Revue neurologique最新文献_第2页

Mothership versus Drip-and-Ship for stroke in a rural area: A French prospective observational study 在农村地区治疗中风的母婴护理与滴注护理：一项法国前瞻性观察研究。

IF 2.8 4区医学 Q2 CLINICAL NEUROLOGY

Revue neurologique

Pub Date : 2025-01-01 DOI: 10.1016/j.neurol.2024.06.007

M. Raquin, C. Lambert, P. Paris, N. Bourgois, P. Clavelou, X. Moisset , A. Ferrier

Background

The availability of mechanical thrombectomy (MT) is limited. Thus, there are two paradigms for patients living closer to a primary stroke center (PSC) than a comprehensive stroke center (CSC) capable of MT: “Mothership” (direct referral to a CSC) and “Drip-and-Ship” (referral to a PSC for imaging and thrombolysis and transfer to a CSC for thrombectomy or monitoring). We aimed to compare the prognosis of patients at three months between the two paradigms in a rural area.

Materials

From September 2019 to March 2021, we prospectively included patients living closer to a PSC than the one CSC, regardless of the type of stroke or reperfusion treatment. The proportion of patients with a good functional outcome (Rankin ≤ 2) at three months was compared between the two initial orientations for all patients and for subgroups: patients with ischemic stroke and patients treated by MT.

Results

Among the 206 patients included, 103 were admitted directly to the CSC (82.5% had an ischemic stroke and 24.3% a MT) and 103 initially admitted to a PSC and then transferred to the CSC (100% had an ischemic stroke and 52.4% a MT). The proportion of patients with a good outcome was comparable between the two groups (54.5% vs. 43.7%, P = 0.22). Among the 79 patients who underwent MT, the prognosis at three months was better in the Mothership group (49.3% vs. 15.3%, P = 0.01).

Conclusion

The functional prognosis is comparable between Mothership and Drip-and-Ship paradigms in our setting, despite a trend towards a better prognosis for the Mothership. As has been shown in urban settings, the mothership paradigm also leads to a better prognosis for patients treated with MT in a rural setting.

背景：机械取栓术（MT）的可用性有限。因此，对于居住地距离初级卒中中心（PSC）比综合卒中中心（CSC）更近且有能力进行机械取栓术的患者，有两种治疗模式："母船"（直接转诊至综合卒中中心）和 "滴灌-转运"（转诊至初级卒中中心进行影像学检查和溶栓，然后转运至综合卒中中心进行血栓切除术或监测）。我们的目的是在农村地区比较两种模式下患者三个月后的预后：从 2019 年 9 月到 2021 年 3 月，我们前瞻性地纳入了居住在离 PSC 比 CSC 更近的患者，无论中风或再灌注治疗的类型如何。结果：在纳入的206名患者中，有103人的功能预后良好（Rankin≤2）：在纳入的 206 例患者中，103 例直接入住 CSC（82.5% 为缺血性卒中，24.3% 为 MT），103 例最初入住 PSC 后转入 CSC（100% 为缺血性卒中，52.4% 为 MT）。两组患者预后良好的比例相当（54.5% 对 43.7%，P=0.22）。在接受MT的79名患者中，母船组患者三个月后的预后更好（49.3%对15.3%，P=0.01）：结论：在我们的环境中，母船式和点滴加船式的功能性预后相当，尽管母船式的预后有更好的趋势。正如在城市环境中所显示的那样，在农村环境中，母船模式也能为接受 MT 治疗的患者带来更好的预后。

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引用次数: 0

Epileptic seizures in patients with primary central nervous system lymphoma: A systematic review 原发性中枢神经系统淋巴瘤患者的癫痫发作:一项系统综述。

IF 2.8 4区医学 Q2 CLINICAL NEUROLOGY

Revue neurologique

Pub Date : 2025-01-01 DOI: 10.1016/j.neurol.2023.08.021

O. Aboubakr , C. Houillier , S. Choquet , S. Dupont , K. Hoang-Xuan , B. Mathon

Background

Primary central nervous system lymphoma (PCNSL) accounts for less than 5% of primary brain tumors. Epileptic seizures are a common manifestation of brain tumors; however, literature on the prevalence, characteristics, and oncological implications of seizures in patients with PCNSL is limited, and the management of antiepileptic drugs (AEDs) is unclear. This review aimed to summarize the existing knowledge on seizures in PCNSL, their potential association with surgery, oncological treatment, survival rates, and management of AEDs.

