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Miyazaki Syndrome as a Complication of Shunt Drainage 作为分流引流并发症的宫崎综合征。
IF 8.1 1区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2024-08-27 DOI: 10.1002/ana.27067
Rick H. G. J. van Lanen MD, MSc, Jasper van Aalst MD, PhD, Mariël P. Ter Laak-Poort MD, PhD
<p>A 41-year-old female was referred to the neurosurgery outpatient clinic with progressive bipyramidal syndrome. Medical history included premature birth and prior intraventricular hemorrhage with hydrocephalus. Ventricular-peritoneal (VP) shunting was performed in the management of hydrocephalus. However, complications related to over-drainage, along with hyperostosis of the skull and craniosynostosis, caused scaphocephaly.</p><p>Over the course of several months, she experienced progressive difficulties walking and developed right-sided weakness. Neurological examination revealed right-sided hemiparesis, hyperreflexia, and bilateral Babinski's sign. Imaging of the brain and cervical spine (Fig 1) showed scaphocephaly with slit-like ventricles and myelopathy extending from the C1 to C3 levels, along with the presence of spinal epidural venous engorgement. In the absence of a diagnosis, she was placed under long-term follow-up care by her neurologist. Follow-up magnetic resonance imaging (MRI) of the cervical spine revealed progressive myelopathy, before the diagnosis of Miyazaki syndrome was made. She underwent VP-shunt revision, with implantation of a Miethke proGAV 2.0 0-20/20 (programmable valve pressure setting 8). Outpatient clinic follow-up at 6 months showed stabilization of the bipyramidal symptoms. Follow-up MRI showed improvement of the epidural venous engorgement.</p><p>Miyazaki syndrome arises as a complication of cerebrospinal fluid (CSF) hypotension caused by excessive drainage through VP-shunting. Although CSF hypotension is often associated with well-known symptoms, like orthostatic headache and cranial nerve palsies, the development of epidural venous engorgement leading to spinal cord compression is a less common but critical manifestation.</p><p>The syndrome is characterized by cervical myelopathy or radiculopathy due to cervical epidural venous congestion, results from complex pathophysiological mechanisms.<span><sup>1, 2</sup></span> These include changes in CSF pressure, consistent with the Monro-Kellie doctrine, and dysfunction in the Starling resistor, leading to the enlargement and dilation of the spinal epidural venous plexus.