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Correction to: MRI-based prediction of DNA methylation grade in IDH-mutant astrocytomas using qualitative imaging features and tumor volumetrics. 更正:利用定性成像特征和肿瘤体积,基于mri预测idh突变星形细胞瘤的DNA甲基化等级。
IF 2.6 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-02-02 DOI: 10.1007/s00234-026-03935-8
Kanwar Partap Bir Singh, Matthew D Lee, Matthew G Young, Daniel Orringer, Yuxiu Wang, Matija Snuderl, Rajan Jain
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引用次数: 0
Recurrent left internal carotid artery stenosis in a patient with intrathoracic carotid bifurcation and right aortic arch. 胸内颈动脉分叉伴右主动脉弓患者复发性左颈内动脉狭窄1例。
IF 2.6 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-02-02 DOI: 10.1007/s00234-025-03773-0
Daiichiro Ishigami, Wataro Tsuruta, Satoshi Miyamoto, Shuhei Egashira, Jun Isozaki

Intrathoracic carotid bifurcation (ITCB) is a rare vascular anomaly often associated with Klippel-Feil syndrome (KFS); however, its coexistence with tetralogy of Fallot (TOF) has not been previously reported. A male patient in his 30s with childhood TOF repair presented with mild right-sided hemiparesis and motor aphasia due to an ischemic infarction. Cerebral angiography revealed ITCB at the T2 level with severe left internal carotid artery stenosis, a right aortic arch, and aberrant left subclavian artery. Percutaneous transluminal angioplasty initially resolved the stenosis, but progressive restenosis required carotid artery stenting, achieving sustained patency. Embryologically, neural crest anomalies and persistence of fetal vascular structures, including the ductus caroticus and the ductus arteriosus, may underpin ITCB associated with TOF. Carotid artery stenting in this setting is technically more challenging, as the guide catheter must be positioned unusually low in the thorax, risking proximal slipping of the guiding system into the aorta.

胸内颈动脉分叉(ITCB)是一种罕见的血管异常,通常与Klippel-Feil综合征(KFS)有关;然而,其与法洛四联症(TOF)的共存尚未见报道。一例30多岁的男性儿童TOF修复患者因缺血性梗塞而出现轻度右侧偏瘫和运动失语。脑血管造影显示T2水平ITCB伴严重左侧颈内动脉狭窄,右侧主动脉弓,左侧锁骨下动脉异常。经皮腔内血管成形术最初解决狭窄,但进行性再狭窄需要颈动脉支架,以实现持续通畅。胚胎学上,神经嵴异常和胎儿血管结构的持续存在,包括颈动脉导管和动脉导管,可能是TOF相关的ITCB的基础。在这种情况下,颈动脉支架置入在技术上更具挑战性,因为引导导管必须放置在胸腔异常低的位置,有引导系统近端滑入主动脉的风险。
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引用次数: 0
European Society of Neuroradiology (ESNR). 欧洲神经放射学会。
IF 2.6 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-01-31 DOI: 10.1007/s00234-026-03919-8
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引用次数: 0
Safety and efficacy of the pipeline vantage flow diverter in small cerebral vessels ≤2.5 mm: a case series. 管道优势分流器在≤2.5 mm小脑血管中的安全性和有效性:一个病例系列。
IF 2.6 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-01-31 DOI: 10.1007/s00234-026-03915-y
Kaishin Tanaka, Alice Ma, Kenneth Faulder, Timothy Harrington, Brendan Steinfort

Background: The Pipeline Vantage Flow Diverter (PVFD) with shield technology is the fourth and latest generation of the Pipeline embolization devices which are flow diverting stents used in the treatment of cerebral aneurysms. In early 2025 the larger PVFD 027s were recalled due to concerns of braid deformities with updated instructions provided for the PVFD 021. This study aimed at evaluating the safety and efficacy of the PVFD for cerebral aneurysm treatment in small parent vessels ≤ 2.5 mm.

Methods: A retrospective review of patients who received a 2.5 mm diameter PVFD for cerebral aneurysm treatment at our institution over 4 years was performed. Baseline patient characteristics, complications, clinical and radiological outcomes were collected for each patient with a mean follow up duration of 1 year and 17 days (median 7 months and 5 days).

Results: There were a total of 14 patients identified with a total of 16 aneurysms treated. At latest follow up complete aneurysm occlusion was achieved in 10 (66.7%) out of 16 treated aneurysms with 1 patient still awaiting follow up imaging. There were 2 (14.3%) of 14 patients who had immediate neurological complications, of which 1 was a transient deficit, and 0 patients with delayed complications. In-stent stenosis was found in 2 (15.4%) of 13 patients and asymptomatic stent occlusion occurred in 1 patient (7.69%).

