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Prognosis of patients with severe cerebral venous thrombosis treated with decompressive craniectomy 颅脑减压术治疗重度脑静脉血栓的预后分析
IF 3.9 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2023-11-01 DOI: 10.1016/j.nrl.2021.04.006
A. Arauz , M.A. Barboza , L.C. Quintero , C. Cantu , E. Chiquete , F. Serrano

Introduction

Despite the highly favorable prognosis, mortality occurs in nearly 2% of patients with cerebral venous thrombosis (CVT), in which decompressive craniectomy (DC) may be the only way to save the patient's life. The aim of this report is to describe the risk factors, neuroimaging features, in-hospital complications and functional outcome of severe CVT in patients treated with DC.

Materials and methods

Consecutive malignant CVT cases treated with DC from a retrospective third-level hospital database were analyzed. Demographic, clinical, and functional outcomes were analyzed.

Results

Twenty-six patients were included (20 female, age 35.4 ± 12.1 years); 53.8% of the patients had acute CVT, with neurological focalization as the most common symptom in 92.3% of the patients. Superior sagittal sinus thromboses were found in 84.6% of cases. Bilateral lesions were present in 10 patients (38.5%). Imaging on admission showed a parenchymal lesion (venous infarction ± hemorrhagic lesion) > 6 cm measured along the longest diameter in 25 patients (96.2%). Mean duration of clinical neurological deterioration was 3.5 days; eleven patients (42.3%) died during hospitalization.

Conclusion

In patients with severe forms of CVT, we found higher mortality than previously reported. DC is an effective life-saving treatment with acceptable functional prognosis for survivors.

尽管预后良好,但仍有近2%的脑静脉血栓形成(CVT)患者死亡率,其中减压颅骨切除术(DC)可能是挽救患者生命的唯一方法。本报告的目的是描述DC治疗的严重CVT患者的危险因素、神经影像学特征、院内并发症和功能结局。材料与方法回顾性分析三级医院数据库中连续行DC治疗的恶性CVT病例。对人口统计学、临床和功能结果进行分析。结果共纳入患者26例(女性20例,年龄35.4±12.1岁);53.8%的患者为急性CVT, 92.3%的患者以神经系统病灶为最常见的症状。84.6%的病例有上矢状窦血栓形成。双侧病变10例(38.5%)。入院时影像学显示实质病变(静脉梗死±出血性病变);25例(96.2%)患者沿最长直径测量6cm。临床神经功能恶化的平均持续时间为3.5 d;11例(42.3%)患者在住院期间死亡。结论在重症CVT患者中,我们发现死亡率高于先前报道。DC是一种有效的挽救生命的治疗方法,对幸存者具有可接受的功能预后。
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引用次数: 1
Síndrome clínico aislado: diagnóstico y riesgo de desarrollar esclerosis múltiple clínicamente definida 孤立临床综合征:临床定义的多发性硬化症的诊断和发展风险
IF 3.9 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2023-11-01 DOI: 10.1016/j.nrl.2021.01.011
J. López-Gómez , B. Sacristán-Enciso , M.A. Caro-Miró , M.R. Querol Pascual

Introduction

In most cases, multiple sclerosis (MS) initially presents as clinically isolated syndrome (CIS). Differentiating CIS from other acute or subacute neurological diseases and estimating the risk of progression to clinically definite MS is essential since presenting a second episode in a short time is associated with poorer long-term prognosis.

Development

We conducted a literature review to evaluate the usefulness of different variables in improving diagnostic accuracy and predicting progression from CIS to MS, including magnetic resonance imaging (MRI) and such biofluid markers as oligoclonal IgG and IgM bands, lipid-specific oligoclonal IgM bands in the CSF, CSF kappa free light-chain (KFLC) index, neurofilament light chain (NfL) in the CSF and serum, and chitinase 3–like protein 1 (CHI3L1) in the CSF and serum.