<span><sup>1</sup></span> Venous congestion can lead to spinal cord or nerve roots compression or circulation hampering,<span><sup>1</sup></span> causing neurological symptoms, whereas presenting a unique diagnostic challenge. One of the striking features of Miyazaki syndrome is that it can manifest without the typical symptom of orthostatic headache, which is commonly associated with CSF hypotension. Instead, patients with Miyazaki syndrome may develop myelopathy symptoms slowly over time, making it challenging to diagnose, potentially leading to misdiagnosis.<span><sup>3</sup></span> This underlines the importance of considering Miyazaki syndrome in the differential diagnosis of patients with VP-shunts who present with myelopathy but do not experience headaches. Timely recognition is crucial and
一名 41 岁的女性因进行性双锥体综合征转诊至神经外科门诊。病史包括早产和脑室内出血并伴有脑积水。在治疗脑积水的过程中进行了脑室-腹膜(VP)分流术。然而,与过度引流有关的并发症以及颅骨骨质增生和颅骨发育不良导致了头颅骨畸形。几个月后,她逐渐出现行走困难,右侧肢体无力。神经系统检查发现她右侧偏瘫、反射亢进和双侧巴宾斯基征。脑部和颈椎的影像学检查(图 1)显示,颅骨后凸,脑室呈狭缝状,脊髓病变从 C1 水平延伸至 C3 水平,并伴有脊髓硬膜外静脉充血。在没有确诊的情况下,神经科医生对她进行了长期随访。随访的颈椎磁共振成像(MRI)显示,在确诊宫崎综合征之前,她的脊髓病变呈进行性发展。她接受了 VP 分流改造手术,植入了 Miethke proGAV 2.0 0-20/20(可编程阀门压力设置为 8)。6 个月的门诊随访显示双锥体症状趋于稳定。宫崎综合征是脑脊液(CSF)低血压的一种并发症,由 VP 分流引流过多引起。虽然 CSF 低血压常伴有众所周知的症状,如正位性头痛和颅神经麻痹,但硬膜外静脉充血导致脊髓压迫是一种不太常见但却非常重要的表现、1 静脉充血可导致脊髓或神经根受压或血液循环障碍,1 引起神经系统症状,同时也是一种独特的诊断难题。宫崎综合征的一个显著特点是,它可以没有典型的正压性头痛症状,而正压性头痛通常与脑脊液低血压有关。相反,宫崎综合征患者的脊髓病症状可能会随着时间的推移而缓慢发展,这就给诊断带来了挑战,有可能导致误诊。3 这就强调了在对出现脊髓病但不伴有头痛的 VP 分流患者进行鉴别诊断时考虑宫崎综合征的重要性。及时识别至关重要,需要进行影像学诊断,尤其是脑部和脊柱磁共振成像,重点检查静脉血流,以确诊宫崎综合征。即使没有其他典型的低血压图像,颈静脉丛肿胀也可能是该综合征的一个关键指标。可能的干预措施包括调整瓣膜系统的开放压力,必要时插入可编程瓣膜,或完全关闭/移除分流管。1-3 总之,本病例强调了了解宫崎综合征的临床重要性,这是一种罕见但可能导致衰弱的脑脊液通过 VP 分流管过度引流的并发症,并强调了早期诊断和适当治疗对改善患者预后的重要意义、J.vA.和M.P.TL-P参与了研究的构思和设计;R.H.G.J.vL.参与了文本的起草和图表的绘制;R.H.G.J.vL.和M.P.TL-P参与了数据的获取和分析。
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引用次数: 0
Population-Based Evidence for the Use of Serum Neurofilaments as Individual Diagnostic and Prognostic Biomarkers in Amyotrophic Lateral Sclerosis 将血清神经丝作为肌萎缩侧索硬化症个体诊断和预后生物标记物的人群证据
IF 8.1 1区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2024-08-23 DOI: 10.1002/ana.27054
Simon Witzel MD, André Huss PhD, Gabriele Nagel MD, Angela Rosenbohm MD, Dietrich Rothenbacher MD, Raphael S. Peter PhD, Hansjörg Bäzner MD, Axel Börtlein MD, Silke Dempewolf MD, Martin Schabet MD, Martin Hecht MD, Andreas Kohler MD, Christian Opherk MD, Andrea Naegele MD, Norbert Sommer MD, Alfred Lindner MD, Christoforos Alexudis, Franziska Bachhuber PhD, Steffen Halbgebauer PhD, David Brenner MD, Wolfgang Ruf MD, Ulrike Weiland MD, Benjamin Mayer PhD, Joachim Schuster PhD, Johannes Dorst MD, Hayrettin Tumani MD, Albert C. Ludolph MD, and the ALS Registry Swabia Study Group