Conclusions: This case series demonstrated an acceptable safety profile with reasonable aneurysm occlusion rates for the use of PVFD in cerebral vessels ≤ 2.5 mm in treating aneurysms.

背景:采用屏蔽技术的管道优势分流器(PVFD)是第四代也是最新一代的管道栓塞装置,是用于治疗脑动脉瘤的分流支架。在2025年初,较大的PVFD 027由于担心编织变形而被召回,并为PVFD 021提供了更新的说明。本研究旨在评估PVFD治疗≤2.5 mm小母血管脑动脉瘤的安全性和有效性。方法:回顾性分析我院4年来接受直径2.5 mm PVFD治疗脑动脉瘤的患者。收集每位患者的基线患者特征、并发症、临床和影像学结果,平均随访时间为1年17天(中位数为7个月5天)。结果:共发现14例患者,治疗16个动脉瘤。在最近的随访中,16例治疗动脉瘤中有10例(66.7%)动脉瘤完全闭塞,1例患者仍在等待随访影像学检查。14例患者中有2例(14.3%)出现即时神经系统并发症,其中1例为一过性缺损,0例为迟发性并发症。13例患者中2例(15.4%)发生支架内狭窄,1例(7.69%)发生无症状支架闭塞。结论:本病例系列表明PVFD在治疗≤2.5 mm脑血管动脉瘤时具有可接受的安全性和合理的动脉瘤闭塞率。
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引用次数: 0
RECOAT study - Retrospective evaluation of surface-modified and coated flow diverters for the treatment of unruptured intracranial aneurysms. RECOAT研究:回顾性评价表面修饰和涂层分流器治疗未破裂颅内动脉瘤的疗效。
IF 2.6 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-01-31 DOI: 10.1007/s00234-025-03873-x
Lukas Goertz, Sophia Hohenstatt, Dominik F Vollherbst, Charlotte S Weyland, Omid Nikoubashman, Hanna Styczen, Christian Gronemann, Daniel A Weiss, Marius Kaschner, Johannes Rueckel, Muriel Pflaeging, Eberhardt Siebert, David Zopfs, Jonathan Kottlors, Lenhard Pennig, Marc Schlamann, Georg Bohner, Bernd Turowski, Franziska Dorn, Cornelius Deuschl, Martin Wiesmann, Thomas Liebig, Markus A Möhlenbruch, Christoph Kabbasch

Purpose: Surface modifications may improve procedural safety of flow diverter (FD) treatment, but larger studies are rare. This multicenter study aims to evaluate the safety and efficacy of unruptured aneurysm treatment with surface-modified and coated FDs.

Methods: Patients treated with the following FDs at 8 neurovascular centers were retrospectively reviewed: Pipeline Vantage Embolization Device, Pipeline Flex with Shield Technology, FRED X, p48/64 HPC, Derivo Embolization Device (DED), and DED 2heal. Aneurysm characteristics, procedural details, and angiographic results were evaluated in detail.

Results: A total of 511 patients with 545 aneurysms were included (mean aneurysm size: 8.2 ± 5.5 mm, 12% posterior circulation, 14% non-saccular morphology). Multiple FDs were used in 26/515 (5%) procedures and adjunctive coiling in 76 (15%). Thromboembolic events occurred in 40 (7.8%) cases, including 9 (1.7%) major ischemic events, 20 (3.9%) minor ischemic events and 11 (2.1%) asymptomatic/technical events. Non-saccular aneurysm morphology (p = 0.014) and use of multiple FDs (p = 0.025) favored thromboembolic events. Hemorrhagic events occurred in 5 (1.0%) cases, of which 3 were symptomatic. The combined morbidity and mortality rate was 11/515 (2.1%). Complete and adequate occlusion rates were 66% (196/297) and 80% (238/297) at 6 months, respectively, and 70% (64/92) and 85% (78/92) at 12 months, respectively.

Conclusion: The present study demonstrates high safety and efficacy for coated or surface-modified FDs. Whether the surface modifications confer a clinical benefit needs to be addressed in comparative studies.

