Journal of the Neurological Sciences最新文献_第8页

Comment on “wearing-off symptoms persist in the majority of patients with multiple sclerosis after switching from natalizumab to ocrelizumab” 评论“大多数多发性硬化症患者在从natalizumab切换到ocrelizumab后，症状持续消退”。

IF 3.2 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Neurological Sciences

Pub Date : 2025-12-25 DOI: 10.1016/j.jns.2025.125705

Bhumesh Tyagi , Leelabati Toppo , Aishwarya Biradar

引用次数: 0

Prevalence and trajectories of post-COVID-19 neuromuscular conditions: A systematic-review and meta-analysis covid -19后神经肌肉疾病的患病率和轨迹：一项系统综述和荟萃分析

IF 3.2 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Neurological Sciences

Pub Date : 2025-12-25 DOI: 10.1016/j.jns.2025.125710

Elisabetta Pupillo , Maurizio A. Leone , Anthony Amato , Elisa Bianchi , Maxwell S. Damian , James Dyck , David Garcia-Azorin , Giorgia Giussani , Alla Guekht , Haruki Koike , Satish Khadilkar , Helmar Lehmann , Ksenia Pochigaeva , Juliya Povolnova , Dmitri Tumurov , Fedor Vetrov , Marianne de Visser , Andrea Sylvia Winkler , Wolfgang Grisold

Introduction

Neuromuscular diseases (NMDs) are a significant component of the post-acute sequelae of COVID-19. However, their long-term prevalence and trajectories remain poorly defined. This systematic review and meta-analysis aimed to determine the long-term prevalence in COVID-19 survivors of fourteen specific NMDs and related symptoms: cranial nerve diseases, Guillain-Barré syndrome, small fiber neuropathy, (poly)radiculopathies, (poly)neuropathies, plexopathies, motor neuron disease, myasthenia gravis, Lambert-Eaton syndrome, neuropathic pain, sarcopenia, myalgia, myalgia associated with other symptoms, and of other muscle diseases.

Methods

We searched MEDLINE, Embase, and the Cochrane Library (January 2020 through November 2024) for studies with at least 3 months of follow-up. Pooled prevalence estimates were calculated at multiple time points (acute phase to 24 months) using random effects models.

Results

Among 180 unique studies representing 15,865,322 cases (54 % female, mean age 50.0 years), the pooled prevalence for individuals with at least one NMD or related symptoms decreased from 36 % in the acute phase to 8 % at 24 months. Myalgia prevalence steadily declined from 35 % to 8 % by two years. A trend towards lower prevalences across the time points was observed also for Guillain-Barré syndrome, and other muscle diseases, while other conditions showed a more erratic pattern. The prevalence of neuropathic pain remained high and persisted almost unchanged through the follow-up period (from 31 % in the acute phase to 25 % at 12 months).

Conclusions

NMDs and related symptoms are common following COVID-19, but their general prevalence decreases with time. However, trajectories varied depending on the type of NMD or symptom.

神经肌肉疾病（NMDs）是COVID-19急性后后遗症的重要组成部分。然而，它们的长期流行率和发展轨迹仍不明确。本系统综述和meta分析旨在确定14种特异性nmd及相关症状的COVID-19幸存者的长期患病率：颅神经疾病、格林-巴罗综合征、小纤维神经病变、（多）神经根病、（多）神经病变、神经丛病、运动神经元病、重症肌无力、兰伯特-伊顿综合征、神经性疼痛、肌肉减少症、肌痛、与其他症状相关的肌痛以及其他肌肉疾病。方法：我们检索MEDLINE、Embase和Cochrane图书馆（2020年1月至2024年11月），寻找至少随访3个月的研究。使用随机效应模型计算多个时间点（急性期至24个月）的合并患病率估计值。结果：在180项独特的研究中，共有15,865,322例病例（54%为女性，平均年龄50.0岁），至少有一种NMD或相关症状的个体的总患病率从急性期的36%下降到24个月时的8%。肌痛的患病率在两年内从35%稳步下降到8%。吉兰-巴勒综合征和其他肌肉疾病的患病率在各个时间点上也呈下降趋势，而其他疾病的患病率则更为不稳定。神经性疼痛的患病率仍然很高，并且在随访期间几乎没有变化（从急性期的31%到12个月时的25%）。结论：nmd及相关症状在COVID-19后很常见，但其总体患病率随时间而降低。然而，轨迹因NMD的类型或症状而异。