Objective

Neurofilament light chains (NfL) and phosphorylated neurofilament heavy chains (pNfH), established as diagnostic and prognostic biomarkers in hospital-based amyotrophic lateral sclerosis (ALS) cohorts, are now surrogate markers in clinical trials. This study extends their evaluation to a population level, with the aim of advancing their full establishment and assessing the transferability of biomarker findings from controlled cohorts to real-world ALS populations.

Methods

We measured serum NfL and pNfH levels in all ALS patients (n = 790) and general population controls (n = 570) with available baseline samples participating in the epidemiological ALS Registry Swabia, providing platform-specific (ELLA™) reference data and Z-scores for controls, as well as reference data, disease-specific Z-scores and longitudinal data in ALS. We evaluated the diagnostic and prognostic utility of neurofilaments and quantified the impact of ALS-related factors and non-ALS confounders.

Results

Neurofilaments showed high diagnostic and prognostic utility at the population level, with NfL superior to pNfH. The novel concept of a population-based ALS Z-score significantly improved the prognostic utility compared to absolute raw values. Both biomarkers increased more strongly with age in controls than in ALS, and age adjustment improved diagnostic accuracy. Our data show that disease progression rates, ALS phenotype, body mass index (BMI), and renal function need to be considered when interpreting neurofilament levels; longitudinal neurofilament levels were generally stable in individual patients, especially when adjusted for age and baseline levels.

Interpretation

Population-based assessment enhances the utility of particularly serum NfL as a diagnostic and prognostic biomarker in ALS and improves the translation of findings from controlled cohorts to real-world populations. ANN NEUROL 2024;96:1040–1057

目的:神经丝蛋白轻链(NfL)和磷酸化神经丝蛋白重链(pNfH神经丝蛋白轻链(NfL)和磷酸化神经丝蛋白重链(pNfH)在以医院为基础的肌萎缩性脊髓侧索硬化症(ALS)队列中被确立为诊断和预后生物标志物,现已成为临床试验中的替代标志物。本研究将对它们的评估扩展到人群水平,目的是推进它们的全面建立,并评估生物标志物研究结果从对照队列到实际 ALS 人群的可转移性:我们测量了参与斯瓦比亚 ALS 流行病学登记的所有 ALS 患者(n = 790)和普通人群对照组(n = 570)的血清 NfL 和 pNfH 水平,为对照组提供了平台特异性(ELLA™)参考数据和 Z 值,以及 ALS 的参考数据、疾病特异性 Z 值和纵向数据。我们评估了神经丝的诊断和预后效用,并量化了ALS相关因素和非ALS混杂因素的影响:结果:神经丝在人群水平上显示出很高的诊断和预后效用,NfL优于pNfH。与绝对原始值相比,基于人群的 ALS Z 评分这一新理念大大提高了预后效用。在对照组中,这两种生物标志物随年龄的增长而增加的程度比在 ALS 中更强,年龄调整提高了诊断的准确性。我们的数据显示,在解释神经丝蛋白水平时,需要考虑疾病进展率、ALS 表型、体重指数(BMI)和肾功能;纵向神经丝蛋白水平在个体患者中通常是稳定的,尤其是在根据年龄和基线水平进行调整后:基于人群的评估提高了血清神经丝蛋白作为 ALS 诊断和预后生物标志物的实用性,并改善了从对照队列到实际人群的研究结果转化。ann neurol 2024.
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引用次数: 0
Electro-Clinical Features and Functional Connectivity Analysis in SYN1-Related Epilepsy. SYN1相关癫痫的电临床特征和功能连接性分析
IF 8.1 1区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2024-08-23 DOI: 10.1002/ana.27063
Vincent Moya Quiros, Ahmed Adham, Philippe Convers, Gaetan Lesca, François Mauguiere, Hugo Soulier, Alexis Arzimanoglou, Allan Bayat, Hilde Braakman, Jean-Philippe Camdessanche, Philippe Casenave, Laurence Chaton, Yves Chaix, Maxime Chochoi, Christel Depienne, Vincent Desportes, Jessie De Ridder, Vera Dinkelacker, Elena Gardella, Gerhard J Kluger, Julien Jung, Martine Lemesle Martin, Maria Margherita Mancardi, Markus Mueller, Anne-Lise Poulat, Konrad Platzer, Agathe Roubertie, Marijn F Stokman, Anneke T Vulto-van Silfhout, Gert Wiegand, Laure Mazzola

Objective: There is currently scarce data on the electroclinical characteristics of epilepsy associated with synapsin 1 (SYN1) pathogenic variations. We examined clinical and electro-encephalographic (EEG) features in patients with epilepsy and SYN1 variants, with the aim of identifying a distinctive electroclinical pattern.

