberrant vertebral arteries. METHODS: The aberrant vertebral arteries were freed after substantial dissection of their dural rings. The compression was released after moving the aberrant vertebral arteries, which were sutured to the lateral mass to the extradural space. New dural rings above the atlas were created to allow the vertebral artery to enter the dura mater into a wider subdural space. RESULTS: The patient experienced full relief from her initial complaints except for mild numbness of the bilateral lower extremities immediately after surgery. Postoperative imaging studies indicated successful transposition of bilateral aberrant vertebral arteries and significant decompression of the spinal cord. Her symptoms did not recur over a 6-month follow-up period. Imaging studies at the 6-month follow-up showed stable vertebral artery transposition and spinal cord decompression. CONCLUSION: A patient with cervical spinal cord compression resulting from aberrant vertebral arteries was successfully treated by our new technique, which includes vertebral artery transposition, fixation, and dural ring reconstruction. Our approach could be an alternative for durable spinal cord decompression in such patients.
Arteriovenous malformations (AVMs) of the central nervous system are high-flow arteriovenous shunts that lead to considerable risks of hemorrhagic stroke and neurological deficits in young patients. Due to the complex angioarchitecture and their close anatomical relationship with the brain and spinal cord, the management of brain and spinal AVMs is challenging. Conventional invasive treatments, including microsurgery, endovascular embolization, and stereotactic radiosurgery, are associated with considerable risks and unsatisfactory efficacy. In addition, the lack of medications for AVMs represents an unmet clinical need. In recent years, the pathogenesis of AVMs has been progressively explored. The increased understanding of the mechanisms of the formation, progression, and rupture of AVMs has opened up several potential directions for AVM pharmacotherapy. In recent years, some promising drugs targeting angiogenesis, inflammation, vessel wall integrity, and the mitogen-activated protein kinase (MAPK)-extracellular receptor kinase (ERK) signaling pathway have been tested in a series of clinical investigations. In this review, we summarize the potential mechanisms, preliminary efficacy, and side effects of the candidate medications, including bevacizumab, minocycline or doxycycline, thalidomide, and trametinib, in the treatment of brain and spinal AVMs.
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