Methods

A systematic review was performed according to the PRISMA recommendations and included articles published between 1953 and 2023 describing seizures in patients with PCNSL.

Results

The search identified 282 studies, of which 21 were included. Up to 33% of patients with PCNSL developed seizures, mostly at the initial presentation. Little information was found on changes in seizure incidence through the course of the disease, and no details were found on seizure frequency, the percentage of treatment-resistant patients, or the evolution of seizures at remission. Younger age, cortical location, and immunodeficiency have been identified as potential risk factors for seizures, but evidence is very limited. The growing use of vigorous treatments including intensive chemotherapy with autologous stem cell transplantation and immunotherapy with CAR-T cells is associated with a higher incidence of seizures. The association between seizure development and patient mortality in PCNSL remains unknown. There are no data on AED prophylaxis or the use of specific AEDs in PCNSL.

Conclusions

Further studies are needed to investigate seizures in larger cohorts of PCNSL, to clarify their prevalence, better characterize them, identify risk factors, analyze survival rates, and make recommendations on AED management. We recommend following general practice guidelines for seizures symptomatic of brain tumors and not to prescribe AED prophylaxis in PCNSL.

背景:原发性中枢神经系统淋巴瘤(PCNSL)占原发性脑肿瘤的不到5%。癫痫发作是脑肿瘤的常见表现;然而，关于PCNSL患者癫痫发作的患病率、特征和肿瘤学意义的文献有限，抗癫痫药物(aed)的管理也不清楚。本综述旨在总结关于PCNSL癫痫发作的现有知识，以及它们与手术、肿瘤治疗、存活率和AEDs管理的潜在关联。方法:根据PRISMA推荐进行系统综述，纳入1953年至2023年间发表的描述PCNSL患者癫痫发作的文章。结果:检索确定了282项研究，其中21项被纳入。高达33%的PCNSL患者出现癫痫发作，大多数在最初出现时。在整个病程中，关于癫痫发作发生率变化的信息很少，关于癫痫发作频率、耐药患者的百分比或缓解期癫痫发作演变的细节也没有发现。年龄较小、皮质位置和免疫缺陷已被确定为癫痫发作的潜在危险因素，但证据非常有限。包括自体干细胞移植强化化疗和CAR-T细胞免疫治疗在内的有力治疗的使用越来越多，这与癫痫发作的高发生率有关。PCNSL中癫痫发作发展与患者死亡率之间的关系尚不清楚。在PCNSL中没有关于AED预防或特定AED使用的数据。结论:需要进一步的研究来调查更大队列的PCNSL癫痫发作，以明确其患病率，更好地描述其特征，识别危险因素，分析生存率，并提出AED管理建议。我们建议遵循脑肿瘤发作症状的一般实践指南，而不是在PCNSL中开AED预防处方。

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引用次数: 0

Safinamide for pain management in patients with Parkinson's disease 治疗帕金森病患者疼痛的沙芬胺

IF 2.8 4区医学 Q2 CLINICAL NEUROLOGY

Revue neurologique

Pub Date : 2025-01-01 DOI: 10.1016/j.neurol.2023.10.013

D. Abilashimova , M. Aubakirova , Y. Abdildin , D. Viderman

Introduction

Pain is often neglected in Parkinson's disease (PD), although it impacts most PD patients. While the mechanism of pain in PD is still being studied, various pharmacological, interventional, and alternative treatment options have been offered for pain relief. Safinamide, a recently approved drug for PD, has shown promising results in improving pain in patients with PD. Several clinical studies report changes in pain scores in PD patients treated with safinamide, but these have not been systematically summarized. Therefore, our main goal was to perform a systematic review and statistical analysis of relevant studies.