Methods: In this retrospective multicenter study, we collected and reviewed demographic, genetic, and epilepsy data of 19 male patients with SYN1 variants. Specifically, we analyzed interictal EEG data for all patients, and electro-clinical data from 10 epileptic seizures in 5 patients, using prolonged video-EEG monitoring recordings. Inter-ictal EEG functional connectivity parameters and frequency spectrum of the 10 patients over 12 years of age, were computed and compared with those of 56 age- and sex-matched controls.

Results: The main electroclinical features of epilepsy in patients with SYN1 were (1) EEG background and organization mainly normal; (2) interictal abnormalities are often rare or not visible on EEG; (3) more than 60% of patients had reflex seizures (cutaneous contact with water and defecation being the main triggers) isolated or associated with spontaneous seizures; (4) electro-clinical semiology of seizures was mainly temporal or temporo-insulo/perisylvian with a notable autonomic component; and (5) ictal EEG showed a characteristic rhythmic theta/delta activity predominating in temporo-perisylvian regions at the beginning of most seizures. Comparing patients with SYN1 to healthy subjects, we observed a shift to lower frequency bands in power spectrum of interictal EEG and an increased connectivity in both temporal regions.

Interpretation: A distinct epilepsy syndrome emerges in patients with SYN1, with a rather characteristic clinical and EEG pattern suggesting predominant temporo-insular involvement. ANN NEUROL 2024.

目的:目前,有关与突触素1(SYN1)致病变异相关的癫痫的电临床特征的数据很少。我们研究了癫痫和 SYN1 变异患者的临床和脑电图(EEG)特征,旨在确定一种独特的临床电模式:在这项回顾性多中心研究中,我们收集并审查了19名SYN1变异体男性患者的人口统计学、遗传学和癫痫数据。具体来说,我们分析了所有患者的发作间期脑电图数据,并利用长时间视频脑电图监测记录分析了 5 名患者 10 次癫痫发作的电临床数据。计算了10名12岁以上患者发作间期脑电图功能连接参数和频谱,并与56名年龄和性别匹配的对照组进行了比较:SYN1患者癫痫的主要电临床特征是:(1)脑电图背景和组织主要正常;(2)发作间期异常通常很少见或在脑电图上不明显;(3)60%以上的患者有反射性发作(皮肤接触水和排便是主要诱因),与自发性发作隔离或伴有反射性发作;(4) 癫痫发作的电-临床特征主要是颞叶或颞内/外周,并伴有明显的自主神经成分;以及 (5) 发作期脑电图显示,在大多数癫痫发作开始时,颞外周区域会出现特有的节律性θ/δ活动。将 SYN1 患者与健康受试者进行比较,我们观察到发作间期脑电图的功率谱向低频带转移,两个颞区的连接性增强:SYN1患者会出现一种独特的癫痫综合征,其临床和脑电图模式颇具特征性,表明主要受累于颞岛部。ann neurol 2024.
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引用次数: 0
Syrinx beyond Cord: Syringocephalia Extending to Corona Radiata 虹膜超出脐带咽鼓管延伸至放射冠。
IF 8.1 1区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2024-08-22 DOI: 10.1002/ana.27056
Aditi Saini MD, Sarbesh Tiwari DM, Jaskaran Singh Gosal Mch, Mayank Garg Mch
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引用次数: 0
Higher Validity, Lower Radiation: A New Ictal Single-Photon Emission Computed Tomography Framework 更高的有效性,更低的辐射:新型椎体单光子发射计算机断层扫描框架
IF 8.1 1区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2024-08-21 DOI: 10.1002/ana.27061
Felix Bitzer BSc, Lennart Walger MSc, Tobias Bauer MD, BSc, Freya Schulte, Florian C. Gaertner MD, Matthias Schmitz, Martin Schidlowski PhD, Randi von Wrede MD, Attila Rácz MD, Tobias Baumgartner MD, Vadym Gnatkovsky MD, Daniel Paech MD, Valeri Borger MD, Hartmut Vatter MD, Bernd Weber MD, Dominik L. Michels PhD, Tony Stöcker PhD, Markus Essler MD, Josemir W. Sander MD, PhD, Alexander Radbruch MD, JD, Rainer Surges MD, MHBA, Theodor Rüber MD

Objective

To assess whether arterial spin labeling perfusion images of healthy controls can enhance ictal single-photon emission computed tomography analysis and whether the acquisition of the interictal image can be omitted.

