Journal of the Neurological Sciences最新文献

英文中文

The utility of Gold Coast criteria for amyotrophic lateral sclerosis 肌萎缩性侧索硬化症黄金海岸标准的应用

IF 3.2 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Neurological Sciences

Pub Date : 2026-01-06 DOI: 10.1016/j.jns.2026.125733

Emi Nomura , Ryuta Morihara , Yosuke Osakada, Taijun Yunoki, Mami Takemoto, Toru Yamashita, Hiroyuki Ishiura

Introduction

Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative disease. Current diagnostic criteria, including the revised El Escorial (rEE) and Awaji (AW) criteria, have limitations in sensitivity. The Gold Coast (GC) criteria were proposed to simplify diagnosis and improve early detection, but their real-world performance remains unclear.

Methods

We retrospectively analyzed 260 patients suspected of ALS who were admitted to our department between 2013 and 2022. The GC, AW, and rEE criteria were applied to data from initial hospitalization. Final diagnoses were based on follow-up data, and sensitivity/specificity were compared using McNemar's test.

Results

The GC criteria showed equivalent sensitivity (91.6 %), but higher specificity (75.9 %) compared to all combined AW and rEE categories. GC sensitivity was significantly higher than that of AW/rEE definite/probable categories. False negatives of GC criteria were often due to insufficient LMN signs, particularly in bulbar-onset cases. Subgroup analysis showed consistent trends.

Conclusion

The GC criteria demonstrated high sensitivity and moderate specificity, supporting their clinical utility in early ALS diagnosis. However, variability in clinical presentation and retrospective limitations suggest the need for further prospective validation.

肌萎缩性侧索硬化症（ALS）是一种快速进展的神经退行性疾病。目前的诊断标准，包括经修订的El Escorial （rEE）和Awaji （AW）标准，在敏感性上存在局限性。黄金海岸（GC）标准的提出是为了简化诊断和提高早期发现，但其实际表现尚不清楚。方法回顾性分析2013年至2022年我科收治的260例疑似ALS患者。GC、AW和rEE标准应用于首次住院的数据。最终诊断基于随访数据，使用McNemar试验比较敏感性/特异性。结果与AW和rEE联合分类相比，GC标准的敏感性为91.6%，特异度为75.9%。GC灵敏度显著高于AW/rEE确定/可能分类。GC标准的假阴性通常是由于LMN征象不足，特别是在球起病的病例中。亚组分析显示出一致的趋势。结论GC标准具有较高的敏感性和中等的特异性，支持其在ALS早期诊断中的临床应用。然而，临床表现的可变性和回顾性限制表明需要进一步的前瞻性验证。

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引用次数: 0

Use of the inactivated SARS-CoV-2 vaccine and the risk of Bell's palsy in China: A case-control study 中国使用SARS-CoV-2灭活疫苗与贝尔氏麻痹风险的病例对照研究

IF 3.2 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Neurological Sciences

Pub Date : 2026-01-06 DOI: 10.1016/j.jns.2026.125731

Tao Zhang , Heng Du , Yifu Wang , Yue Gu , Xiaoyan Song , Yijue Shen , Haiyan Lu , Kan Fang , Qimin Hu , Qiaoshu Wang , Guodong Wang

Background

Bell's palsy has been suggested as a possible adverse event of the inactivated SARS-CoV-2 vaccine. The aim of this study is to examine the association between the inactivated SARS-CoV-2 vaccine and Bell's palsy.

Methods

A large-scale muticenter case-control study carried out in two medical centers in Shanghai, China. We evaluated the risk of Bell's palsy after the inactivated SARS-CoV-2 vaccination. Patients with Bell's palsy admitted to the hospitals from February 1, 2021, to January 31, 2022 were recruited and controls were matched (1:2) by age, sex and admission date. Bell's palsy risk was evaluated as the proportion of patients exposed to the inactivated vaccine between groups using logistic regression to estimate the odds ratio (OR).

Results

Seven hundred and ninety-three Bell's palsy patients were matched to 1586 controls. The excess of Bell's palsy occurred post-vaccination compared with the controls, and the adjusted OR for exposure was 1.208 (95 % CI, 1.009–1.446; P = 0.040). The risk of Bell's palsy was more pronounced after the first vaccine dose (adjusted OR 1.603, 95 % CI, 1.105–2.325, P = 0.013) and most of the vaccines (51/58, 88 %) were administered within 60 days prior to Bell's palsy.