目的:表面修饰可以提高分流器(FD)治疗的安全性,但大规模的研究很少。这项多中心研究旨在评估表面修饰和涂层fd治疗未破裂动脉瘤的安全性和有效性。方法:回顾性分析在8个神经血管中心接受以下fd治疗的患者:Pipeline Vantage Embolization Device, Pipeline Flex with Shield Technology, FRED X, p48/64 HPC, derived Embolization Device (DED)和DED 2heal。动脉瘤特征,手术细节和血管造影结果进行了详细评估。结果:共纳入511例动脉瘤545例(平均动脉瘤大小8.2±5.5 mm,后循环12%,非囊状形态14%)。515例中有26例(5%)使用了多个fd, 76例(15%)使用了辅助卷绕。40例(7.8%)发生血栓栓塞事件,包括9例(1.7%)严重缺血事件,20例(3.9%)轻微缺血事件和11例(2.1%)无症状/技术性事件。非囊性动脉瘤形态(p = 0.014)和使用多个fd (p = 0.025)有利于血栓栓塞事件。5例(1.0%)发生出血事件,其中3例有症状。总发病率和死亡率为11/515(2.1%)。6个月时完全和充分闭塞率分别为66%(196/297)和80%(238/297),12个月时完全和充分闭塞率分别为70%(64/92)和85%(78/92)。结论:本研究表明包被或表面修饰的FDs具有较高的安全性和有效性。表面修饰是否具有临床益处需要在比较研究中解决。
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引用次数: 0
Widened arterial time-density curves of CTP may identify cardioembolic etiology in acute large-artery occlusion stroke. CTP增宽动脉时间-密度曲线可鉴别急性大动脉闭塞性卒中的心栓塞病因。
IF 2.6 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-01-30 DOI: 10.1007/s00234-026-03914-z
Ludan Chen, Zhuhao Li, Dingxiang Xie, Liping Lin, Jingjing Li, Zhiyun Yang, Li Jiang, Wenquan Zhuang, Jing Zhao

Background: Identifying cardioembolism (CE) in large artery occlusion acute ischemic stroke (LAO-AIS) is critical for treatment decisions but often requires time-consuming diagnostic workups. Arterial and venous time-density curves (ATDC and VTDC), automatically derived from brain CTP, may reflect cardiac dysfunction and thrombus formation, offering a rapid alternative for CE detection.

Purpose: To evaluate the diagnostic utility of CTP-derived ATDC and VTDC parameters, combined with thrombus attenuation, for identifying CE in LAO-AIS patients.

Materials and methods: This retrospective study analyzed 103 LAO-AIS patients (median age 69 [IQR 63-77] years; 63 men) with anterior circulation occlusions who underwent CTP within 24 h of onset. Patients were classified into CE (n = 56) and non-CE (n = 47) groups per TOAST criteria. ATDC and VTDC parameters (onset, peak, end, width) and thrombus CT attenuation were measured. Diagnostic performance was assessed using univariable and multivariable logistic regression.

Results: Compared to the non-CE group, the CE group exhibited significant delays in onset, peak, and end times, along with wider curve widths of ATDC and VTDC, and higher thrombus attenuation (PFDR≤0.001). ATDC width demonstrated the highest discriminative power for CE (AUC: 0.83 [95%CI: 0.74-0.89]; sensitivity: 73.2%, specificity: 87.2%; cutoff-value: 27s). Multivariable analysis confirmed ATDC width (OR = 1.214; 95% CI: 1.080-1.365; P = 0.001) and thrombus attenuation (OR = 1.114; 95% CI: 1.006-1.233; P = 0.038) as independent CE predictors, with a combined AUC of 0.93.

Conclusions: Prolonged ATDC and VTDC phases, wider curve widths, and elevated thrombus attenuation on CTP are strongly associated with CE in LAO-AIS, providing a rapid, imaging-based diagnostic tool.