Methods

We developed 2 pipelines: The first uses ictal and interictal images and compares these to single-photon emission computed tomography and arterial spin labeling of healthy controls. The second pipeline uses only the ictal image and the analogous healthy controls. Both pipelines were compared to the gold standard analysis and evaluated on data of individuals with epilepsy who underwent ictal single-photon emission computed tomography imaging during presurgical evaluation between 2010 and 2022. Fifty healthy controls prospectively underwent arterial spin labeling imaging. The correspondence between the detected hyperperfusion and the postoperative resection cavity or the presumably affected lobe was assessed using Dice score and mean Euclidean distance. Additionally, the outcomes of the pipelines were automatically assigned to 1 of 5 concordance categories.

Results

Inclusion criteria were met by 43 individuals who underwent epilepsy surgery and by 73 non-surgical individuals with epilepsy. Compared to the gold standard analysis, both pipelines resulted in significantly higher Dice scores and lower mean distances (p < 0.05). The combination of both provided localizing results in 85/116 cases, compared to 54/116 generated by the current gold standard analysis and the ictal image alone produced localizing results in 60/116 (52%) cases.

Interpretation

We propose a new ictal single-photon emission computed tomography protocol; it finds relevantly more ictal hyperperfusion, and halves the radiation dose in about half of the individuals. ANN NEUROL 2024;96:1160–1173

目的评估健康对照组的动脉自旋标记灌注图像能否增强发作期单光子发射计算机断层扫描分析,以及能否省略发作间期图像的采集:我们开发了两个管道:第一个管道使用发作期和发作间期图像,并将其与健康对照组的单光子发射计算机断层扫描和动脉自旋标记进行比较。第二种方法只使用发作期图像和类似的健康对照组。两个管道都与金标准分析进行了比较,并对 2010 年至 2022 年期间在手术前评估期间接受发作期单光子发射计算机断层扫描成像的癫痫患者数据进行了评估。50 名健康对照组前瞻性地接受了动脉自旋标记成像。使用 Dice 评分和平均欧氏距离评估检测到的高灌注与术后切除腔或推测受影响脑叶之间的对应关系。此外,管道结果被自动分配到 5 个一致性类别中的 1 个:43名接受癫痫手术的患者和73名未接受手术的癫痫患者符合纳入标准。与金标准分析相比,两种管道的 Dice 得分都明显较高,平均距离也较低(p 解释):我们提出了一种新的发作期单光子发射计算机断层扫描方案;它能发现更多相关的发作期高灌注,并将大约一半患者的辐射剂量减半。ann neurol 2024.
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引用次数: 0
Assessing the Efficacy and Limitations of Rescue Thrombectomy in Acute Ischemic Stroke 评估急性缺血性脑卒中抢救性血栓切除术的疗效和局限性。
IF 8.1 1区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2024-08-20 DOI: 10.1002/ana.27065
Qian Xu MMed, Shicai Huang CS
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引用次数: 0
Allele-Specific Editing of a Dominant SCN8A Epilepsy Variant Protects against Seizures and Lethality in a Murine Model 对显性 SCN8A 癫痫变异体进行等位基因特异性编辑可防止小鼠模型中的癫痫发作和致死。
IF 8.1 1区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2024-08-19 DOI: 10.1002/ana.27053
Wenxi Yu PhD, Sophie F. Hill PhD, Yumei Huang PhD, Limei Zhu PhD, Yiannos Demetriou BS, Julie Ziobro MD, PhD, Faith Reger MS, Xiaoyan Jia PhD, Joanna Mattis MD, PhD, Miriam H. Meisler PhD

Objective

Developmental and epileptic encephalopathies (DEEs) can result from dominant, gain of function variants of neuronal ion channels. More than 450 de novo missense variants of the sodium channel gene SCN8A have been identified in individuals with DEE.