Interpretation

This study suggested that the inactivated SARS-CoV-2 vaccination is associated with an increased risk of Bell's palsy, and that this association appears to be more pronounced in patients after the first dose vaccination. However, potential residual confounding may exist in the study due to its retrospective nature.

贝尔麻痹已被认为是灭活SARS-CoV-2疫苗的可能不良事件。本研究的目的是研究灭活SARS-CoV-2疫苗与贝尔麻痹之间的关系。方法在上海两家医疗中心开展大规模多中心病例对照研究。我们评估了灭活SARS-CoV-2疫苗接种后贝尔麻痹的风险。研究招募了2021年2月1日至2022年1月31日住院的贝尔氏麻痹患者，对照组按年龄、性别和入院日期进行1:2匹配。使用logistic回归估计比值比（OR），以各组间暴露于灭活疫苗的患者比例来评估贝尔麻痹风险。结果793名贝尔氏麻痹患者与1586名对照者相匹配。与对照组相比，接种疫苗后贝尔麻痹发生率增加，暴露后校正OR为1.208 （95% CI, 1.009-1.446; P = 0.040）。Bell’s麻痹的风险在第一次接种疫苗后更为明显（调整后的OR为1.603,95% CI为1.105 ~ 2.325,P = 0.013），大多数疫苗（51/ 58,88 %）在Bell’s麻痹前60天内接种。本研究提示，灭活的SARS-CoV-2疫苗接种与贝尔麻痹风险增加相关，并且这种关联在首次接种疫苗后的患者中更为明显。然而，由于该研究是回顾性的，因此可能存在潜在的残留混淆。

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引用次数: 0

Evaluating ALSFRS-R as an indicator of disease milestones and functional independence: An observational study of US neurologists and their patients with amyotrophic lateral sclerosis 评估ALSFRS-R作为疾病里程碑和功能独立性的指标：美国神经学家及其肌萎缩侧索硬化症患者的观察性研究

IF 3.2 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Neurological Sciences

Pub Date : 2026-01-06 DOI: 10.1016/j.jns.2026.125732

Nicole F. Mehdiyoun , Jack Wright , Rebecca L. Robinson , Alexander U. Brandt , Margaret Hoyt , Jiaying Guo , Nathan Ball , Halima Iqbal , Ciara Ringland , Gary Milligan , Sarah E. Curtis

Background

The Revised Amyotrophic Lateral Sclerosis (ALS) Functional Rating Scale (ALSFRS-R) is a clinician-reported outcome measure monitoring disease progression in people living with ALS (pALS). This study examined the relationship of ALSFRS-R scores with disease progression and independence levels for activities of daily living (ADLs) among pALS.

Methods

Real-world data, including the ALSFRS-R, were drawn from a cross-sectional survey of US neurologists treating pALS (Adelphi ALS Disease Specific Programme™), conducted between July 2020 and March 2021. ALSFRS-R scores were modeled against 11 pre-defined disease milestones. The relationship between ALSFRS-R scores and levels of independence in 24 ADLs was examined using ordered logistic regression.

Results

Fifty-nine neurologists provided data for 379 pALS (mean age: 59.5 years; mean disease duration: 16.1 months). Estimated mean ALSFRS-R total score decreased (worsened) from 46.1 at first consultation regarding ALS symptoms to 25.1 upon receipt of a feeding tube. In general, pALS were likely to be completely dependent in most ADLs when their ALSFRS-R total scores were ≤ 25. A 1-point decrease in ALSFRS-R total score was associated with increased risks of losing independence across all ADLs. For each ADL, a 1-point decrease in domain score was associated with varying risks of losing independence across different domains.

Conclusions

There is a correlation between ALSFRS-R scores and levels of independence in ADLs among pALS, facilitating score interpretation for monitoring disease and function status. Yet, the relevance of the ALSFRS-R total score diminishes in advanced stages of ALS, indicating a need for additional measures to provide comprehensive evaluation.