背景:在大动脉闭塞性急性缺血性卒中(LAO-AIS)中识别心脏栓塞(CE)对治疗决策至关重要,但通常需要耗时的诊断检查。动脉和静脉时间密度曲线(ATDC和VTDC),自动从脑CTP中提取,可能反映心功能障碍和血栓形成,为CE检测提供了快速的替代方法。目的:评价ctp衍生的ATDC和VTDC参数,结合血栓衰减,对LAO-AIS患者CE的诊断价值。材料和方法:本回顾性研究分析了103例LAO-AIS患者(中位年龄69 [IQR 63-77]岁,男性63例),均为前循环闭塞,在发病24小时内行CTP。根据TOAST标准将患者分为CE组(n = 56)和非CE组(n = 47)。测量ATDC和VTDC参数(起、峰、端、宽)和血栓CT衰减。采用单变量和多变量logistic回归评估诊断效果。结果:与非CE组相比,CE组表现出明显的发病、峰值和结束时间延迟,ATDC和VTDC曲线宽度更宽,血栓衰减更高(PFDR≤0.001)。ATDC宽度对CE的鉴别能力最高(AUC: 0.83 [95%CI: 0.74-0.89];敏感性:73.2%,特异性:87.2%;截止值:27s)。多变量分析证实ATDC宽度(OR = 1.214; 95% CI: 1.080-1.365; P = 0.001)和血栓衰减(OR = 1.114; 95% CI: 1.006-1.233; P = 0.038)是独立的CE预测因子,合并AUC为0.93。结论:LAO-AIS的ATDC和VTDC期延长、曲线宽度变宽、CTP上血栓衰减升高与CE密切相关,提供了一种快速的、基于成像的诊断工具。
{"title":"Widened arterial time-density curves of CTP may identify cardioembolic etiology in acute large-artery occlusion stroke.","authors":"Ludan Chen, Zhuhao Li, Dingxiang Xie, Liping Lin, Jingjing Li, Zhiyun Yang, Li Jiang, Wenquan Zhuang, Jing Zhao","doi":"10.1007/s00234-026-03914-z","DOIUrl":"https://doi.org/10.1007/s00234-026-03914-z","url":null,"abstract":"<p><strong>Background: </strong>Identifying cardioembolism (CE) in large artery occlusion acute ischemic stroke (LAO-AIS) is critical for treatment decisions but often requires time-consuming diagnostic workups. Arterial and venous time-density curves (ATDC and VTDC), automatically derived from brain CTP, may reflect cardiac dysfunction and thrombus formation, offering a rapid alternative for CE detection.</p><p><strong>Purpose: </strong>To evaluate the diagnostic utility of CTP-derived ATDC and VTDC parameters, combined with thrombus attenuation, for identifying CE in LAO-AIS patients.</p><p><strong>Materials and methods: </strong>This retrospective study analyzed 103 LAO-AIS patients (median age 69 [IQR 63-77] years; 63 men) with anterior circulation occlusions who underwent CTP within 24 h of onset. Patients were classified into CE (n = 56) and non-CE (n = 47) groups per TOAST criteria. ATDC and VTDC parameters (onset, peak, end, width) and thrombus CT attenuation were measured. Diagnostic performance was assessed using univariable and multivariable logistic regression.</p><p><strong>Results: </strong>Compared to the non-CE group, the CE group exhibited significant delays in onset, peak, and end times, along with wider curve widths of ATDC and VTDC, and higher thrombus attenuation (P<sub>FDR</sub>≤0.001). ATDC width demonstrated the highest discriminative power for CE (AUC: 0.83 [95%CI: 0.74-0.89]; sensitivity: 73.2%, specificity: 87.2%; cutoff-value: 27s). Multivariable analysis confirmed ATDC width (OR = 1.214; 95% CI: 1.080-1.365; P = 0.001) and thrombus attenuation (OR = 1.114; 95% CI: 1.006-1.233; P = 0.038) as independent CE predictors, with a combined AUC of 0.93.</p><p><strong>Conclusions: </strong>Prolonged ATDC and VTDC phases, wider curve widths, and elevated thrombus attenuation on CTP are strongly associated with CE in LAO-AIS, providing a rapid, imaging-based diagnostic tool.</p>","PeriodicalId":19422,"journal":{"name":"Neuroradiology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2026-01-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146086678","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Preliminary assessment of an MRI-based grading system for leptomeningeal disease: an exploratory prognostic framework. 基于mri的脑膜疾病分级系统的初步评估:一个探索性的预后框架。
IF 2.6 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-01-26 DOI: 10.1007/s00234-026-03904-1
Ahmed Msherghi, Maria Glue-Monroe, Rami W Eldaya, Maryam Pirhoushiaran, Heba Al Qudah, Ceylan Altintas Taslicay, Sahar Alizada, Hamza A Salim, Leomar Y Ballester, Max Wintermark

Background: Current diagnostic approaches of leptomeningeal disease (LMD) rely heavily on cerebrospinal fluid (CSF) cytology, which shows significant limitations and the requirement for invasive procedures. We aim to develop an MRI-based grading scores for LMD diagnosis and prognosis that address current diagnostic limitations and provide standardized, reproducible assessment criteria.

Methods: We conducted a retrospective analysis of 32 adult cancer patients evaluated for suspected LMD. Two experienced neuroradiologists independently assessed MRI studies using our novel grading system, which incorporates leptomeningeal enhancement/intensity patterns (grades 1-6), Evans index for hydrocephalus assessment, brain metastases characteristics, and spinal involvement. Confirmation of LMD cases was employed using dual confirmation approach combining CSF cytology and follow-up MRI.

Results: Our MRI grading system demonstrated promising inter-observer performance. Inter-rater reliability between two attending level neuroradiologists was excellent (ICC = 0.953, P-value < 0.001) using a cutoff score of 2 or higher, the system demonstrated comparable performance. Risk stratification analysis revealed clear prognostic value, with mortality rates of 8.6% for low-risk patients (Grade 1-2), 50% for medium-risk patients (Grade 3-4), and 80.0% for high-risk patients (Grade 5 +). The Kaplan-Meier survival curves demonstrate a statistically significant difference in overall survival between patients with varying grades (p-value of 0.00011). Notably, survival probability drops steeply in the Grade 5 + group early on, suggesting that higher LMD burden is associated with rapid clinical deterioration. In contrast, low risk patients appear to have a more indolent course.