Methods

We studied a mouse model carrying the patient Scn8a variant p.Asn1768Asp. An AAV-PHP.eB virus carrying an allele-specific single guide RNA (sgRNA) was administered by intracerebroventricular injection. Cas9 was provided by an inherited transgene.

Results

Allele-specific disruption of the reading frame of the pathogenic transcript generated out-of-frame indels in 1/4 to 1/3 of transcripts throughout the brain. This editing efficiency was sufficient to rescue lethality and seizures. Neuronal hyperexcitability was reduced in cells expressing the virus.

Interpretation

The data demonstrate efficient allele-specific editing of a dominant missense variant and support the feasibility of allele-specific therapy for DEE epilepsy. ANN NEUROL 2024;96:958–969

目的:神经元离子通道的显性功能增益变异可导致发育性和癫痫性脑病(DEEs)。在DEE患者中已发现450多个钠通道基因SCN8A的从头错义变异:我们研究了携带患者 Scn8a 变异 p.Asn1768Asp 的小鼠模型。方法:我们研究了携带患者 Scn8a 变体 p.Asn1768Asp 的小鼠模型。通过脑室内注射,给小鼠注射了携带等位基因特异性单导 RNA(sgRNA)的 AAV-PHP.eB 病毒。Cas9由遗传转基因提供:等位基因特异性地破坏了致病转录本的阅读框,在整个大脑中1/4到1/3的转录本中产生了框外嵌合。这种编辑效率足以挽救致死率和癫痫发作。表达病毒的细胞中神经元过度兴奋性降低:这些数据证明了对显性错义变体进行等位基因特异性编辑的效率,并支持对DEE癫痫进行等位基因特异性治疗的可行性。ann neurol 2024.
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引用次数: 0
Factors Affecting Resilience and Prevention of Alzheimer's Disease and Related Dementias 影响抗病能力和预防阿尔茨海默病及相关痴呆症的因素。
IF 8.1 1区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2024-08-17 DOI: 10.1002/ana.27055
Arjun V. Masurkar, Karyn Marsh, Brianna Morgan, Dominique Leitner, Thomas Wisniewski

Alzheimer's disease (AD) is a devastating, age-associated neurodegenerative disorder and the most common cause of dementia. The clinical continuum of AD spans from preclinical disease to subjective cognitive decline, mild cognitive impairment, and dementia stages (mild, moderate, and severe). Neuropathologically, AD is defined by the accumulation of amyloid β (Aβ) into extracellular plaques in the brain parenchyma and in the cerebral vasculature, and by abnormally phosphorylated tau that accumulates intraneuronally forming neurofibrillary tangles (NFTs). Development of treatment approaches that prevent or even reduce the cognitive decline because of AD has been slow compared to other major causes of death. Recently, the United States Food and Drug Administration gave full approval to 2 different Aβ-targeting monoclonal antibodies. However, this breakthrough disease modifying approach only applies to a limited subset of patients in the AD continuum and there are stringent eligibility criteria. Furthermore, these approaches do not prevent progression of disease, because other AD-related pathologies, such as NFTs, are not directly targeted. A non-mutually exclusive alternative is to address lifestyle interventions that can help reduce the risk of AD and AD-related dementias (ADRD). It is estimated that addressing such modifiable risk factors could potentially delay up to 40% of AD/ADRD cases. In this review, we discuss some of the many modifiable risk factors that may be associated with prevention of AD/ADRD and/or increasing brain resilience, as well as other factors that may interact with these modifiable risk factors to influence AD/ADRD progression. [Color figure can be viewed at www.annalsofneurology.org] ANN NEUROL 2024;96:633–649