背景：修订的肌萎缩性侧索硬化症（ALS）功能评定量表（ALSFRS-R）是一种临床报告的监测ALS （pALS）患者疾病进展的结果指标。本研究探讨了ALSFRS-R评分与pal患者疾病进展和日常生活活动独立水平的关系。方法：真实世界的数据，包括ALSFRS-R，来自于2020年7月至2021年3月期间对治疗pALS的美国神经科医生进行的横断面调查（Adelphi ALS Disease Specific program™）。ALSFRS-R评分根据11个预先定义的疾病里程碑进行建模。采用有序逻辑回归检验24例adl患者ALSFRS-R评分与独立性水平的关系。结果：59名神经科医生提供了379例pal的资料，平均年龄59.5岁，平均病程16.1个月。估计平均ALSFRS-R总分从首次咨询ALS症状时的46.1下降（恶化）到接受喂食管时的25.1。一般来说，大多数adl患者的ALSFRS-R总分≤25分时，pALS可能完全依赖。ALSFRS-R总分降低1分与所有ADLs中丧失独立性的风险增加相关。对于每个ADL，领域得分降低1分与不同领域失去独立性的不同风险相关。结论：ALSFRS-R评分与pALS中adl的独立性水平存在相关性，有助于对评分进行解释，以监测疾病和功能状态。然而，在ALS晚期，ALSFRS-R总分的相关性降低，这表明需要额外的措施来提供全面的评估。

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引用次数: 0

Variations in clinical features and disease burden of myasthenia gravis between racial and ethnic groups: A retrospective cohort study of two national databases 重症肌无力的临床特征和疾病负担在种族和民族之间的差异：两个国家数据库的回顾性队列研究

IF 3.2 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Neurological Sciences

Pub Date : 2026-01-05 DOI: 10.1016/j.jns.2026.125740

Nilay McLaren , Amaia Zurinaga Gutierrez , Daniel Joo , Bhaskar Roy , Minjee Park , Richard J. Nowak

Previous studies have reported that clinical characteristics and outcomes of myasthenia gravis (MG) vary across racial and ethnic groups. This study leverages two nationwide datasets, the MG Foundation of America Global MG Patient Registry (MGFAPR) and the National Inpatient Sample (NIS) to further examine these reports. We compared clinical characteristics, socioeconomic determinants, therapeutic regimens, and inpatient outcomes across racial/ethnic groups of MGFAPR enrollees between July 2013 and November 2024 that self-reported MG diagnosis by a physician and were US residents over the age of 18. Of 3744 patients, race was reported for: 3099 White, 178 African American (AA), 47 Asian, and 151 Indigenous patients; ethnicity was reported for: 188 Hispanic and 3431 non-Hispanic patients. AA and Hispanic patients had higher frequencies of early-onset MG and generalized symptoms; AA and Indigenous patients had higher MG-ADL and MG-QOL15r scores at enrollment; and White and Indigenous patients had higher frequencies of comorbid cancer. In the MGFAPR, women were 30 % less likely to be hospitalized and anxiety and/or depression was 300 % more common in hospitalized patients. In the NIS, we identified 62,055 hospitalizations for MG worsening from 2012 to 2019. AA and Hispanic MG patients had 1.4 and 1.7-times greater odds of intubation than White patients. Average cost of hospitalization was 150 % higher for AA patients than White patients. Overall, MG presented differently across racial/ethnic groups: we observed a greater disease burden in terms of ability to perform activities of daily life, quality of life, inpatient outcomes, and hospitalization costs in AA, Hispanic, and Indigenous patients.