Conclusions: Our preliminary findings detail a promising approach in evaluating LMD patients which offers valuable prognostic information for clinical decision making. Furthermore, the high inter-rater reliability across various tumor types further encourages the potential utility of this approach, although further research on a broader population is needed before clinical implementation.

背景:目前轻脑膜病(LMD)的诊断方法严重依赖脑脊液(CSF)细胞学,这显示出明显的局限性和侵入性手术的要求。我们的目标是开发一种基于mri的LMD诊断和预后分级评分,以解决当前的诊断局限性,并提供标准化、可重复的评估标准。方法:我们对32例疑似LMD的成年癌症患者进行回顾性分析。两位经验丰富的神经放射学家使用我们的新分级系统独立评估MRI研究,该分级系统包括脑膜增强/强度模式(1-6级)、脑积水评估的Evans指数、脑转移特征和脊柱受累。LMD病例的确诊采用脑脊液细胞学和随访MRI相结合的双重确诊方法。结果:我们的MRI分级系统在观察者间表现良好。两名主治级别的神经放射科医生之间的评分间信度非常好(ICC = 0.953, p值)。结论:我们的初步研究结果详细描述了一种评估LMD患者的有希望的方法,为临床决策提供了有价值的预后信息。此外,不同肿瘤类型间的高可靠性进一步鼓励了这种方法的潜在效用,尽管在临床应用之前需要对更广泛的人群进行进一步的研究。
{"title":"Preliminary assessment of an MRI-based grading system for leptomeningeal disease: an exploratory prognostic framework.","authors":"Ahmed Msherghi, Maria Glue-Monroe, Rami W Eldaya, Maryam Pirhoushiaran, Heba Al Qudah, Ceylan Altintas Taslicay, Sahar Alizada, Hamza A Salim, Leomar Y Ballester, Max Wintermark","doi":"10.1007/s00234-026-03904-1","DOIUrl":"https://doi.org/10.1007/s00234-026-03904-1","url":null,"abstract":"<p><strong>Background: </strong>Current diagnostic approaches of leptomeningeal disease (LMD) rely heavily on cerebrospinal fluid (CSF) cytology, which shows significant limitations and the requirement for invasive procedures. We aim to develop an MRI-based grading scores for LMD diagnosis and prognosis that address current diagnostic limitations and provide standardized, reproducible assessment criteria.</p><p><strong>Methods: </strong>We conducted a retrospective analysis of 32 adult cancer patients evaluated for suspected LMD. Two experienced neuroradiologists independently assessed MRI studies using our novel grading system, which incorporates leptomeningeal enhancement/intensity patterns (grades 1-6), Evans index for hydrocephalus assessment, brain metastases characteristics, and spinal involvement. Confirmation of LMD cases was employed using dual confirmation approach combining CSF cytology and follow-up MRI.</p><p><strong>Results: </strong>Our MRI grading system demonstrated promising inter-observer performance. Inter-rater reliability between two attending level neuroradiologists was excellent (ICC = 0.953, P-value < 0.001) using a cutoff score of 2 or higher, the system demonstrated comparable performance. Risk stratification analysis revealed clear prognostic value, with mortality rates of 8.6% for low-risk patients (Grade 1-2), 50% for medium-risk patients (Grade 3-4), and 80.0% for high-risk patients (Grade 5 +). The Kaplan-Meier survival curves demonstrate a statistically significant difference in overall survival between patients with varying grades (p-value of 0.00011). Notably, survival probability drops steeply in the Grade 5 + group early on, suggesting that higher LMD burden is associated with rapid clinical deterioration. In contrast, low risk patients appear to have a more indolent course.</p><p><strong>Conclusions: </strong>Our preliminary findings detail a promising approach in evaluating LMD patients which offers valuable prognostic information for clinical decision making. Furthermore, the high inter-rater reliability across various tumor types further encourages the potential utility of this approach, although further research on a broader population is needed before clinical implementation.</p>","PeriodicalId":19422,"journal":{"name":"Neuroradiology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2026-01-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146046781","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Neuroimaging in inborn errors of immunity: More than infections. 先天性免疫缺陷的神经影像学:多于感染。
IF 2.6 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-01-26 DOI: 10.1007/s00234-025-03895-5
Pedro Sousa Brandão, Carla Conceição, João Farela Neves

Purpose: Inborn errors of immunity (IEIs) are a heterogeneous group of germline disorders whose recognition is increasing but which remain underdiagnosed and associated with significant pediatric morbidity and mortality. This review aims to systematize the main neuroimaging manifestations of IEIs, addressing how neuroradiologists can identify characteristic patterns to support earlier diagnosis and guide clinical management.