Conclusions

Codetection of oligoclonal IgG bands and MRI lesions reduces diagnostic delays and suggests a high risk of CIS progression to MS. A KFLC index > 10.6 and CSF NfL concentrations > 1150 ng/L indicate that CIS is more likely to progress to MS within one year (40-50%); 90% of patients with CIS and serum CHI3L1 levels > 33 ng/mL and 100% of those with lipid-specific oligoclonal IgM bands present MS within one year of CIS onset.

引言在大多数情况下,多发性硬化症(MS)最初表现为临床孤立综合征(CIS)。将CIS与其他急性或亚急性神经系统疾病区分开来,并估计进展为临床明确的MS的风险是至关重要的,因为在短时间内出现第二次发作与较差的长期预后有关。发展我们进行了一项文献综述,以评估不同变量在提高诊断准确性和预测从CIS到MS的进展方面的有用性,包括磁共振成像(MRI)和生物流体标记物,如寡克隆IgG和IgM带、CSF中的脂质特异性寡克隆IgM带,CSF和血清中的神经丝轻链(NfL)和CSF和血清的几丁质酶3样蛋白1(CHI3L1)。结论寡克隆IgG带和MRI病变的检测减少了诊断延迟,并提示CIS进展为MS的高风险。KFLC指数>;10.6和CSF NfL浓度>;1150 ng/L表明CIS在一年内更可能发展为MS(40-50%);90%的CIS患者和血清CHI3L1水平>;33ng/mL和100%具有脂质特异性寡克隆IgM条带的患者在CIS发病一年内出现MS。
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引用次数: 4
Inequidad de acceso a los nuevos tratamientos para la migraña 获得新的偏头痛治疗方法的不平等
IF 3.9 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2023-10-01 DOI: 10.1016/j.nrl.2022.08.004
P. Irimia , R. Belvís , N. García González , J. García Ull , J.M. Laínez
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引用次数: 0
Distrofia miotónica tipo 1: 13 años de experiencia en un hospital terciario. Estudio clínico y epidemiológico. Correlación genotipo-fenotipo 1型强直性肌营养不良:在三级医院的13年经验。临床和流行病学研究。基因型-表型相关性
IF 3.9 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2023-10-01 DOI: 10.1016/j.nrl.2021.02.012
J.P. Sánchez Marín , P. Sienes Bailo , R. Lahoz Alonso , J.L. Capablo Liesa , J. Gazulla Abio , J.A. Giménez Muñoz , P.J. Modrego Pardo , B. Pardiñas Barón , S. Izquierdo Álvarez

Introduction

The incidence of myotonic dystrophy type 1 (DM1), a disease with great phenotypic variety, in our region is unknown. This study aims to estimate the incidence of DM1 at our hospital (a reference centre in Aragon, Spain) and to identify the characteristics of our population (genotype-phenotype correlation).

Methods

Retrospective, descriptive study of 459 patients classified according to the number of CTG repeats, as follows: normal (5-35), premutation (36-50), protomutation (51-80), small expansions (81-150), intermediate expansions (151-1000), and large expansions (> 1000). Furthermore, according to clinical phenotype, patients were categorised as unaffected (5-50 CTG repeats), mild form or asymptomatic (51-150), classical form (151-1000), and severe form (> 1000).

Results

The incidence of DM1 was 20.61 cases per million person-years (95% CI: 19.59-21.63). An inverse correlation was observed between the number of CTG repeats and the age at genetic diagnosis (ρ = −0.547; 95% CI: −0.610 to −0.375; P < .001). CTG5 was the most frequent polymorphic allele in healthy individuals. Of all patients with DM1, 28.3% presented the mild or asymptomatic form, 59.1% the classical form, and 12.6% the severe form. Inheritance was maternal in 35.1% of cases, paternal in 59.4%, and uncertain in 5.5%. In mild forms, frontal balding in men was the most prevalent phenotypic trait, as well as myotonia and cataracts, while in the classical form, ptosis, facial weakness, voice and pronunciation alterations, myotonia, and fatigue/sleepiness were most frequent.