Methods

A systematic search of studies was conducted using four databases: Pubmed, Cochrane Library, Google Scholar, and Scopus. The nine included randomized controlled trials did not provide sufficient data for a meta-analysis; therefore, we conducted a qualitative systematic review.

Results

Our results suggest that safinamide at a daily dose of 100 mg is more effective for treating PD pain than that of 50 mg. Moreover, the reduction in fluctuation-related pain and pain from edema was more consistent when treated with safinamide compared to other PD pain types. We also attempted to suggest a mechanism of action for safinamide on pain processing in the brain, which should be explored in more detail in future studies.

Conclusion

Clinical evidence suggests that safinamide may be particularly beneficial for PD patients experiencing fluctuation-related pain and pain from edema, as these subtypes of pain showed greater improvement compared to other types of pain. Based on the findings of the included studies, safinamide appears to relieve the overall pain burden. However, the lack of sufficient data for conducting a meta-analysis highlights the need for future studies to report mean pain scores and their standard deviations.

导言帕金森病（PD）中的疼痛常常被忽视，尽管它影响着大多数帕金森病患者。虽然帕金森病的疼痛机制仍在研究之中，但已有多种药物、介入和替代治疗方案可用于缓解疼痛。最近获批的一种治疗帕金森氏症的药物--萨菲那胺在改善帕金森氏症患者的疼痛方面取得了可喜的成果。有几项临床研究报告了使用沙芬胺治疗的帕金森病患者疼痛评分的变化，但尚未对这些研究进行系统总结。因此，我们的主要目标是对相关研究进行系统综述和统计分析：我们使用 Pubmed、Cochrane Library、Google Scholar 和 Scopus 四个数据库对研究进行了系统检索。结果我们的研究结果表明，每日剂量为 100 毫克的沙芬那胺比 50 毫克的沙芬那胺治疗 PD 疼痛更有效。此外，与其他类型的帕金森病疼痛相比，使用沙芬那胺治疗波动相关疼痛和水肿引起的疼痛的减轻效果更为一致。我们还尝试提出了沙芬那胺对大脑疼痛处理的作用机制，这需要在今后的研究中进行更详细的探讨。结论临床证据表明，沙芬那胺可能对出现波动相关性疼痛和水肿引起的疼痛的帕金森病患者特别有益，因为与其他类型的疼痛相比，这些亚型疼痛的改善程度更大。根据纳入研究的结果，沙芬那胺似乎可以减轻总体疼痛负担。然而，由于缺乏足够的数据进行荟萃分析，今后的研究需要报告平均疼痛评分及其标准偏差。

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引用次数: 0

Postural balance and visual dependence in patients with demyelinating neuropathies differ between acquired and hereditary etiologies 脱髓鞘神经病患者的姿势平衡和视觉依赖在获得性病因和遗传性病因之间存在差异。

IF 2.8 4区医学 Q2 CLINICAL NEUROLOGY

Revue neurologique

Pub Date : 2025-01-01 DOI: 10.1016/j.neurol.2024.10.002

L. Dupont , L. Defebvre , J.-B. Davion , A. Delval , C. Tard

Background

Demyelinating polyneuropathies affect posture and can be either hereditary, as in Charcot-Marie-Tooth type 1A (CMT1A), or autoimmune, as in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Clinical differentiation between these two neuropathies can be challenging and biomarkers are lacking. No comparative analysis of their balance profiles has been conducted.