Methods: A narrative review of IEIs with neurological involvement was performed, focusing on reported primary and secondary neuroimaging features across major IEI categories. Imaging findings were correlated with underlying immune defects, typical clinical phenotypes, and their impact on diagnostic workup, risk assessment, and genetic counseling.

Results: IEIs demonstrate a broad neurological spectrum that extends beyond recurrent infections to encompass autoinflammatory, autoimmune, allergic, and malignant phenotypes with central nervous system involvement. Neuroimaging reveals both primary manifestations directly related to immune dysregulation and secondary features, allowing recognition of patterns in IEI subgroups that can provide crucial diagnostic clues in otherwise non-specific clinical scenarios.

Conclusion: Neurological involvement in IEIs is not uncommon and may be radiologically detectable, making imaging an important ancillary tool in their evaluation. Systematic recognition of primary and secondary neuroimaging features by neuroradiologists can raise suspicion for an underlying IEI, prompting appropriate immunologic and genetic work-up. Earlier diagnosis is crucial to enable timely implementation of preventive or targeted therapies, with the potential to improve long-term outcomes.

目的:先天性免疫错误(IEIs)是一组异质性生殖系疾病,其认可度不断提高,但仍未得到充分诊断,并与显著的儿科发病率和死亡率相关。本综述旨在系统化iei的主要神经影像学表现,探讨神经放射学家如何识别特征模式以支持早期诊断和指导临床管理。方法:对神经系统受累的IEI进行叙述性回顾,重点报道主要IEI类别的原发性和继发性神经影像学特征。影像学结果与潜在的免疫缺陷、典型的临床表型及其对诊断检查、风险评估和遗传咨询的影响相关。结果:IEIs表现出广泛的神经系统频谱,超出复发性感染,包括自身炎症、自身免疫、过敏和恶性表型,并累及中枢神经系统。神经影像学显示了与免疫失调直接相关的主要表现和次要特征,允许识别IEI亚组的模式,可以在其他非特异性临床情景中提供关键的诊断线索。结论:iei的神经系统受累并不罕见,放射学上可以检测到,使影像学成为评估其重要的辅助工具。神经放射学家对原发性和继发性神经影像学特征的系统识别可以引起对潜在IEI的怀疑,从而促使适当的免疫和遗传检查。早期诊断对于及时实施预防性或靶向治疗至关重要,有可能改善长期结果。
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引用次数: 0
Improving catheter navigation in neurointerventional procedures: single-center insights on next-generation steerable guidewires. 神经介入手术中导管导航的改进:下一代可操纵导丝的单中心见解。
IF 2.6 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-01-24 DOI: 10.1007/s00234-026-03907-y
Om H Gandhi, Suraj R Dumasia, Sami Almasri, Nathan Yu, Erin N Walker, Linda Bagley, Omar A Choudhri

Purpose: Steerable guidewires with deflectable tips enable real-time navigation through complex cerebrovascular anatomy without requiring wire removal and tip reshaping. Despite promising early reports, systematic clinical evaluation remains limited.

Methods: We conducted a retrospective analysis of 58 consecutive neurointerventional procedures utilizing steerable guidewires at two centers from September 2024 to August 2025. The cohort comprised 48 cases using the Drivewire 24 (DW24, 0.024-inch diameter) and 10 cases using the Artiria SmartGUIDE (0.014-inch diameter). Primary outcomes included technical success, safety profile, and operator assessment. We also performed a scoping literature review of all published steerable guidewire studies in neurointerventional applications.

Results: Technical success in reaching target vessels was achieved in 100% of cases for both systems with no intraoperative or postoperative complications. Mean fluoroscopy time was 32.7 ± 29.5 min for DW24 and 26.1 ± 16.2 min for Artiria procedures. Treatment indications included aneurysm procedures (53.4%), diagnostic angiographies (20.7%), venous sinus stenting (13.8%), middle meningeal artery embolizations (8.6%), and stroke interventions (3.4%). 8.3% of procedures using DW24 were considered higher-risk by the primary operator without steerable capability, including successful navigation of complex posterior circulation anatomy in giant basilar aneurysms and recurrent superior cerebellar artery aneurysms with prior stent constructs. Our limited experience with the Artiria system (n = 10) revealed operator-noted challenges with shape retention. The scoping review identified only three prior studies encompassing 65 procedures, establishing this as the largest reported experience.

Conclusion: Both steerable guidewire systems achieved perfect technical success with excellent safety profiles. Based on our preliminary experience, the DW24 appeared to offer advantages for procedures requiring navigating larger catheters through tortuous anatomy. These findings support selective clinical use of steerable guidewire technology in challenging neurointerventional procedures.