Conclusions

The incidence of DM1 in Aragon is significant. Multidisciplinary study of the phenotype of patients with DM1 is key to early diagnosis and personalised management.

引言强直性肌营养不良1型(DM1)是一种表型多样性很强的疾病,在我们地区的发病率尚不清楚。本研究旨在估计我们医院(西班牙阿拉贡的参考中心)DM1的发病率,并确定我们人群的特征(基因型-表型相关性),中间膨胀(151-1000)和大膨胀(>;1000)。此外,根据临床表型,患者被分为未受影响(5-50个CTG重复)、轻度或无症状(51-150)、经典型(151-1000)、,结果DM1的发生率为20.61例/百万人年(95%可信区间:19.59-21.63),CTG重复序列数与遗传诊断年龄呈负相关(ρ=-0.547;95%可信区间为-0.610~-0.375;P<;.001)。在所有DM1患者中,28.3%表现为轻度或无症状型,59.1%表现为典型型,12.6%表现为严重型。35.1%的病例为母体遗传,59.4%为父系遗传,5.5%为不确定遗传。在轻度DM1中,男性额秃是最常见的表型特征,还有肌强直和白内障,声音和发音改变、肌强直和疲劳/嗜睡最为常见。结论DM1在阿拉贡的发病率较高。DM1患者表型的多学科研究是早期诊断和个性化管理的关键。
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引用次数: 2
Test UD Interferencia: Creación y validación de un nuevo instrumento de resistencia a la interferencia. Normalización y estandarización en población española UD干扰测试:建立和验证新的抗干扰仪器。西班牙人口的标准化和标准化
IF 3.9 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2023-10-01 DOI: 10.1016/j.nrl.2021.01.014
O. Sáez-Atxukarro , R. del Pino , J. Peña , D.J. Schretlen , N. Ibarretxe-Bilbao , N. Ojeda

Introduction

The UD Interference Test measures processing speed and attention, and is based on the concept of interference of the Stroop Test. The main purpose of the study is to provide an alternative version of the Stroop Test that overcomes some of the limitations of previous versions in assessing individuals with daltonism or age-related reading difficulties, and to obtain normative and standardised data for the Spanish population.

Methods

This study is part of the Normacog project. We evaluated 905 individuals (age range, 18-93 years) to analyse the test's reliability and concurrent and construct validity. We evaluated the effect of age, sex, and level of education on UD Interference Test performance and calculated percentiles and age- and education-adjusted scaled scores.

Results

The test has good reliability (α = 0.875) and concurrent (r = 0.443-0.725; p < .001) and construct validity (r = 0.472-0.737; p < .001). We observed age and educational level to have a significant effect on UD Interference Test scores, explaining 12-40% of variance. Sex only had a significant effect on the resistance to interference index.

Conclusions

We present an alternative version of the Stroop Color and Word Test with some advantages over previous versions. We provide standardised and normalised data for the Spanish population to correct the test according to the subject's age and level of education.