Methods

The postural balance of 23 patients with CIDP and 23 patients with CMT1A, matched for age, sex, and functional scores, were recorded using a force platform under various conditions. The effects of visual dependence were examined based on center of pressure velocity, 90% confidence ellipse area, and the Romberg quotient which represents the ratio between posturography with eyes closed and eyes open.

Results

With eyes open, the two groups exhibited similar area and velocity. They increased their postural sway when visual input was eliminated. Nevertheless, the increase in postural sway was less pronounced in CMT1A patients than in patients with CIDP, who then had a higher Romberg quotient.

Conclusion

Patients with CMT1A appear to have developed compensatory mechanisms over time resulting in reduced visual dependence. Further studies are necessary to explore other compensatory mechanisms of equilibrium that could be targeted by rehabilitation for patients with CIDP.

背景：脱髓鞘性多发性神经病会影响姿势，既可能是遗传性的，如夏科-玛丽-牙1A型（CMT1A），也可能是自身免疫性的，如慢性炎症性脱髓鞘性多发性神经病（CIDP）。这两种神经病的临床鉴别具有挑战性，而且缺乏生物标记物。目前尚未对这两种神经病的平衡特征进行比较分析：方法：使用力平台记录了 23 名 CIDP 患者和 23 名 CMT1A 患者在不同条件下的姿势平衡情况。根据压力中心速度、90%置信椭圆面积和罗姆伯格商数（代表闭眼和睁眼时的体位测量比率）对视觉依赖性的影响进行了研究：结果：睁眼时，两组显示出相似的面积和速度。当视觉输入消失时，他们的姿势摇摆增加。然而，CMT1A 患者的姿势摇摆增加不如 CIDP 患者明显，后者的朗伯格商数更高：结论：随着时间的推移，CMT1A 患者似乎形成了代偿机制，从而降低了视觉依赖性。有必要开展进一步的研究，探索 CIDP 患者康复治疗可针对的其他平衡代偿机制。

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引用次数: 0

Update of French migraine epidemiology: A narrative review 法国偏头痛流行病学的最新进展：述评。

IF 2.8 4区医学 Q2 CLINICAL NEUROLOGY

Revue neurologique

Pub Date : 2025-01-01 DOI: 10.1016/j.neurol.2024.10.006

M. Lanteri-Minet , C. Lucas

This narrative review concerned the studies performed on representative samples of the French general population carried out over more than thirty years and aims to provide an update on the French migraine epidemiology. Eleven studies were selected (GRIM-1, MIG-ACCESS, GRIM-2, FRAMIG-2000, FRAMIG-3, GRIM-3, EUROLIGHT, IBMS, SNDS PACA/CORSICA, EGB FRANCE, CaMEO-I). The data extracted relates to four healings: prevalence and distribution, individual burden, recognition and care and societal burden.

这篇叙述性综述涉及30多年来对法国普通人群的代表性样本进行的研究，旨在提供法国偏头痛流行病学的最新情况。共选择了11项研究（GRIM-1、米格- access、GRIM-2、FRAMIG-2000、FRAMIG-3、GRIM-3、EUROLIGHT、IBMS、SNDS PACA/CORSICA、EGB FRANCE、CaMEO-I）。提取的数据涉及四种治疗：患病率和分布、个人负担、认识和护理以及社会负担。

引用次数: 0

Woodhouse-Sakati syndrome: A review 伍德豪斯-萨卡蒂综合征：综述。

IF 2.8 4区医学 Q2 CLINICAL NEUROLOGY

Revue neurologique

Pub Date : 2025-01-01 DOI: 10.1016/j.neurol.2023.11.008

C. Messina

Neurodegeneration with brain iron accumulation (NBIA) is a rare and inherited spectrum of movement disorders caused by mutations affecting the function of proteins that participate in the homeostasis of tissue metals such as iron or copper and other metabolic pathways, although the precise function of the proteins encoded are not always known. Woodhouse-Sakati Syndrome (WSS) is one of the rarest NBIAs. Patients with WSS are characterized by endocrinological and neurological manifestations and neuroradiological findings. However, diagnostic criteria have not been published yet. This article reviews updates on the genetic, clinical, biological and imaging findings of WSS and provides a practical guide to recognize this extremely rare disorder.