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引用次数: 0

Dear Editor, 亲爱的编辑,

IF 3.2 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Neurological Sciences

Pub Date : 2025-12-24 DOI: 10.1016/j.jns.2025.125707

Laura Mannismäki, Sami Curtze

引用次数: 0

Episodic encephalopathy in NOTCH2NLC-related neuronal intranuclear inclusion disease: Clinical spectrum and a proposed classification framework notch2nnc相关神经元核内包涵病的发作性脑病：临床谱和拟议的分类框架

IF 3.2 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Neurological Sciences

Pub Date : 2025-12-24 DOI: 10.1016/j.jns.2025.125706

Jingzhen Liang , Shaoping Zhong , Yangye Lian , Binbin Zhou , Yuzhe Li , Ying Liu , Kaiyuan Shen , Wei Sun , Yuwen Zhang , Jianying Liu , Xin Wang , Jing Ding

Objective

Episodic encephalopathy is an important yet underexplored manifestation of NOTCH2NLC-related neuronal intranuclear inclusion disease (NIID). Current studies are limited by small sample sizes and a lack of long-term follow-up, hindering a comprehensive understanding of its clinical and radiological features, which this study aims to address.

Methods

Patients diagnosed as NOTCH2NLC-related NIID were evaluated retrospectively and categorized into episodic encephalopathy-positive group (NIID-EP) and episodic encephalopathy-negative group (NIID-EN) depending on the presence of persistent disturbances of consciousness. Clinical information was collected and compared between the groups.

Results

This study evaluated 65 NIID patients, with a median onset age of 63 years and comprising 27 NIID-EP patients. Compared to NIID-EN, NIID-EP patients exhibited higher incidence of fever (p = 0.000), bladder dysfunction (p = 0.002), and abnormal behaviors (p = 0.001), and brainstem hyperintensities on MRI (p = 0.024). Focal imaging lesions such as cerebral edema, hyperperfusion, and cortical laminar necrosis were found in 5 patients that were classified as MELAS-like episodic encephalopathy and exhibited transhemispheric distribution and longitudinal changes of lactate peaks on magnetic resonance spectroscopy. Three NIID-EP patients were found to have hyperammonemia and ammonia-lowering therapy alleviated conscious disturbance rapidly. NIID-EP patients experienced more complications, prolonged hospitalizations, and worse modified Rankin Scale outcomes. A refined classification framework was proposed for paroxysmal events in NIID.

Significance

Episodic encephalopathy is a frequent, heterogeneous, and prognostically significant manifestation in NIID, driven by mechanisms such as inflammation, mitochondrial dysfunction, hyperammonemia, and may represent a shared clinical phenotype of genetic leukoencephalopathies with corticomedullary junction DWI hyperintensities.

目的：发作性脑病是notch2nnc相关神经元核内包涵病（NIID）的一个重要但尚未被充分探讨的表现。目前的研究受限于小样本量和缺乏长期随访，阻碍了对其临床和放射学特征的全面了解，本研究旨在解决这一问题。方法回顾性评估诊断为notch2nnc相关NIID的患者，并根据是否存在持续性意识障碍将其分为发作性脑病阳性组（NIID- ep）和发作性脑病阴性组（NIID- en）。收集两组患者的临床资料并进行比较。结果本研究评估了65例NIID患者，中位发病年龄为63岁，其中27例为NIID- ep患者。与NIID-EN相比，NIID-EP患者表现出更高的发热发生率（p = 0.000）、膀胱功能障碍发生率（p = 0.002）、行为异常发生率（p = 0.001）和脑干MRI高信号发生率（p = 0.024）。5例诊断为melas样发作性脑病的患者发现局灶性影像学病变，如脑水肿、高灌注、皮质层状坏死等，磁共振波谱显示出跨半球分布和乳酸峰的纵向变化。3例NIID-EP患者出现高氨血症，降氨治疗可迅速缓解意识障碍。NIID-EP患者出现更多并发症、住院时间延长和更差的修正兰金量表结果。提出了一种细化的NIID发作性事件分类框架。发作性脑病是NIID中一种常见的、异质性的、具有预后意义的表现，由炎症、线粒体功能障碍、高氨血症等机制驱动，并可能代表具有皮质-髓质交界处DWI高信号的遗传性白质脑病的共同临床表型。