先前的研究报道重症肌无力（MG）的临床特征和结果在不同种族和民族群体中存在差异。本研究利用两个全国性的数据集，美国MG基金会全球MG患者登记处（MGFAPR）和国家住院患者样本（NIS）来进一步检查这些报告。我们比较了2013年7月至2024年11月期间MGFAPR参与者的临床特征、社会经济决定因素、治疗方案和住院结果，这些参与者是18岁以上的美国居民，由医生自我报告MG诊断。在3744例患者中，种族报告为：3099例白人，178例非洲裔美国人（AA）， 47例亚洲人和151例土著患者；188名西班牙裔和3431名非西班牙裔患者报告了种族差异。AA和西班牙裔患者早发性MG和全身性症状的频率更高；AA和土著患者入组时MG-ADL和MG-QOL15r评分较高；白人和土著患者的合并症发病率更高。在MGFAPR中，女性住院的可能性降低了30%，住院患者中焦虑和/或抑郁的发生率增加了300%。在NIS中，我们确定了2012年至2019年因MG恶化住院的62,055例患者。AA和西班牙裔MG患者插管的几率分别是白人患者的1.4倍和1.7倍。AA患者的平均住院费用比White患者高150%。总体而言，MG在种族/民族群体中表现不同：我们观察到AA、西班牙裔和土著患者在日常生活活动能力、生活质量、住院结果和住院费用方面的疾病负担更大。

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引用次数: 0

Response to the letter to the editor “Myotonic dystrophy and cancer risk: Insights, limitations, and the need for genetic certainty” 对致编辑“肌强直性营养不良和癌症风险：洞察、限制和对基因确定性的需求”的回复

IF 3.2 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Neurological Sciences

Pub Date : 2026-01-02 DOI: 10.1016/j.jns.2025.125718

Bilal Hameed , Shahinaz M. Gadalla

引用次数: 0

Vestibulo-ocular reflex suppression: A practical guide for neurologists 前庭眼反射抑制：神经科医生的实用指南。

IF 3.2 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Neurological Sciences

Pub Date : 2026-01-02 DOI: 10.1016/j.jns.2025.125719

Juan Alcalá-Torres , Ana de la Torre Pérez , Pade Colligris Michelaraki

Vestibulo-ocular reflex suppression (VORS) testing remains an underutilized yet useful bedside tool for differentiating central and peripheral vestibular disorders in patients with dizziness. While most clinicians focus on the head impulse test (HIT) for detecting vestibular hypofunction, assessing the VORS can reveal central dysfunction, by observing compensatory saccades when turning their head while simultaneously following a moving object. Modern clinical practice incorporates visual-interactive smartphone-based methods for VORS assessment. This sign does not have a topographical value per se, although its impairment indicates a central lesion (frequently in flocculonodular cerebellum). It is absent in pure bilateral vestibulopathies. Regarding parkinsonism, VORS may be a diagnostic clue to distinguish between Multiple System Atrophy from other parkinsonian syndromes (89 % of sensitivity and specificity), although this fact is a component of a complete oculomotor examination. VORS must be integrated within the global clinical evaluation of the patient, as this alteration is less frequent than other cerebellar signs (i.e, gaze-evoked nystagmus or saccadic smooth pursuit). Here we present a practical review about VORS testing, according to pathophysiology, clinical and etiological aspects.

前庭-眼反射抑制（VORS）测试仍然是一种未充分利用但有用的床边工具，用于区分眩晕患者的中枢性和外周性前庭疾病。虽然大多数临床医生关注的是头部脉冲测试（HIT）来检测前庭功能障碍，但评估VORS可以通过观察他们在跟随移动物体时转动头部时的代偿性扫视来揭示中枢功能障碍。现代临床实践结合了基于智能手机的视觉交互式VORS评估方法。该征象本身不具有地形学意义，尽管其损害表明中心病变（常发生在小脑小叶结节）。在单纯的双侧前庭病变中不存在。对于帕金森病，VORS可能是区分多系统萎缩和其他帕金森综合征的诊断线索（89%的敏感性和特异性），尽管这一事实是完整的动眼肌检查的组成部分。VORS必须整合到患者的整体临床评估中，因为这种改变比其他小脑体征（即凝视诱发的眼球震颤或跳眼平滑追踪）更少发生。本文从病理生理学、临床和病因学等方面对VORS检测进行综述。

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引用次数: 0

Critique on “Severe cerebral small vessel disease burden is associated with intraventricular extension of intracerebral hemorrhage” 评“严重的脑血管疾病负担与脑出血脑室内延伸有关”。

IF 3.2 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Neurological Sciences

Pub Date : 2025-12-31 DOI: 10.1016/j.jns.2025.125715

Muhammad Muaz , Haniya Bilal

引用次数: 0

Reply to Critique on “Severe cerebral small vessel disease burden is associated with intraventricular extension of intracerebral hemorrhage” 批复“严重的脑血管疾病负担与脑出血脑室内延伸有关”。