脑铁积聚性神经变性（NBIA）是一种罕见的遗传性运动障碍，是由于参与组织金属（如铁或铜）平衡和其他代谢途径的蛋白质功能受到突变影响而引起的，但所编码蛋白质的确切功能并不总是已知的。伍德豪斯-萨卡蒂综合征（Woodhouse-Sakati Syndrome，WSS）是最罕见的 NBIAs 之一。伍德豪斯-萨卡蒂综合征患者的特征是内分泌和神经系统表现以及神经放射学发现。然而，诊断标准尚未公布。本文回顾了 WSS 在遗传学、临床、生物学和影像学方面的最新发现，并为认识这种极其罕见的疾病提供了实用指南。

引用次数: 0

French validation of the sexual complaints screener (SCS) for patients with multiple sclerosis 多发性硬化症患者性主诉筛选器（SCS）的法国验证。

IF 2.8 4区医学 Q2 CLINICAL NEUROLOGY

Revue neurologique

Pub Date : 2025-01-01 DOI: 10.1016/j.neurol.2024.09.009

S. Bresch , H. Joly , R. Colamarino , I. Bentellis , S. Tur , S. Fakir , C. Burte , C. Lebrun-Frenay

Background

Sexual dysfunctions are frequent in the general population and even more frequent in individuals with chronic neurological disorders like multiple sclerosis (MS). Several studies indicate that patients would like healthcare workers to address sexual problems. Indeed, such complaints are not currently sufficiently detected and treated. In clinical practice, a screening tool is lacking to help French-speaking patients and healthcare professionals address this issue.

Objective

The main aim of this study was to evaluate the reliability and validity of the French adaptation of the self-assessment Sexual Complaints Screener scale for Women (SCS-W) and Men (SCS-M).

Method

A prospective study was conducted among patients with MS in two centers. The SCS-W was adapted into French (QPS-F: questionnaire de plaintes sexuelles – Femmes) and compared to the reference questionnaire Female Sexual Function Index-19 (FSFI-19). The SCS-M was adapted into French (QPS-H: questionnaire de plaintes sexuelles – Hommes) and compared to the reference International Index of Erectile Function-15 (IIEF-15).

Results

Included were 101 women and 35 men with MS. Median age was 40.5 (range: 20–68) years. Based on the Cronbach alpha coefficient, the internal coherence of the QPS in French was 0.89 for women (QPS-F) and 0.71 for men (QPS-H), indicating high reliability. For QPS-F, the bivariate Pearson correlation coefficient indicated good convergence for desire and satisfaction, and average convergence for orgasm, pain, and arousal excitability. For QPS-H, the convergence was good for desire, pleasure, and ejaculation.

Conclusion

The French versions of the SCS-W/M scales, namely QPS-F and QPS-H, are reliable and validated tools compared with the reference questionnaires, FSFI and IIEF-15, respectively. The QPS-F/H are useful tools for brief, simple, and accurate screening and assessment of sexual complaints. They provide supportive information for clinicians who are less familiar with the clinical significance of sexual complaints and hence can be helpful to achieve more adapted care. These scales are adapted, but not specific, to MS. They could be used in other pathologies and the general population.