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引用次数: 0

The world federation of neurology and its global engagement with the World Health Organization and the United Nations economic and social council 世界神经病学联合会及其与世界卫生组织和联合国经济及社会理事会的全球参与。

IF 3.2 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Neurological Sciences

Pub Date : 2025-12-24 DOI: 10.1016/j.jns.2025.125708

Alla Guekht , Ksenia Pochigaeva , Morris Freedman, Steven L. Lewis, Guy Rouleau, Chandrashekhar Meshram, Mohammad Wasay, Tissa Wijeratne, Wolfgang Grisold

The World Federation of Neurology (WFN) has increased efforts in global advocacy and promotion of neurology. These efforts formed the basis of global WFN activities between 2023 and 2025, concurring with worldwide interest in promoting brain health along with the dissemination of the World Health Organization (WHO) Intersectoral Global Action Plan on Epilepsy and Other Neurological Disorders (IGAP). To achieve these goals, the WFN leveraged the concept of advocacy, which allows promoting neurology at the macro- (global), meso- (regional), and micro-levels. Two main strategic WFN advocacy instruments are the World Brain Day and a global joint project with the American Academy of Neurology on advocacy and leadership. As a non-state actor, the WFN cooperates with the WHO through a special consultive status at the United Nations Economic and Social Council (UN ECOSOC) to promote neurology and brain health globally. The WHO and UN ECOSOC meetings from 2023 to 2025 promoted access to care and universal health coverage, brain health, capacity building, prevention of neurological diseases, the IGAP, and noncommunicable diseases, which are also the most frequently mentioned topics by WFN. The way forward is to promote neurology with the WHO and UN based on the Global Burden of Disease study, which reported on the substantial burden of neurological diseases, enhancing the importance of neurology in accordance with the IGAP.

世界神经病学联合会（WFN）加大了在全球倡导和促进神经病学的努力。这些努力构成了世界脑联在2023年至2025年期间全球活动的基础，与全世界对促进大脑健康的兴趣以及世界卫生组织（世卫组织）《癫痫和其他神经系统疾病部门间全球行动计划》的传播相一致。为了实现这些目标，世界神经联盟利用宣传的概念，允许在宏观（全球）、中观（区域）和微观层面促进神经病学。世界大脑联盟的两个主要战略宣传工具是世界大脑日和与美国神经病学学会关于宣传和领导的全球联合项目。作为一个非国家行为体，世界神经联盟通过在联合国经济及社会理事会（经社理事会）的特别咨商地位与世卫组织合作，在全球促进神经病学和大脑健康。世卫组织和联合国经社理事会在2023年至2025年期间举行的会议促进了获得保健和全民健康覆盖、大脑健康、能力建设、预防神经系统疾病、政府间发展目标和非传染性疾病，这些也是世界卫生大会最常提到的主题。今后的道路是在全球疾病负担研究报告的基础上，与世卫组织和联合国一起促进神经病学，该研究报告了神经系统疾病的重大负担，并根据IGAP加强神经病学的重要性。

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引用次数: 0

Corrigendum to ‘Response to letter to the editor (JOTNS-D-25-02717 by Dr. Nie Lei)’ [The Journal of the Neurological Sciences 2025 Volume 479 123752] 对“给编辑的回信（JOTNS-D-25-02717 by Dr. Nie Lei）”的更正[the Journal of the Neurological Sciences 2025 Volume 479 123752]。

