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Diagnosis and treatment of disorders of intracranial pressure: consensus statement of the Spanish Society of Neurology's Headache Study Group. 颅内压紊乱的诊断与治疗:西班牙神经病学学会头痛研究小组共识声明。
Pub Date : 2025-01-01 Epub Date: 2024-02-29 DOI: 10.1016/j.nrleng.2024.02.009
J García-Ull, N González-García, M Torres-Ferrús, D García-Azorín, I F J Molina-Martínez, I Beltrán-Blasco, S Santos-Lasaosa, G Latorre, A B Gago-Veiga, J M Láinez, J Porta-Etessam, C Nieves-Castellanos, A Mínguez-Olaondo, A López-Bravo, S Quintas, N Morollón, S Díaz-Insa, R Belvís, P Irimia

Primary intracranial pressure disorders include idiopathic intracranial hypertension and spontaneous intracranial hypotension. Remarkable advances have been made in the diagnosis and treatment of these 2entities in recent years. Therefore, the Spanish Society of Neurology's Headache Study Group (GECSEN) deemed it necessary to prepare this consensus statement, including diagnostic and therapeutic algorithms to facilitate and improve the management of these disorders in clinical practice. This document was created by a committee of experts belonging to GECSEN, and is based on a systematic review of the literature, incorporating the experience of the participants, and establishes practical recommendations with levels of evidence and grades of recommendation.

原发性颅内压疾病包括特发性颅内高压和自发性颅内低血压。近年来,这两种疾病在诊断和治疗技术方面取得了显著进步。因此,西班牙神经病学学会头痛研究小组(GECSEN)认为有必要编写这份共识文件,其中包括诊断和治疗算法,以促进和改善临床实践中的管理。本文件由 GECSEN 专家委员会在系统回顾文献的基础上编写而成,其中纳入了参与者的经验,并制定了具有证据级别和推荐等级的实用建议。
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引用次数: 0
Guideline: Neurorehabilitation in patients with disorder of consciousness. Recommendations from the Spanish Society of Neurorehabilitation. 指南:意识障碍患者的神经康复。来自西班牙神经康复学会的建议。
Pub Date : 2025-01-01 Epub Date: 2025-01-10 DOI: 10.1016/j.nrleng.2025.01.002
E Noé, M D Navarro, B Moliner, M O'Valle, J Olaya, A Maza, R Llorens, J Ferri, R Rodríguez, T Pérez, M Bernabéu, C Colomer, A Gómez, C González, A Juárez-Belaúnde, C López, S Laxe, R Pelayo, M Ríos, I Quemada

Introduction: Clinical practice guidelines in neurorehabilitation for adults with disorders of consciousness by the Spanish Neurorehabilitation Society. This document is based on a review of international clinical practice guidelines published between 2015 and 2022.

Method: A total of 7 articles, corresponding to 5 clinical practice guidelines published between 2015 and 2022, were selected by the group of authors from a pool of 48 bibliographic references extracted from various databases in accordance with predefined search criteria. Following this review, forty recommendations were formulated and subjected to evaluation by an expert committee using a 9-point Likert scale: 1-3 (inappropriate recommendation), 4-6 (uncertain recommendation), and 7-9 (appropriate recommendation), following the methodology of the "Modified Nominal Group Technique." Any recommendation endorsed by at least 75% of the experts as "appropriate" (with a score of 7-9) was considered accepted.

Conclusions: This document presents 40 recommendations categorised according to the level of evidence provided by the reviewed studies. These recommendations represent a consensus among experts and pertain to various aspects related to: 1) clinical assessment, 2) complementary diagnostic tests, 3) prognosis, and 4) treatment in this specific population.