目的:具有可偏折尖端的可操纵导丝能够实时导航复杂的脑血管解剖结构,而无需拆除导丝和尖端重塑。尽管早期的报告很有希望,但系统的临床评估仍然有限。方法:回顾性分析2024年9月至2025年8月在两个中心连续58例使用可操纵导丝的神经介入手术。该队列包括48例使用Drivewire 24 (DW24,直径0.024英寸)和10例使用Artiria SmartGUIDE(直径0.014英寸)。主要结果包括技术成功、安全性概况和操作人员评估。我们还对所有已发表的可导向导丝在神经介入应用方面的研究进行了文献综述。结果:两种系统均100%成功到达靶血管,无术中或术后并发症。DW24的平均透视时间为32.7±29.5 min, artiia的平均透视时间为26.1±16.2 min。治疗指征包括动脉瘤手术(53.4%)、诊断性血管造影(20.7%)、静脉窦支架植入术(13.8%)、脑膜中动脉栓塞(8.6%)和卒中干预(3.4%)。8.3%的使用DW24的手术被没有操作能力的主要操作者认为是高风险的,包括成功导航复杂的后循环解剖的巨基底动脉瘤和复发的小脑上动脉瘤,这些动脉瘤之前已经安装了支架。我们使用artiia系统的有限经验(n = 10)揭示了操作员注意到的形状保持问题。范围审查只确定了三个先前的研究,包括65个程序,确定这是报告的最大经验。结论:两种导向导丝系统均取得了完美的技术成功,具有良好的安全性。根据我们的初步经验,DW24似乎为需要通过弯曲解剖引导较大导管的手术提供了优势。这些发现支持在具有挑战性的神经介入手术中选择性地使用可操纵导丝技术。
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引用次数: 0
The diagnostic value of cortical high-flow sign combined with cystathionine on 1H-MRS for prediction of 1p/19q-codeletion status in IDH-mutant adult-type diffuse glioma. 皮质高流量征象联合半胱硫氨酸1H-MRS预测idh突变成人型弥漫性胶质瘤1p/19q编码状态的诊断价值
IF 2.6 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-01-24 DOI: 10.1007/s00234-026-03909-w
Koji Yamashita, Moyoko Tomiyasu, Kazufumi Kikuchi, Daichi Momosaka, Masaoki Kusunoki, Daisuke Kuga, Ryusuke Hatae, Yutaka Fujioka, Ryosuke Otsuji, Osamu Togao, Koji Yoshimoto, Kousei Ishigami

Objectives: To leverage a combination of cortical high-flow sign on arterial spin labeling (ASL) and cystathionine measurement using 1H-MR spectroscopy (1H-MRS) to distinguish oligodendroglioma, IDH-mutant and 1p/19q-codeleted (O_IDHm-codel) from astrocytoma, IDH-mutant (A_IDHm-noncodel).

Materials and methods: We implemented pseudo-continuous ASL technique (post-labeling delay = 2000 ms) using a 3.0-T MRI scanner. Relative perfusion maps were generated by subtracting paired labeled images from control images. 1H-MRS data were acquired by using the single-voxel point-resolved spectroscopy sequence (PRESS) sequence (TR = 2000 ms, TE = 97 ms, NEX = 128, volume of interest = 203 mm3). Our study included cases with a Cramér-Rao Lower Bound of cystathionine levels at 2.7 ppm that were below 50%. The presence or absence of cortical high-flow sign and the estimated concentration of cystathionine were compared between A_IDHm-noncodel and O_IDHm-codel. The receiver operating characteristic curves were used to evaluate the diagnostic performance of each parameter, as well as the combination of both.

Results: The cortical high-flow sign was identified more frequent in O_IDHm-codel (7/12, 58.3%) than in A_IDHm-noncodel (3/18, 16.7%; p = 0.018). The cystathionine levels in O_IDHm-codel (1.50 ± 0.63 mM) was significantly higher than in A_IDHm-noncodel (0.85 ± 0.24 mM; p = 0.001). The area under the curve for distinguishing O_IDHm-codel from A_IDHm-noncodel using the presence of cortical high-flow sign, cystathionine levels, and their combination was 0.708 (0.509-0.908), 0.750 (0.553-0.947), and 0.875 (0.737-1.000), respectively.

Conclusion: The presence of cortical high-flow sign on ASL, along with elevated cystathionine levels measured by 1H-MRS, could differentiate O_IDHm-codel from A_IDHm-noncodel.