UD干扰测试测量处理速度和注意力,并基于Stroop测试的干扰概念。该研究的主要目的是提供一种替代版本的Stroop测试,该测试克服了以前版本在评估患有多动症或与年龄相关的阅读困难的个体方面的一些局限性,并获得西班牙人群的规范和标准化数据。方法本研究是Normacog项目的一部分。我们评估了905名个体(年龄范围18-93岁),以分析测试的可靠性、并发性和结构有效性。我们评估了年龄、性别和教育水平对UD干扰测试成绩的影响,并计算了百分位数以及年龄和教育程度调整后的量表得分。结果该测试具有良好的信度(α=0.875)、并发性(r=0.443-0.725;p<;.001)和结构有效性(r=0.772-0.737;p&llt;.001)。我们观察到年龄和教育水平对UD干扰测试得分有显著影响,解释了12-40%的方差。性别只对抗干扰指数有显著影响。结论我们提出了一种替代版本的Stroop颜色和单词测试,与以前的版本相比有一些优势。我们为西班牙人口提供标准化和规范化的数据,以根据受试者的年龄和教育水平更正测试。
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引用次数: 1
Efecto de la toxina botulínica tipo A en la funcionalidad, las sincinesias y la calidad de vida en secuelas de parálisis facial periférica A型肉毒毒素对周围性面瘫后遗症患者功能、运动和生活质量的影响
IF 3.9 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2023-10-01 DOI: 10.1016/j.nrl.2021.01.015
U. Díaz-Aristizabal, M. Valdés-Vilches, T.R. Fernández-Ferreras, E. Calero-Muñoz, E. Bienzobas-Allué, L. Aguilera-Ballester, J. Carnicer-Cáceres

Objectives

This study aimed to assess the effects of botulinum toxin A (BTX-A) infiltration on face muscle function, synkinesis, and quality of life in patients with sequelae of peripheral facial palsy (PFP).

Material and methods

We present the results of a prospective study including a sample of 20 patients with sequelae of PFP (15 women, 5 men) who underwent BTX-A (Botox® or Xeomin®) infiltration. All patients had previously received personalised treatment with neuromuscular retraining. A clinical assessment was performed before BTX-A infiltration and 4 weeks after treatment. The effect of BTX-A on face muscle function, quality of life, and synkinesis was evaluated using the Sunnybrook Facial Grading System (SFGS), the Facial Clinimetric Evaluation (FaCE) questionnaire, and the Synkinesis Assessment Questionnaire (SAQ), respectively.

Results

Mean SFGS scores increased from 64.8 to 69.9 after BTX-A infiltration (P = .004). Increases were also observed in mean total FaCE scores (from 52.42 to 64.5; P < .001) and the mean score on the FaCE social function subscale (from 61.15 to 78.44; P < .001). Mean SAQ scores decreased from 46.22 to 37.55 after BTX-A infiltration (P = .001).

Conclusions

BTX-A infiltration increases face muscle function, improves quality of life, and reduces synkinesis in patients with sequelae of PFP.

目的本研究旨在评价肉毒杆菌毒素A(BTX-A)浸润对面部肌肉功能、联合运动功能和运动功能的影响,材料和方法我们介绍了一项前瞻性研究的结果,该研究包括20名接受BTX-a(肉毒杆菌毒素®或Xeomin®)浸润的PFP后遗症患者(15名女性,5名男性)。所有患者都曾接受过神经肌肉再训练的个性化治疗。在BTX-A浸润前和治疗后4周进行临床评估。分别使用Sunnybrook面部分级系统(SFGS)、面部临床评估问卷(face)和运动机能综合评估问卷(SAQ)评估BTX-A对面部肌肉功能、生活质量和联合运动的影响。结果BTX-A浸润后,平均SFGS评分从64.8分增加到69.9分(P=.004)。平均总FaCE评分(从52.42分增加到64.5分;P<;.001)和FaCE社会功能分量表的平均评分(从61.15分增加到78.44分;P>;.001)也有所增加。平均SAQ评分从46.22分减少到37.55分(P=.001)面部肌肉功能,改善生活质量,并减少PFP后遗症患者的联合运动。
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引用次数: 1
Direct carotid puncture for the endovascular treatment of acute ischaemic stroke associated to aortic arch atheroma 直接颈动脉穿刺血管内治疗主动脉弓动脉粥样硬化相关的急性缺血性卒中
IF 3.9 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2023-10-01 DOI: 10.1016/j.nrl.2022.12.002
I. Saldaña-Inda , E. Garcés-Antón , J.M. Navasa-Melado , C. Bernal-Lafuente , H. Tejada-Meza
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引用次数: 0
Inflamación relacionada con angiopatía amiloide: características clínicas y respuesta al tratamiento en una serie de casos 淀粉样血管病相关炎症:一系列病例的临床特征和治疗反应
IF 3.9 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2023-10-01 DOI: 10.1016/j.nrl.2020.12.008
P. Martín-Jiménez , M. Sánchez-Tornero , S. Llamas-Velasco , M.P. Guerrero-Molina , M. González-Sánchez , A. Herrero-San Martín , V. Blanco-Palmero , P. Calleja-Castaño , J. Francisco-Gonzalo , A. Hilario , A. Ramos , E. Salvador , Ó. Toldos , A. Hernández-Lain , D.A. Pérez-Martínez , A. Villarejo-Galende