简介:西班牙神经康复学会的成人意识障碍神经康复临床实践指南。本文件基于对2015年至2022年期间发布的国际临床实践指南的审查。方法:作者组按照预先设定的检索标准,从各种数据库中提取48篇参考文献,筛选出2015 - 2022年间发表的5篇临床实践指南对应的7篇文章。在此综述之后,制定了40项建议,并由专家委员会使用9点李克特量表进行评估:1-3(不适当的建议),4-6(不确定的建议)和7-9(适当的建议),遵循“修改名义小组技术”的方法。任何被至少75%的专家认可为“合适”的建议(得分在7-9分)都被认为是被接受的。结论:本文件提出了40项建议,根据所审查的研究提供的证据水平进行分类。这些建议代表了专家之间的共识,涉及以下各个方面:1)临床评估,2)补充诊断测试,3)预后,以及4)该特定人群的治疗。
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引用次数: 0
Thrombus composition and its implication in ischemic stroke assessment and revascularization treatments. 血栓组成及其在缺血性卒中评估和血运重建治疗中的意义。
Pub Date : 2025-01-01 Epub Date: 2024-12-21 DOI: 10.1016/j.nrleng.2024.12.001
Juan Marta-Enguita, Florencio J D Machado, Josune Orbe, Roberto Muñoz

Introduction: Since mechanical thrombectomy has allowed ischaemic stroke thrombus retrieval, the exhaustive study of this material has enabled better understanding of the potential physiopathological processes involved in thrombus formation.

Development: Thrombotic pathways involved in the different vascular beds share common mechanisms, causing difficulties in the identification of specific patterns associated with stroke aetiology. However, other factors such as clot formation time, associated inflammatory status, or activation of additional immune and coagulation pathways (neutrophil extracellular trap [NET] delivery, platelet aggregation, endothelial activation, and von Willebrand Factor release) have been described as determinants in thrombus characteristics. Thus, variable proportions of fibrin-/platelet-rich and erythrocyte-rich areas are closely interrelated within the thrombus, frequently associated with a protective outer shell with high concentrations of fibrin, NETs, and von Willebrand Factor. The presence of these components, as well as their distribution and interrelationships, have been shown to have effects on the thrombus' resistance to revascularisation treatments. Understanding of these pathways has enabled the development of adjuvant therapies capable of enhancing current fibrinolytic drugs and/or increasing the efficacy of endovascular treatments.

Conclusion: Understanding of thrombus components and mechanisms involved in thrombus formation represent a potential pathway for the development of ischaemic stroke therapeutics with promising perspectives.

导读:由于机械取栓已经允许缺血性卒中血栓回收,其详尽的研究已经涉及到在其形成隐含的生理病理过程的更好的知识。发展:涉及不同血管床的血栓形成途径具有共同的机制,在确定与卒中病因学相关的特定模式方面存在调节困难。其他因素如血栓形成时间、相关炎症状态或额外免疫和凝血途径的激活[中性粒细胞细胞外陷阱(NETs)递送、血小板聚集、内皮活化和VonWillebrand因子释放]被认为是血栓特征的决定因素。因此,不同比例的富含纤维蛋白/血小板的区域和富含红细胞的区域在血栓中共存,并在它们内部有着密切的关系,经常伴随着含有高浓度纤维蛋白、NETs和VonWillebrand因子的保护性外壳。这些成分的存在,以及它们的分布和相互关系已被证明对血栓对血运重建治疗的抵抗有影响。对这一途径的充分了解使得开发辅助疗法能够增强当前的纤溶药物和/或提高血管内治疗的疗效。结论:对血栓组成及其形成机制的认识为缺血性脑卒中的治疗开辟了一条新的途径,具有广阔的前景。
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引用次数: 0
Clinical variability in STIM1 variant c.262A>G associated with Stormorken syndrome.
Pub Date : 2025-01-01 DOI: 10.1016/j.nrleng.2025.01.001
L Silva-Hernández, B Cabal-Paz, J Jiménez-Almonacid, E González-Vioque, J Bohm
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引用次数: 0
Thrombosis with thrombocytopenia syndrome following adenovirus vector-based vaccines to prevent COVID-19: Epidemiology and clinical presentation in Spain. 接种基于腺病毒载体的疫苗以预防 COVID-19 后出现血栓形成伴血小板减少综合征:西班牙的流行病学和临床表现。
Pub Date : 2024-11-01 Epub Date: 2024-10-31 DOI: 10.1016/j.nrleng.2024.10.001
D García-Azorín, E Lázaro, D Ezpeleta, R Lecumberri, R de la Cámara, M Castellanos, C Iñiguez Martínez, L Quiroga-González, G Elizondo Rivas, A Sancho-López, P Rayón Iglesias, E Segovia, C Mejías, D Montero Corominas

Background: We describe the epidemiological and clinical characteristics of thrombosis with thrombocytopenia syndrome (TTS) cases reported in Spain.