背景：性功能障碍在普通人群中很常见，在多发性硬化症（MS）等慢性神经系统疾病患者中更为常见。多项研究表明，患者希望医护人员能够解决性问题。事实上，目前对这类投诉的检测和治疗还不够充分。在临床实践中，缺乏一种筛查工具来帮助讲法语的患者和医护人员解决这一问题：本研究的主要目的是评估改编自法语的女性（SCS-W）和男性（SCS-M）性投诉筛查自评量表的可靠性和有效性：方法：在两个中心的多发性硬化症患者中开展了一项前瞻性研究。将 SCS-W 改编成法文（QPS-F：sexuelles questionnaire de plaintes sexuelles - Femmes），并与参考问卷 "女性性功能指数-19"（FSFI-19）进行比较。SCS-M 被改编成法文（QPS-H：sexuelles questionnaire de plaintes sexuelles - Hommes），并与参考的国际勃起功能指数-15（IIEF-15）进行比较：结果：共纳入 101 名女性和 35 名男性多发性硬化症患者。中位年龄为 40.5 岁（范围：20-68 岁）。根据克朗巴赫α系数，法语 QPS（QPS-F）的内部一致性为 0.89，而法语 QPS（QPS-H）的内部一致性为 0.71，这表明 QPS 具有很高的可靠性。对于 QPS-F，双变量皮尔逊相关系数表明，欲望和满意度的趋同性较好，性高潮、疼痛和唤醒兴奋性的趋同性一般。对于 QPS-H，欲望、快感和射精的趋同性较好：结论：与参考问卷 FSFI 和 IIEF-15 相比，法文版 SCS-W/M 量表，即 QPS-F 和 QPS-H 是可靠且经过验证的工具。QPS-F/H 是简短、简单、准确筛查和评估性主诉的有用工具。它们为不太了解性主诉临床意义的临床医生提供了辅助信息，因此有助于实现更适合的护理。这些量表适用于多发性硬化症，但不具有特异性。它们可用于其他病症和普通人群。

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引用次数: 0

Congenital myasthenic syndromes by Epsilon subunit mutations: Phenotypic profiles of 17 Algerian families 由 Epsilon 亚基突变引起的先天性肌无力综合征：17 个阿尔及利亚家庭的表型特征。

IF 2.8 4区医学 Q2 CLINICAL NEUROLOGY

Revue neurologique

Pub Date : 2025-01-01 DOI: 10.1016/j.neurol.2024.09.007

M.I. Kediha , M. Tazir , D. Sternberg , B. Eymard , L. Ali Pacha

Background

Congenital myasthenic syndromes (CMS) are a heterogeneous group of rare genetic disorders. The acetyl choline receptor contains five subunits, with a predominance of mutations affecting the epsilon subunit gene called cholinergic receptor nicotinic epsilon (CHRNE) gene.

Objective

To study the clinical phenotype of 17 families with CHRNE gene mutations.

Methods

We report a series of 17 families with 22 affected patients carrying different mutations encoding CHRNE proteins.

Results

We studied their clinical and biological phenotypes, as well as their evolutionary profile and their response to the different therapies proposed. A phenotypic comparison was made between the families carrying the founding Maghrebian mutation and the other mutations found in this series.

Conclusion

The CHRNE gene mutations are the most frequent ones in CMS. The phenotypes reported in this study are heterogeneous, and can depend on the causative mutation.

背景：先天性肌无力综合征（CMS）是一组异质性的罕见遗传疾病。乙酰胆碱受体包含五个亚基，其中epsilon亚基基因（即胆碱能受体烟碱epsilon（CHRNE）基因）的突变占绝大多数：研究17个CHRNE基因突变家族的临床表型：方法：我们报告了一系列 17 个家族的 22 位患者，他们携带不同的 CHRNE 蛋白编码突变：结果：我们研究了这些患者的临床和生物学表型，以及他们的进化特征和对不同疗法的反应。我们对携带马格里布基因突变的家族与该系列中发现的其他基因突变家族进行了表型比较：结论：CHRNE 基因突变是 CMS 中最常见的突变。结论：CHRNE 基因突变是 CMS 中最常见的突变。本研究中报告的表型具有异质性，可能取决于致病突变。