阿尔茨海默病(AD)是一种与年龄相关的破坏性神经退行性疾病,也是最常见的痴呆症病因。阿兹海默病的临床连续性从临床前疾病到主观认知能力下降、轻度认知障碍和痴呆阶段(轻度、中度和重度)。从神经病理学角度看,AD 的定义是淀粉样蛋白 β(Aβ)在脑实质和脑血管中积聚成细胞外斑块,以及异常磷酸化的 tau 在神经元内积聚形成神经纤维缠结(NFT)。与其他主要死亡原因相比,预防甚至减少注意力缺失症认知能力下降的治疗方法发展缓慢。最近,美国食品和药物管理局全面批准了 2 种不同的 Aβ 靶向单克隆抗体。然而,这种突破性的疾病改变方法仅适用于注意力缺失症连续体中的少数患者,而且有严格的资格标准。此外,这些方法并不能阻止疾病的进展,因为其他与AD相关的病变,如NFTs,并不是直接靶向的。一种非相互排斥的替代方法是采取生活方式干预措施,这有助于降低 AD 和 AD 相关痴呆症(ADRD)的发病风险。据估计,解决这些可改变的风险因素有可能延缓多达 40% 的 AD/ADRD 病例。在这篇综述中,我们将讨论可能与预防 AD/ADRD 和/或提高大脑恢复力有关的许多可改变的风险因素中的一些因素,以及可能与这些可改变的风险因素相互作用而影响 AD/ADRD 进展的其他因素。ann neurol 2024.
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引用次数: 0
Annals of Neurology: Volume 96, Number 3, September 2024 神经病学年鉴》:第 96 卷第 3 号,2024 年 9 月
IF 8.1 1区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2024-08-14 DOI: 10.1002/ana.26702
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引用次数: 0
Lacosamide and Levetiracetam Are Not Toxic to the Developing Mouse Brain 拉科萨胺和左乙拉西坦对发育中的小鼠大脑无毒性
IF 8.1 1区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2024-08-13 DOI: 10.1002/ana.27052
Kevin K. Noguchi PhD, Cory W. Palmer, Nicole A. Fuhler, Eric Neblock, Maya Fotedar, Chrysanthy Ikonomidou MD, PhD

Many antiseizure medications cause apoptotic cell death in developing brains. The newer antiseizure medication lacosamide is increasingly used in neonates and infants. Neurotoxicity of lacosamide and its combination with levetiracetam was studied in neonatal mice. Animals received single or repeat injections of saline, phenobarbital (75mg/kg), lacosamide (20–40mg/kg), levetiracetam (100mg/kg), lacosamide (40mg/kg) + levetiracetam (100mg/kg) and euthanized at 6 to 30 hours. Cells undergoing apoptosis were increased in the brains of phenobarbital-treated animals. Densities of apoptotic profiles following lacosamide and levetiracetam treatment did not differ from saline-treated controls. Findings suggest that lacosamide, levetiracetam and their combination do not cause apoptosis in developing mouse brains. ANN NEUROL 2024;96:812–818

许多抗癫痫药物会导致发育中的大脑细胞凋亡。新生儿和婴儿越来越多地使用较新的抗癫痫药物拉科萨胺。研究人员在新生小鼠体内研究了拉科酰胺及其与左乙拉西坦复方制剂的神经毒性。小鼠分别单次或重复注射生理盐水、苯巴比妥(75 毫克/千克)、拉科沙胺(20-40 毫克/千克)、左乙拉西坦(100 毫克/千克)、拉科沙胺(40 毫克/千克)+左乙拉西坦(100 毫克/千克),并在 6 至 30 小时后安乐死。苯巴比妥处理过的动物大脑中发生凋亡的细胞增多。拉科萨胺和左乙拉西坦处理后的凋亡细胞密度与生理盐水处理的对照组没有差异。研究结果表明,拉科萨胺、左乙拉西坦及其复方制剂不会导致发育中的小鼠大脑凋亡。ann neurol 2024.
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Annals of Neurology
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