Introduction

Cerebral amyloid angiopathy–related inflammation (CAA-ri) is an entity characterised by an inflammatory response to β-amyloid deposition in the walls of cerebral microvessels.

Methods

We conducted a retrospective review of a series of patients with a diagnosis of CAA-ri according to histopathological study findings or clinical-radiological diagnostic criteria.

Results

The study included 7 patients (5 men) with a mean age of 79 years. Disease onset was acute or subacute in 6 patients. The most frequent symptoms were cognitive impairment (n = 6), behavioural alterations (n = 5), epileptic seizures (n = 5), focal neurological signs (n = 4), and headache (n = 2). Cerebrospinal fluid was abnormal in 3 patients (lymphocytic pleocytosis and high protein levels). The most frequent MRI findings were microbleeds (n = 7), subcortical white matter hyperintensities on T2-FLAIR sequences (n = 7), and leptomeningeal enhancement (n = 6). Lesions were bilateral in 3 patients and most frequently involved the parieto-occipital region (n = 5). Amyloid PET studies were performed in 2 patients, one of whom showed pathological findings. Two patients underwent brain biopsy, which confirmed diagnosis. All patients received immunosuppressive therapy. An initially favourable clinical-radiological response was observed in all cases, with 2 patients presenting radiological recurrence after treatment withdrawal, with a subsequent improvement after treatment was resumed.

Conclusions

Early diagnosis of CAA-ri is essential: early treatment has been shown to improve prognosis and reduce the risk of recurrence. Although a histopathological study is needed to confirm diagnosis, clinical-radiological criteria enable diagnosis without biopsy.

引言脑淀粉样血管病相关炎症(CAA-ri)是一种以对脑微血管壁中β-淀粉样蛋白沉积的炎症反应为特征的实体。方法根据组织病理学研究结果或临床放射学诊断标准,对一系列诊断为CAA ri的患者进行回顾性分析。结果本研究包括7名患者(5名男性),平均年龄79岁。6例患者发病为急性或亚急性。最常见的症状是认知障碍(n=6)、行为改变(n=5)、癫痫发作(n=5个)、局灶性神经系统体征(n=4个)和头痛(n=2个)。3例患者脑脊液异常(淋巴细胞增多症和高蛋白水平)。最常见的MRI表现为微出血(n=7)、T2-FLAIR序列上的皮质下白质高信号(n=7个)和软脑膜增强(n=6个)。3例患者为双侧病变,最常累及顶枕区(n=5)。对2名患者进行了淀粉样PET研究,其中一名患者表现出病理学表现。两名患者接受了脑活检,证实了诊断。所有患者均接受免疫抑制治疗。在所有病例中观察到最初良好的临床放射学反应,2名患者在停药后出现放射学复发,随后在恢复治疗后有所改善。结论CAA-ri的早期诊断至关重要:早期治疗已被证明可以改善预后并降低复发风险。尽管需要进行组织病理学研究来确认诊断,但临床放射学标准可以在没有活检的情况下进行诊断。
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引用次数: 1
Safety and efficacy of the Low-Profile Visualized Intraluminal Support stent in treating intracranial atherosclerotic stenosis 低剖面可视化管腔内支架治疗颅内动脉粥样硬化性狭窄的安全性和有效性
IF 3.9 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2023-10-01 DOI: 10.1016/j.nrl.2021.02.013
J.-W. Wang, X.-Y. Li, C.-H. Li, J.-F. Liu, H. Li, Y.-Y. Tian, B.-L. Gao