IF 3.2 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Neurological Sciences

Pub Date : 2025-12-23 DOI: 10.1016/j.jns.2025.125702

C.S. Musaeus , H.S. Gleerup , F.K. Clemmensen , F. Sellebjerg , M.B. Hansen , H.B. Søndergaard , G. Waldemar , S.G. Hasselbalch , A.H. Simonsen

引用次数: 0

Effects of surgical therapies on cognitive function in patients with essential tremor 手术治疗对特发性震颤患者认知功能的影响

IF 3.2 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Neurological Sciences

Pub Date : 2025-12-20 DOI: 10.1016/j.jns.2025.125697

Vibhash D. Sharma , Diane S. Berry , Stephanie Cosentino , Elan D. Louis

Background

Essential tremor (ET) is associated with increased risk of cognitive decline, particularly with advancing age. Surgical therapies, such as deep brain stimulation (DBS) and MR-guided focused ultrasound (MRgFUS), are effective treatment options for tremor, but their long-term cognitive effects remain unclear. This is the first study to compare ET patients who underwent surgery to those who had not.

Objective

We assessed cognitive outcomes in ET patients who underwent surgical therapies and compared them to non-surgical controls.

Methods

We identified both surgical (n = 12) and non-surgical ET cases (n = 188) from a large prospective longitudinal study and compared their cognitive function. Cognitive function was assessed prior to and following surgery, over the course of 36- and 54-month intervals. Mixed model analyses of covariance were used to assess cognitive changes over time, controlling for age, global cognitive scores, years of education, and study visit.

Results

Although both groups showed modest cognitive decline over time, few significant differences were observed between the surgical and non-surgical groups. Significant group × time interactions were observed for several tests, with the surgical group demonstrating greater decline in some subdomains of Memory and Executive Function.

Conclusion

Surgical therapies such as DBS and MRgFUS do not appear to broadly impact global cognitive function in older ET patients. However, specific cognitive domains may be more susceptible to decline.

特发性震颤（ET）与认知能力下降的风险增加有关，尤其是随着年龄的增长。外科治疗，如深部脑刺激（DBS）和磁共振引导聚焦超声（MRgFUS），是治疗震颤的有效选择，但其长期认知效果尚不清楚。这是第一个比较接受手术和未接受手术的ET患者的研究。目的：我们评估接受手术治疗的ET患者的认知结局，并将其与非手术对照进行比较。方法我们从一项大型前瞻性纵向研究中选择手术（n = 12）和非手术（n = 188） ET病例，并比较他们的认知功能。在手术前后以36个月和54个月为间隔评估认知功能。使用协方差的混合模型分析来评估随时间的认知变化，控制年龄、整体认知评分、受教育年限和研究访问。结果虽然两组随着时间的推移都表现出适度的认知能力下降，但在手术组和非手术组之间没有观察到明显的差异。在几项测试中观察到显著的组与时间相互作用，手术组在记忆和执行功能的某些子域表现出更大的下降。手术治疗如DBS和MRgFUS似乎不会广泛影响老年ET患者的整体认知功能。然而，特定的认知领域可能更容易衰退。

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引用次数: 0

Serological testing patterns among individuals newly diagnosed with myasthenia gravis 重症肌无力患者的血清学检测模式

IF 3.2 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Neurological Sciences

Pub Date : 2025-12-20 DOI: 10.1016/j.jns.2025.125700

A. Pesa Jacqueline , N. Liberman Joshua , D. Darer Jonathan , Yang Xiaoyun , A. Jackson Louis , Sergenyenko Artem , Campbell Nolan , J. Nowak Richard