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引用次数: 0

Brain MRI in status epilepticus: Relevance of findings 癫痫状态的脑磁共振成像：研究结果的相关性

IF 2.8 4区医学 Q2 CLINICAL NEUROLOGY

Revue neurologique

Pub Date : 2025-01-01 DOI: 10.1016/j.neurol.2023.12.011

T. Bonduelle , M. Ollivier , A. Gradel , J. Aupy

Status epilepticus (SE) represents one of the most common neurological emergencies, associated with high mortality and an important risk of functional sequelae in survivors. Magnetic resonance imaging (MRI) offers the possibility of early and noninvasive observation of seizure-induced parenchymal disturbances secondary to the epileptic process. In the present review, we propose a descriptive and comprehensive understanding of current knowledge concerning seizure-induced MRI abnormalities in SE, also called peri-ictal MRI abnormalities (PMAs). We then discuss how PMAs, as a noninvasive biomarker, could be helpful to optimize patient prognostication in SE management. Finally, we discuss alternative promising MRI approaches, including arterial spin labeling (ASL), susceptibility-weighted imaging (SWI), dynamic contrast-enhanced (DCE) MRI and dynamic susceptibility contrast (DSC) MRI that could refine our understanding of SE, particularly in non-convulsive form.

癫痫状态（SE）是最常见的神经系统急症之一，死亡率高，幸存者出现功能性后遗症的风险也很高。磁共振成像（MRI）为早期无创观察癫痫过程继发的癫痫诱发的实质障碍提供了可能。在本综述中，我们对目前有关 SE 癫痫发作诱发的 MRI 异常（也称为癫痫发作周 MRI 异常（PMAs））的知识进行了描述性的全面了解。然后，我们讨论了作为一种非侵入性生物标志物，PMAs 如何有助于优化 SE 管理中的患者预后。最后，我们讨论了其他有前景的 MRI 方法，包括动脉自旋标记 (ASL)、感度加权成像 (SWI)、动态对比增强 (DCE) MRI 和动态感度对比 (DSC) MRI，它们可以完善我们对 SE（尤其是非惊厥型 SE）的理解。

引用次数: 0

Could PLS represent a UMN-predominant ALS syndrome? PLS 是否代表一种 UMN 显性 ALS 综合征？

IF 2.8 4区医学 Q2 CLINICAL NEUROLOGY

Revue neurologique

Pub Date : 2025-01-01 DOI: 10.1016/j.neurol.2024.04.006

P. Corcia , P. Couratier , C. Ingre

Primary lateral sclerosis (PLS) is a motor neuron condition marked by pure upper motor neuron (UMN) degeneration. PLS represents around 3% of all motor neuron diseases. Classically the prognosis of PLS is less severe than those of amyotrophic lateral sclerosis (ALS). This explains the necessity to distinguish both conditions as early as possible. The key hallmark between the two diseases is the involvement of the lower motor neuron (LMN) system which is classically considered spared in PLS contrary to ALS. Although it seemed clinically easy to distinguish PLS from ALS with the aid of clinical and complementary examinations, there is a large body of evidence highlighting that the LMN system might be impaired in PLS. This led us to suggest that PLS might be considered as an almost pure UMN ALS phenotype.

原发性侧索硬化症（PLS）是一种以单纯上运动神经元（UMN）变性为特征的运动神经元疾病。原发性侧索硬化症约占所有运动神经元疾病的 3%。与肌萎缩侧索硬化症（ALS）相比，原发性肌萎缩侧索硬化症的预后通常较轻。因此，有必要尽早区分这两种疾病。这两种疾病的主要特征是下运动神经元（LMN）系统受累，与 ALS 相反，下运动神经元系统在 PLS 中通常被认为是不受影响的。虽然在临床上，借助临床和辅助检查似乎很容易将 PLS 与 ALS 区分开来，但有大量证据表明，LMN 系统可能在 PLS 中受损。这使我们认为，PLS 可被视为一种几乎纯粹的 UMN ALS 表型。

引用次数: 0