Methods: We included all cases of venous or arterial thrombosis with thrombocytopenia following administration of adenoviral vector vaccines (AstraZeneca or Janssen) against COVID-19 disease between 1 February and 26 September 2021. We describe the crude rate and the standardised morbidity ratio. We assessed the predictors of mortality.

Results: Sixty-one cases were reported and 45 fulfilled eligibility criteria; 82% of patients were women. The crude TTS rate was 4 cases/1 000 000 doses, and 14-15 cases/1 000 000 doses among patients aged 30-49 years. The number of observed cases of cerebral venous thrombosis was 6-18 times higher than that expected in patients younger than 49 years. Symptoms started a median (quartiles 1 and 3 [Q1-Q3]) of 10 (7-14) days after vaccination. Eighty percent (95% confidence interval [CI]: 65%-90%) had thrombocytopenia at the time of the emergency department visit, and 65% (49%-78%) had D-dimer levels > 2000 ng/mL. Patients had thromboses affecting multiple locations in 36% of cases and fatal outcomea in 24%. Platelet nadir < 50 000/μL (odds ratio [OR]: 7.4; 95% CI: 1.2-47.5) and intracranial hemorrhage (OR: 7.9; 95% CI: 1.3-47.0) were associated with fatal outcomes.

Conclusion: TTS must be suspected in patients with symptoms 10 days after vaccination and thrombocytopenia and/or elevated D-dimer levels.

背景:我们描述了西班牙报告的血栓伴血小板减少综合征(TTS)病例的流行病学和临床特征:我们描述了西班牙报告的血栓伴血小板减少综合征(TTS)病例的流行病学和临床特征:我们纳入了 2021 年 2 月 1 日至 9 月 26 日期间接种腺病毒载体疫苗(阿斯利康或杨森)以预防 COVID-19 疾病的所有伴血小板减少的静脉或动脉血栓形成病例。我们描述了粗死亡率和标准化发病率。我们评估了死亡率的预测因素:共报告了 61 例病例,其中 45 例符合资格标准,82% 为女性。粗TTS率为4/1,000,000剂量,30-49岁为14-15/1,000,000剂量。在 49 岁以下的患者中,观察到的脑静脉血栓病例数比预期高出 6-18 例。症状开始于接种疫苗后 10 天(四分位距(IQR):7-14 天)。80%(95% 置信区间 (CI):65-90%)的患者在急诊就诊时血小板减少,65%(95% 置信区间 (CI):49-78%)的患者 D-二聚体大于 2000 纳克/毫升。36%的患者有多处血栓形成,24%的患者有致命后果。血小板最低点接种疫苗 10 天后出现症状、血小板减少和/或 D-二聚体增加的患者必须怀疑 TTS。
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引用次数: 0
Sporadic fatal insomnia: a rapidly progressive phenotype resembling progressive supranuclear palsy. 零星致命性失眠:快速进展的 PSP 样表型。
Pub Date : 2024-11-01 Epub Date: 2024-02-20 DOI: 10.1016/j.nrleng.2024.02.005
J C Romero-Fábrega, R Lorenzo-López, E Rivas-Infante, F Escamilla-Sevilla, M Rashki, A Mínguez-Castellanos, A Carvajal-Hernández
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引用次数: 0
Encephalitis after COVID-19 vaccination 接种 COVID-19 疫苗后出现脑炎。
Pub Date : 2024-10-01 DOI: 10.1016/j.nrleng.2024.02.002
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引用次数: 0
Unilateral MRIgFUS thalamotomy: Long-term follow-up in fragile X-associated tremor/ataxia syndrome 单侧 MRIgFUS 丘脑切开术:脆性X相关震颤/共济失调综合征的长期随访
Pub Date : 2024-10-01 DOI: 10.1016/j.nrleng.2024.09.004
M. Campins-Romeu , R. Conde-Sardón , J.L. León-Guijarro , I. Sastre-Bataller
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引用次数: 0
Ischemic encephalopathic debut of CADASIL, a case report: It is better to be safe than sorry CADASIL缺血性脑病首发病例报告:安全胜于遗憾
Pub Date : 2024-10-01 DOI: 10.1016/j.nrleng.2024.09.005
C. González-Mingot , A. Gil-Sánchez , R. Begué-Gómez , R. López-Ortega , B.R. Luis
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引用次数: 0
Cerebral venous thrombosis in children an 18-year review of a Portuguese hospital 葡萄牙一家医院 18 年来的儿童脑静脉血栓病回顾
Pub Date : 2024-10-01 DOI: 10.1016/j.nrleng.2024.09.002
J. Tenente , S. Lopes , P. Bem , M. Vila-Real , D. Ferreira , A.F. Geraldo , F. Santos