IF 3.2 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Neurological Sciences

Pub Date : 2025-12-31 DOI: 10.1016/j.jns.2025.125713

Takeo Sato , Yuka Tsuchimochi , Yuki Hamada , Kaishi Kukihara , Yutaro Kawabata , Kana Iwamoto , Go Takaguchi , Yujiro Higuchi , Hideki Matsuoka , Hiroshi Takashima

引用次数: 0

Myotonic dystrophy and cancer risk: Insights, limitations, and the need for genetic certainty 强直性肌营养不良和癌症风险：洞察、限制和对基因确定性的需求

IF 3.2 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Neurological Sciences

Pub Date : 2025-12-30 DOI: 10.1016/j.jns.2025.125717

Christian Messina

引用次数: 0

Safety and efficacy of vigabatrin add on compared to placebo in Lennox-Gastaut syndrome (LennoVig): A single center randomized double-blind placebo-controlled trial 与安慰剂相比，vigabatrin在lenox - gastaut综合征（LennoVig）中的安全性和有效性增加：一项单中心随机双盲安慰剂对照试验。

IF 3.2 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Neurological Sciences

Pub Date : 2025-12-30 DOI: 10.1016/j.jns.2025.125712

Jayantee Kalita , Firoz M. Nizami , Ashish K. Dubey , Prakash C. Pandey , Alok P. Singh , Zafar Neyaz

Purpose

Lennox-Gastaut syndrome (LGS) is a developmental epileptic encephalopathy and is often refractory to antiseizure medication. We report the safety and efficacy of adjunctive vigabatrin compared to placebo in the patients with LGS in a randomized double-blind placebo-controlled trial.

Methods

Children aged 2 to 18 years with LGS were screened for eligibility and were observed for 4 weeks of baseline recording of different types of seizure and the Clinical Global Impression (CGI) scale. Patients were randomized to vigabatrin or placebo using simple 1:1 randomization. Outcome was defined at 12 weeks. The primary outcome was ≥50 % reduction in drop attacks, and secondary outcomes were 1) percentage change in drop attacks, 2) ≥50 % reductions in different seizure types, 3) number of seizure free days, 4) improvement in the CGI and 5) adverse events.

Results

Fifty patients each received vigabatrin and placebo. At 12 weeks, 8 patients were lost to follow-up (3 vigabatrin, 5 placebo). More patients in the vigabatrin group achieved the primary outcome than placebo (51.7 % vs 8.9 %; P < 0.001). Vigabatrin reduced all seizure types except generalized tonic-clonic seizures, reduced total number of seizures (−50 % vs 0 %; P < 0.001), and increased seizure-free days (4.6 vs 0.53; P = 0.02). The CGI scores improved significantly (P < 0.001). Adverse effects were similar, though sedation was more common with vigabatrin (P = 0.007).

Conclusions

In LGS, vigabatrin is effective in reducing drop attacks, and focal, myoclonic and atypical absence seizures along with improvement in the CGI.

Clinical Trial Registry of India, Indian Council of Medical Research (CTRI/2022/12/048517).

目的：lenox - gastaut综合征（LGS）是一种发展性癫痫性脑病，通常对抗癫痫药物难以治疗。在一项随机双盲安慰剂对照试验中，我们报告了与安慰剂相比，辅助维加巴林在LGS患者中的安全性和有效性。方法：筛选2 ~ 18岁LGS患儿，观察4周不同类型癫痫发作的基线记录和临床总体印象（CGI）量表。采用简单的1:1随机分配，将患者随机分配到维加巴林或安慰剂组。12周时确定结局。主要结局为跌落发作减少≥50%，次要结局为1)跌落发作百分比变化，2)不同发作类型减少≥50%，3)无发作天数，4)CGI改善和5)不良事件。结果：50例患者分别接受维加巴林和安慰剂治疗。12周时，8例患者失去随访（3例vigabatrin， 5例安慰剂）。结论：在LGS中，vigabatrin在减少跌落发作、局灶性、肌阵挛性和非典型性失神发作以及改善CGI方面是有效的。印度临床试验登记处，印度医学研究理事会（CTRI/2022/12/048517）。

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