目的:利用动脉自旋标记(ASL)皮质高流量标志和1H-MR光谱(1H-MRS)测量胱硫氨酸的组合来区分少突胶质细胞瘤,idh -突变体和1p/19q-编码缺失(o_idh -编码)与星形细胞瘤,idh -突变体(a_idh -非编码)。材料和方法:我们使用3.0 t MRI扫描仪实现伪连续ASL技术(标记后延迟= 2000 ms)。通过从对照图像中减去成对标记的图像来生成相对灌注图。1H-MRS数据采用单体素点分辨光谱序列(PRESS)序列(TR = 2000 ms, TE = 97 ms, NEX = 128,感兴趣体积= 203 mm3)获取。我们的研究包括了克拉姆塞姆-拉奥的胱氨酸水平下限为2.7 ppm,低于50%的病例。比较a_idhm -non -codel和O_IDHm-codel的皮质高血流征的存在和不存在以及胱硫氨酸的估计浓度。采用受试者工作特征曲线评价各参数的诊断性能,以及两者的结合。结果:O_IDHm-codel组皮质高血流征(7/12,58.3%)高于A_IDHm-noncodel组(3/18,16.7%,p = 0.018)。O_IDHm-codel组胱胱硫氨酸水平(1.50±0.63 mM)显著高于a_idhm -non -codel组(0.85±0.24 mM, p = 0.001)。利用皮质高流量标志、半胱甘氨酸水平及其组合区分O_IDHm-codel和a_idhm -non -codel的曲线下面积分别为0.708(0.509 ~ 0.908)、0.750(0.553 ~ 0.947)和0.875(0.737 ~ 1.000)。结论:ASL皮层高血流征的出现及1H-MRS检测的半胱硫氨酸水平升高可作为区分O_IDHm-codel和a_idhm -non -codel的重要指标。
{"title":"The diagnostic value of cortical high-flow sign combined with cystathionine on <sup>1</sup>H-MRS for prediction of 1p/19q-codeletion status in IDH-mutant adult-type diffuse glioma.","authors":"Koji Yamashita, Moyoko Tomiyasu, Kazufumi Kikuchi, Daichi Momosaka, Masaoki Kusunoki, Daisuke Kuga, Ryusuke Hatae, Yutaka Fujioka, Ryosuke Otsuji, Osamu Togao, Koji Yoshimoto, Kousei Ishigami","doi":"10.1007/s00234-026-03909-w","DOIUrl":"https://doi.org/10.1007/s00234-026-03909-w","url":null,"abstract":"<p><strong>Objectives: </strong>To leverage a combination of cortical high-flow sign on arterial spin labeling (ASL) and cystathionine measurement using <sup>1</sup>H-MR spectroscopy (<sup>1</sup>H-MRS) to distinguish oligodendroglioma, IDH-mutant and 1p/19q-codeleted (O_IDHm-codel) from astrocytoma, IDH-mutant (A_IDHm-noncodel).</p><p><strong>Materials and methods: </strong>We implemented pseudo-continuous ASL technique (post-labeling delay = 2000 ms) using a 3.0-T MRI scanner. Relative perfusion maps were generated by subtracting paired labeled images from control images. <sup>1</sup>H-MRS data were acquired by using the single-voxel point-resolved spectroscopy sequence (PRESS) sequence (TR = 2000 ms, TE = 97 ms, NEX = 128, volume of interest = 20<sup>3</sup> mm<sup>3</sup>). Our study included cases with a Cramér-Rao Lower Bound of cystathionine levels at 2.7 ppm that were below 50%. The presence or absence of cortical high-flow sign and the estimated concentration of cystathionine were compared between A_IDHm-noncodel and O_IDHm-codel. The receiver operating characteristic curves were used to evaluate the diagnostic performance of each parameter, as well as the combination of both.</p><p><strong>Results: </strong>The cortical high-flow sign was identified more frequent in O_IDHm-codel (7/12, 58.3%) than in A_IDHm-noncodel (3/18, 16.7%; p = 0.018). The cystathionine levels in O_IDHm-codel (1.50 ± 0.63 mM) was significantly higher than in A_IDHm-noncodel (0.85 ± 0.24 mM; p = 0.001). The area under the curve for distinguishing O_IDHm-codel from A_IDHm-noncodel using the presence of cortical high-flow sign, cystathionine levels, and their combination was 0.708 (0.509-0.908), 0.750 (0.553-0.947), and 0.875 (0.737-1.000), respectively.</p><p><strong>Conclusion: </strong>The presence of cortical high-flow sign on ASL, along with elevated cystathionine levels measured by 1H-MRS, could differentiate O_IDHm-codel from A_IDHm-noncodel.</p>","PeriodicalId":19422,"journal":{"name":"Neuroradiology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2026-01-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146041314","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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Neuroradiology
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