Serologic antibody testing supports myasthenia gravis (MG) diagnosis and informs appropriate treatment. The research objective was to evaluate real-world MG serostatus testing patterns in the United States. Using insurance claims linked to serological test results, a retrospective, observational cohort study was conducted among adults with newly diagnosed MG between 01-January-2018 and 30-June-2022. Serostatus tests included acetylcholine receptor (anti-AChR), muscle-specific kinase (anti-MuSK), and low-density lipoprotein receptor-related protein 4 (anti-LRP4) antibodies. Of 5788 cases, 2590 (44.7 %) had at least one valid serology test result: 1453 (56.3 %) seronegative, 1094 (42.4 %) anti-AChR+, 28 (1.1 %) anti-MuSK+, and 5 (0.2 %) anti-LRP4+. Among the seronegative, 56.6 % were only tested for anti-AChR antibodies, 33.7 % tested both anti-AChR and anti-MuSK antibodies, and 5.0 % tested all three antibodies. Of the seronegative and untested populations, 40.9 % and 37.4 % initiated ≥2 unique MG treatments, with 699 (69.4 %) and 1267 (64.7 %) initiating acetylcholinesterase inhibitor. Factors associated with a higher likelihood of obtaining serologic testing included diagnosis by neurology, presence of MG-associated symptoms, residence in large metropolitan areas, fewer comorbidities, and Medicare insurance (vs. commercial or Medicaid). Variation in MG serologic testing, including infrequent MuSK and LRP4 autoantibody testing among seronegative patients, highlights a potential need to promote best practices in patient care.

血清学抗体测试支持重症肌无力（MG）的诊断和告知适当的治疗。研究目的是评估美国真实世界MG血清状态检测模式。利用与血清学检测结果相关的保险索赔，在2018年1月1日至2022年6月30日期间对新诊断为MG的成年人进行了一项回顾性观察性队列研究。血清状态检测包括乙酰胆碱受体（抗achr）、肌肉特异性激酶（抗musk）和低密度脂蛋白受体相关蛋白4（抗lrp4）抗体。5788例患者中，2590例（44.7%）至少有一项有效血清学检测结果：血清阴性1453例（56.3%），抗achr + 1094例（42.4%），抗musk + 28例（1.1%），抗lrp4 + 5例（0.2%）。血清阴性患者中，仅检测抗achr抗体的占56.6%，同时检测抗achr和抗musk抗体的占33.7%，三种抗体均检测的占5.0%。在血清阴性和未检测的人群中，40.9%和37.4%的人开始了≥2种独特的MG治疗，其中699人（69.4%）和1267人（64.7%）开始使用乙酰胆碱酯酶抑制剂。与获得血清学检测的可能性较高相关的因素包括神经学诊断、mg相关症状的存在、居住在大城市地区、合并症较少以及医疗保险（与商业或医疗补助相比）。MG血清学检测的差异，包括在血清阴性患者中罕见的MuSK和LRP4自身抗体检测，突出了促进患者护理最佳实践的潜在需求。

{"title":"Serological testing patterns among individuals newly diagnosed with myasthenia gravis","authors":"A. Pesa Jacqueline , N. Liberman Joshua , D. Darer Jonathan , Yang Xiaoyun , A. Jackson Louis , Sergenyenko Artem , Campbell Nolan , J. Nowak Richard","doi":"10.1016/j.jns.2025.125700","DOIUrl":"10.1016/j.jns.2025.125700","url":null,"abstract":"<div><div>Serologic antibody testing supports myasthenia gravis (MG) diagnosis and informs appropriate treatment. The research objective was to evaluate real-world MG serostatus testing patterns in the United States. Using insurance claims linked to serological test results, a retrospective, observational cohort study was conducted among adults with newly diagnosed MG between 01-January-2018 and 30-June-2022. Serostatus tests included acetylcholine receptor (anti-AChR), muscle-specific kinase (anti-MuSK), and low-density lipoprotein receptor-related protein 4 (anti-LRP4) antibodies. Of 5788 cases, 2590 (44.7 %) had at least one valid serology test result: 1453 (56.3 %) seronegative, 1094 (42.4 %) anti-AChR+, 28 (1.1 %) anti-MuSK+, and 5 (0.2 %) anti-LRP4+. Among the seronegative, 56.6 % were only tested for anti-AChR antibodies, 33.7 % tested both anti-AChR and anti-MuSK antibodies, and 5.0 % tested all three antibodies. Of the seronegative and untested populations, 40.9 % and 37.4 % initiated ≥2 unique MG treatments, with 699 (69.4 %) and 1267 (64.7 %) initiating acetylcholinesterase inhibitor. Factors associated with a higher likelihood of obtaining serologic testing included diagnosis by neurology, presence of MG-associated symptoms, residence in large metropolitan areas, fewer comorbidities, and Medicare insurance (vs. commercial or Medicaid). Variation in MG serologic testing, including infrequent MuSK and LRP4 autoantibody testing among seronegative patients, highlights a potential need to promote best practices in patient care.</div></div>","PeriodicalId":17417,"journal":{"name":"Journal of the Neurological Sciences","volume":"480 ","pages":"Article 125700"},"PeriodicalIF":3.2,"publicationDate":"2025-12-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145840882","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

