Seizure Related 6 Homolog Like 2 (SEZ6L2) protein has been shown to have implications in neuronal and especially motor function development. In oncology, overexpression of SEZ6L2 serves as a negative prognostic marker in several tumor entities. Recently, few cases of anti-SEZ6L2 antibody mediated cerebellar syndromes were reported. In this article, we present a case of a 70-year-old woman with subacute onset of gait disturbance, dysarthria and limb ataxia. Serum anti-SEZ6L2 antibodies were markedly increased, and further diagnostic workup revealed left sided breast cancer. Neurological symptoms and SEZ6L2 titer significantly improved after curative tumor therapy. This is a very rare and educationally important report of anti-SEZ6L2 autoimmune cerebellar syndrome with a paraneoplastic etiology. Additionally, we performed a review of the current literature for SEZ6L2, focusing on comparing the published cases on autoimmune cerebellar syndrome.

Objectives The aim of this study was to demonstrate the efficacy and feasibility of treating patients with extracranial arteriovenous malformations (AVM) of the head and neck with interventional embolization followed by surgical resection. Methods We reviewed the charts of all patients between 2012 and 2021 with extracranial AVM of the head and neck scheduled for interdisciplinary treatment according to University Hospital RWTH Aachen’s protocol and conducted standardized interviews using a newly developed questionnaire. Interview results, as well as clinical examination and radiographic outcome results were analyzed to help determine the efficacy of our treatment approach. Results We included 10 patients (8 female, 2 male), with a mean age of 33.5 (11–61) years who were scheduled for treatment of the AVM with interventional embolization followed by surgical resection. In 6 of the 10 patients (60%) the lesion was located in extracranial soft tissue only. In one patient (10%), the lesion was located in bone tissue only. A combined intraosseous and oral soft tissue lesion was seen in the remaining 3 patients (30%). Radiographic resolution was achieved in 62.5% of cases and a significant decrease of symptoms was identified (p = 0.002). None of the patients reported dissatisfaction and no major complications occurred. Conclusion An interdisciplinary treatment approach combining neuroradiological interventions with surgical resection appears to be an effective treatment with an acceptable complication rate. Patients treated according to our protocol showed a high satisfaction rate, regardless of the radiographic outcome. Standardized follow-up allows for early detection of recurrences and helps with subjective patient satisfaction.

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BackgroundCatheter size, location and circle of Willis anatomy impact the flow conditions during interventional stroke therapy. The aim of the study was to systematically investigate the influence of these factors on flow control in the middle cerebral artery by means of a computational model based on 100 patients with stroke who received endovascular treatment.MethodsThe dimensions of the cervical and intracranial cerebral arteries of 100 patients who received endovascular mechanical thrombectomy for acute ischemic stroke were measured and a three-dimensional model of the circle of Willis was created based on these data. Flow control in the middle cerebral artery with variations in catheter size, catheter location and configurations of collateral vessels was determined using a computational model. A total of 48 scenarios were analyzed.ResultsFlow reversal with a distal aspiration catheter alone was not possible in the internal carotid artery and only sometimes possible in the middle cerebral artery (14 of 48 cases). The Catalyst 7 catheter was more often successful in achieving flow reversal than Catalyst 5 or 6 catheters (p<0.001). In a full circle of Willis anatomy, flow reversal was almost never possible. The absence of one or more communicating arteries significantly influenced flow direction compared with the full anatomy with all communicating arteries present (p=0.028).ConclusionChoosing the biggest possible aspiration catheter and locating it in the middle cerebral artery significantly increases the chances of successful flow control. Flow through the collaterals may impair the flow, and circle of Willis anatomy should be considered during aspiration thrombectomy.

Objectives The primary aim of this study was to conduct a meta-analysis of the literature on interventional treatment for patients with extracranial AVM of the head and neck to identify a superior treatment. The secondary aim was to evaluate the methodological quality of associated articles published between 2000–2020. Methods The literature search was conducted on PubMed, Embase, the Cochrane Library, and scholar.google.com. Studies, meeting the acceptable reference standard underwent meta-analysis. All identified literature underwent methodological quality analysis. Results Of 1560 screened articles, 56 were included in the literature review. Appropriate diagnostic tests were reported in 98% of included articles. 13% of included articles did not specify the embolization agent. Outcome analysis varied throughout. 45% of the authors used radiographic imaging for follow-up. 77% specified the span of follow-up of their entire patient collective. Two articles met the inclusion criteria for meta-analysis. Curing rate of transarterial ethanol embolization for intraosseous AVM was 83% with a complication rate of 58%. Curing rate of ethanol combined with NBCA or Onyx in soft tissue AVM was 18% with a complication rate of 87%. Conclusion Our literature review revealed an absence of treatment or reporting standards for extracranial AVM of the head and neck. The meta-analysis is comprised of two articles and methodological quality is heterogeneous. We recommend implementing consistent reporting standards to facilitate comparability of studies and to provide robust data for the development of an evidence-based treatment strategy. Advances in knowledge Meta-analysis showed a favorable radiological outcome for intraosseous AVM when treated with intraarterial ethanol embolization. Our analysis demonstrated that the published data on extracranial AVMs of the head and neck is lacking in consistency and quality, prompting agreement for the need of standardized reporting on AVM treatments.