Purpose

The performance of the Low-Profile Visualized Intraluminal Support (LVIS) stent deployed following balloon angioplasty is unknown in treating intracranial atherosclerotic stenosis, and this study was to investigate the safety and efficacy of the LVIS stent in treating intracranial atherosclerotic stenosis in the middle cerebral artery M1 segment.

Methods

Thirty-five patients were enrolled with 35 atherosclerotic stenoses at the M1 segment. The stenosis was about 75% in 16 patients, 80% in 15, and 90% in the rest four. The LVIS stent was used to treat these patients.

Results

The success rate of stenting was 97.1%. The stenting procedure was failed in one patient because of intraprocedural dissection of the stenotic (75%) segment, resulting in a 30-day periprocedural complication rate of 2.9% (1/35). Before stenting, the stenosis rate ranged 75%–90% (mean 78.9% ± 4.7%), and after stenting, the diameter of the stented segment was significantly (P < 0.0001) increased to 1.5–3.4 mm (mean 2.1 ± 0.32 mm) ranging 68.2%–100% (mean 94.0% ± 5.8%) of the normal arterial diameter, with the residual stenosis ranging 0–31.8% (median 4.8%, IQR 2.4%–7.3%). Follow-up was performed at 6–20 months (mean 8.5) after stenting. One patient (2.9%) had occlusion of the stented M1 segment with no symptoms, and two patients (5.7%) had slight asymptomatic instent stenosis (40%) at the M1 segment, with the instent restenosis and occlusion rate of 8.6% (3/35).

Conclusion

The braided LVIS stent can be safely applied for treatment of intracranial atherosclerotic stenosis in the middle cerebral artery with good safety and efficacy immediately after stenting and at follow-up.

目的低剖面可视化管腔内支架(LVIS)治疗颅内动脉粥样硬化性狭窄的疗效尚不清楚,本研究旨在探讨LVIS支架治疗大脑中动脉M1段颅内动脉粥样硬化性病变的安全性和有效性。方法35例M1段动脉粥样硬化性狭窄患者。16例患者的狭窄率约为75%,15例为80%,其余4例为90%。LVIS支架用于治疗这些患者。结果支架置入成功率为97.1%,其中1例因术中剥离狭窄段(75%)而失败,30天围手术期并发症发生率为2.9%(1/35)。支架置入前,狭窄率为75%-90%(平均78.9%±4.7%),支架置入后,支架段的直径显著(P<;0.0001)增加到1.5–3.4 mm(平均2.1±0.32 mm),范围为正常动脉直径的68.2%-100%(平均94.0%±5.8%),残余狭窄范围为0–31.8%(中位数4.8%,IQR 2.4%–7.3%)。支架植入后6–20个月(平均8.5个月)进行随访。1名患者(2.9%)支架M1段闭塞,无症状,2名患者(5.7%)M1段轻度无症状狭窄(40%),支架再狭窄和闭塞率为8.6%(3/35)。结论编织型LVIS支架可安全应用于治疗大脑中动脉颅内动脉粥样硬化性狭窄,支架置入后即刻及随访安全有效。
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引用次数: 1
Réplica: Perfil clínico, tratamiento antibiótico y riesgo de ictus en pacientes con endocarditis infecciosa 感染性心内膜炎患者的临床概况、抗生素治疗和中风风险
IF 3.9 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2023-10-01 DOI: 10.1016/j.nrl.2022.09.008
J. Rodríguez-Montolio , D. Meseguer-González , M. Almeida-Zurita , S. Santos-Lasaosa
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引用次数: 0
期刊
Neurologia
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