Global, regional and national burden of Parkinson's disease, 1990–2021: Update from the GBD 2021 study 1990-2021年全球、区域和国家帕金森病负担：GBD 2021研究的最新进展

IF 3.2 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Neurological Sciences

Pub Date : 2025-12-18 DOI: 10.1016/j.jns.2025.125703

Rong Yang , Mou Sun , Wen Chen , Hao Feng , Bing Chen , Yi Liu , Qiuguo He , Lin Wang , Chengong Zou , Xiaoquan Luo , Zhou Li , Anhui Fu , Fei Qiao , Hui Tang , Jing Yang , Haibo Ren

Background and objective

Parkinson's disease (PD) poses an escalating global health burden. This study evaluates PD burden, key drivers, and health system implications.

Methods

Using Global Burden of Disease 2021 data, we analyzed incidence, prevalence, mortality, and DALYs and their age-standardized rates (ASRs) for PD across 204 countries from 1990 to 2021, examining spatial and demographic patterns. Decomposition analysis identified burden drivers, and relationship between socio-demographic index (SDI) and health workforce density with DALYs were assessed.

Results

In 2021, PD caused 1.335 million (95 % UI: 1.200 to 1.490) incident cases, 11.767 million (95 % UI: 10.400 to 13.400) prevalent cases, 0.388 million (95 %UI: 0.347 to 0.417 million) deaths, and 7.472 million (95 %UI: 6.740 to 8.140 million) DALYs globally. ASR for all indicators increased significantly since 1990, with the Average Annual Percent Changes exceeding 0 for each (P > 0.01). Burden heterogeneity was marked, with the highest in East Asia. Males bear a higher overall burden, though females exceeded them above age 90. Population growth and aging were primary drivers. DALYs correlated non-linearly with SDI, and weakly with health workforce density (|r| < 0.20).

Conclusion

From 1990 to 2021, the burden of PD intensified globally, with notable spatial and demographic disparities, primarily driven by population growth and aging. The current alignment between healthcare workforce allocation and disease burden is weak, indicating that the health system requires targeted reinforcement of its response capabilities.

背景与目的：帕金森病（PD）造成了日益严重的全球健康负担。本研究评估帕金森病负担、主要驱动因素和卫生系统影响。方法：使用2021年全球疾病负担数据，我们分析了1990年至2021年204个国家PD的发病率、患病率、死亡率、DALYs及其年龄标准化率（ASRs），并检查了空间和人口模式。分解分析确定了负担驱动因素，并评估了社会人口统计指数（SDI）和卫生人力密度与DALYs之间的关系。结果：2021年全球PD发病133.5万例（95% UI: 1200 ~ 1490），流行1176.7万例（95% UI: 10400 ~ 13400），死亡338.8万例（95% UI: 0.347 ~ 0.417万），DALYs 747.2万例（95% UI: 6740 ~ 814万）。自1990年以来，各指标的ASR均显著增加，年均变化百分比均超过0 （P < 0.01）。负担异质性明显，东亚负担最重。男性的总体负担更高，但90岁以上的女性负担更重。人口增长和老龄化是主要驱动因素。结论：1990 - 2021年，PD负担在全球范围内呈加重趋势，且存在显著的空间和人口差异，主要受人口增长和老龄化的驱动。目前卫生保健人力配置与疾病负担之间的一致性较弱，这表明卫生系统需要有针对性地加强其应对能力。