Introduction

Cerebral venous thrombosis (CVT) is an uncommon and clinically heterogeneous cerebrovascular particularly in children, only a few published case series focused in the pediatric population.

Patients and methods

Retrospective single-center observational and analytical study of consecutive pediatric patients admitted in a level II Portuguese hospital with a confirmed diagnosis of CVT, from 2003 to 2021. Clinical presentation, neuroimaging findings, prothrombotic factors, treatment strategies, outcome and recanalization were documented.

Results

Twelve children were included (58% female). Mean age was 7.3 years. The most frequent symptoms were vomiting, headache and behavioral alterations. Infection was the triggering factor in 50% of the cases. The diagnosis of CVT was made based on imaging evidence of thrombosis through magnetic imaging resonance (MRI) with venography and/or computed tomography (CT) with venography. In 67% of cases there were multiples sinuses involved; the transverse sinus was the most affected, followed by the sigmoid sinus. In 83% of cases anticoagulant therapy was initiated with low molecular weight heparin (LMWH) and associated prothrombotic factors were investigated, with no major prothrombotic factors identified. No deaths occurred, but 30% had long-term neurological sequelae. One patient recurred 18 years later.

Conclusion

The results of this study are consistent with data from other published studies. MRI is the preferred imaging method for diagnosis in children by avoiding ionizing radiation and allowing identification of subjacent causes. Anticoagulation with LMWH is recommended and important to reduce mortality and sequelae. Infectious diseases are the most common trigger for CVT and can also be the cause for high morbidity and poor outcomes.
导言脑静脉血栓(CVT)是一种不常见的临床异质性脑血管病,尤其是在儿童中,只有少数发表的系列病例集中在儿科人群中。患者和方法对葡萄牙一家二级医院 2003 年至 2021 年连续收治的确诊为脑静脉血栓的儿科患者进行回顾性单中心观察和分析研究。研究记录了临床表现、神经影像学检查结果、血栓形成诱因、治疗策略、疗效和再通情况。平均年龄为 7.3 岁。最常见的症状是呕吐、头痛和行为改变。在50%的病例中,感染是诱发因素。CVT的诊断依据是通过磁共振成像(MRI)和静脉造影术和/或计算机断层扫描(CT)和静脉造影术获得的血栓形成影像学证据。67%的病例涉及多个静脉窦;横窦受影响最大,其次是乙状窦。83%的病例开始使用低分子量肝素(LMWH)进行抗凝治疗,并对相关的促血栓形成因素进行了调查,但未发现主要的促血栓形成因素。无死亡病例,但30%的患者有长期神经系统后遗症。结论本研究结果与其他已发表的研究数据一致。核磁共振成像可避免电离辐射,并能识别亚邻近病因,是诊断儿童疾病的首选成像方法。建议使用 LMWH 抗凝,这对降低死亡率和减少后遗症非常重要。感染性疾病是 CVT 最常见的诱因,也是导致高发病率和不良后果的原因。
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引用次数: 0
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Neurologia
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