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引用次数: 0

Clinical safety of ultra-high-dose methylcobalamin in patients with amyotrophic lateral sclerosis: Open-label extension of a phase 2/3 randomized controlled study 超高剂量甲基钴胺素治疗肌萎缩性侧索硬化症的临床安全性：一项2/3期随机对照研究的开放标签扩展

IF 3.2 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Neurological Sciences

Pub Date : 2025-12-18 DOI: 10.1016/j.jns.2025.125701

Ryuji Kaji , Yurie Nishi , Takayuki Ishida , Takao Takase , Tamotsu Ueda , Takaya Maeda , Yuishin Izumi , on behalf of the Methylcobalamin 762 study group

Objective

To develop combined therapies for amyotrophic lateral sclerosis (ALS), we investigated the long-term safety of ultra-high-dose methylcobalamin (50-mg intramuscular, twice weekly) in patients with advanced ALS.

Methods

As an open-label extension of a multicenter, randomized, double-blind, placebo-controlled phase 2/3 study, patients were enrolled and administered methylcobalamin 50 mg intramuscularly twice weekly for up to 52 weeks.

Results

In total, 144 patients (mean age, 62.1 years; 61.1 % male) were included, and the overall disease duration was 53.2 ± 17.9 months, with 85.4 % of patients having an ALS severity stage ≥3. The incidence of adverse events was 94.4 %, and adverse drug reactions occurred in 3.5 % of patients, which included proteinuria (2.1 %) and single cases of supraventricular arrhythmia, increased blood urea, and hypertension (0.7 % each). None led to discontinuation or death. The survival rate at 52 weeks was 85.7 %, and as shown for the following patient subgroups: by ALS severity (stage 1–2, 100 %; 3, 85.3 %; 4, 82.8 %; 5, 82.0 %) and by presence of tracheostomy (with, 88.8 %; without, 84.1 %). The median change in the ALS functional rating scale total score from baseline to 52 weeks was −1.0.

Conclusion

There were no particular safety issues as reported in the phase 2/3 study and no clear deterioration in survival rate or physical function when ultra-high-dose methylcobalamin was administered intramuscularly in patients with advanced ALS. This regimen could be a candidate for initial therapy with further add-on to overcome ALS in the future.

目的：为了开发治疗肌萎缩性侧索硬化症（ALS）的联合疗法，我们研究了超高剂量甲基钴胺素（50 mg肌肉注射，每周两次）在晚期ALS患者中的长期安全性。方法：作为一项多中心、随机、双盲、安慰剂对照2/3期研究的开放标签延伸，患者入组，每周两次肌肉注射甲钴胺50 mg，持续52周。结果：共纳入144例患者，平均年龄62.1岁，男性占61.1%，总病程53.2±17.9个月，85.4%的患者ALS严重程度≥3期。不良事件发生率为94.4%，药物不良反应发生率为3.5%，包括蛋白尿（2.1%）、单例室上性心律失常、尿素升高、高血压（0.7%）。没有一个导致停产或死亡。52周存活率为85.7%，以下患者亚组显示：ALS严重程度（1-2期，100%;3,85.3%;4,82.8%;5,82.0%）和存在气管切开术（有，88.8%；无，84.1%）。从基线到52周，ALS功能评定量表总分的中位数变化为-1.0。结论：在2/3期研究中没有报道的特别的安全性问题，在晚期ALS患者中肌肉注射超高剂量甲基钴胺素没有明显的生存率或身体功能恶化。该方案可能成为未来治疗渐冻症的初始治疗方案，并有进一步的附加治疗。

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引用次数: 0