Journal of Neurology最新文献_第7页

Initiation of tocilizumab within 24 hours in acute necrotizing encephalopathy is related to a good outcome: a systematic review. 急性坏死性脑病24小时内开始使用托珠单抗与良好的预后相关：一项系统评价。

IF 4.6 2区医学

Journal of Neurology Pub Date : 2025-09-07 DOI: 10.1007/s00415-025-13355-y

Yalin Ju, Chang Geng, Hongzhi Guan

{"title":"Initiation of tocilizumab within 24 hours in acute necrotizing encephalopathy is related to a good outcome: a systematic review.","authors":"Yalin Ju, Chang Geng, Hongzhi Guan","doi":"10.1007/s00415-025-13355-y","DOIUrl":"https://doi.org/10.1007/s00415-025-13355-y","url":null,"abstract":"Objective: To evaluate the clinical efficacy of tocilizumab, a interleukin-6 (IL-6) receptor blocker, for the treatment of acute necrotizing encephalopathy (ANE).Methods: PubMed, Cochrane Library, Embase, and Web of Science were searched for systematic review based on PRISMA guidelines. ANE patients treated with and without tocilizumab were included. Methodological quality was assessed independently by two authors. Data on clinical features, neuroimaging patterns and outcomes were analyzed.Results: In total, 77 cases from 21 studies were included. Most patients had fever and seizures. Respiratory viruses were common precipitating infection. All patients had bilateral thalamic lesions, and brainstem lesions were more frequently observed in the tocilizumab group (88.0% vs 64.0%, p = 0.04). The length of hospital stay was significantly longer in the tocilizumab group both before (p = 0.01) and after propensity score matching (PSM) (p = 0.02), while the mortality rate was significantly lower in tocilizumab compared with non-tocilizumab group (8.0% vs 33.3%, p = 0.02). Initiation of tocilizumab within 24 h was related to a good outcome both before (p < 0.01) and after PSM (p < 0.01). In multivariate regression analysis, elder age and < 24 h immunotherapy were independently related with good outcome (age: aOR = 1.35; 95% CI 1.06-1.74, p = 0.02; < 24 h immunotherapy: aOR = 8.60; 95% CI 1.08-68.63, p = 0.04). Brainstem lesions was an independent risk factor of poor outcome (aOR = 0.03; 95% CI 0.003-0.29, p < 0.01). Tocilizumab use was independently associated with reduced mortality (aOR = 7.95, 95%CI 1.47-43.03, p = 0.02). No adverse effects were reported.Conclusion: This review suggests that timely initiation of tocilizumab therapy within 24 h is safe, and may be an effective treatment in ANE, providing a strong rationale for a clinical trial.","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 9","pages":"618"},"PeriodicalIF":4.6,"publicationDate":"2025-09-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145015656","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"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

Case report: anti-argonaute antibodies in Epstein-Barr virus encephalitis. Epstein-Barr病毒性脑炎的抗argonaute抗体。

IF 4.6 2区医学

Journal of Neurology Pub Date : 2025-09-06 DOI: 10.1007/s00415-025-13331-6

Juyuan Pan, Dan Yu, Huihua Jiang, Ke Cui, Yuanlin Zhou

引用次数: 0

Low-grade persistent poliovirus infection in long-term polio survivors diagnosed with post-polio syndrome: diagnostic and clinical implications. 诊断为脊髓灰质炎后综合征的长期脊髓灰质炎幸存者中持续性低级别脊髓灰质炎病毒感染：诊断和临床意义

IF 4.6 2区医学

Journal of Neurology Pub Date : 2025-09-06 DOI: 10.1007/s00415-025-13364-x

Antonio Toniolo, Angelo Genoni, Giuseppe Maccari, Konstantin Chumakov, Fulvio Basolo, Giorgio Bono, Marco Mauri, Franco Molteni, Luisa Arrondini, Laura Bertolasi, Salvatore Monaco

{"title":"Low-grade persistent poliovirus infection in long-term polio survivors diagnosed with post-polio syndrome: diagnostic and clinical implications.","authors":"Antonio Toniolo, Angelo Genoni, Giuseppe Maccari, Konstantin Chumakov, Fulvio Basolo, Giorgio Bono, Marco Mauri, Franco Molteni, Luisa Arrondini, Laura Bertolasi, Salvatore Monaco","doi":"10.1007/s00415-025-13364-x","DOIUrl":"https://doi.org/10.1007/s00415-025-13364-x","url":null,"abstract":"Despite extensive research, the pathogenesis of Post-Polio Syndrome (PPS) remains unclear. We investigated 251 participants from Northern Italy: long-term polio survivors with PPS, long-term polio survivors with stable polio, family members of both groups, subjects with neurological disorders other than poliomyelitis, and healthy controls. This study investigated whether persistent viral activity or the existence of viral reservoirs contributes to causing PPS. Poliovirus (PV) genomes and proteins were detected in 87.2% of PPS cases versus 12.0% of stable polio cases and 3.5% of control family members, but not in pathologic and healthy controls. Among PPS patients, the highly concordant detection of PV strains in both peripheral blood leukocytes and cerebrospinal fluid (CSF) suggests the presence of an ongoing low-grade infection. Conversely, the very low detection rate in family members indicates the minimal transmissibility of these PV variants. Molecular analysis of the detected PV strains revealed mutations across most genome regions, likely leading to defects in virus replication. Furthermore, in cell cultures, PPS-derived PV strains induced the release of inflammatory mediators (IL6, IL8, MCP1) that may play a pathogenic role. These findings have several clinical implications. First, the presence of mutated PV forms in blood leukocytes and CSF could serve as a diagnostic marker for PPS. Second, the persistent virus infection suggests that antiviral treatments might help reduce PPS progression. Furthermore, advanced genome sequencing techniques hold potential for distinguishing vaccine-derived from wild-type PV strains, thereby refining our understanding of PPS and the full spectrum of polio disorders.","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 9","pages":"617"},"PeriodicalIF":4.6,"publicationDate":"2025-09-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145006262","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"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

Rethinking positional nystagmus: beyond BPPV. 重新思考体位性眼球震颤：超越BPPV。

IF 4.6 2区医学

Journal of Neurology Pub Date : 2025-09-05 DOI: 10.1007/s00415-025-13335-2

Amanda J Male, George Korres, Nehzat Koohi, Diego Kaski

引用次数: 0

Association between weekend recovery sleep and risk of incident dementia: a prospective cohort study in the UK Biobank. 周末恢复性睡眠与痴呆风险之间的关系：英国生物银行的一项前瞻性队列研究

IF 4.6 2区医学

Journal of Neurology Pub Date : 2025-09-05 DOI: 10.1007/s00415-025-13363-y

Bo Zhao, Shaojiong Zhou, Jie Chang, Aonan Li, Chaofan Geng, Tao Wei, Yiwei Zhao, Peiyang Gao, Zhibin Wang, Yi Tang

{"title":"Association between weekend recovery sleep and risk of incident dementia: a prospective cohort study in the UK Biobank.","authors":"Bo Zhao, Shaojiong Zhou, Jie Chang, Aonan Li, Chaofan Geng, Tao Wei, Yiwei Zhao, Peiyang Gao, Zhibin Wang, Yi Tang","doi":"10.1007/s00415-025-13363-y","DOIUrl":"https://doi.org/10.1007/s00415-025-13363-y","url":null,"abstract":"Background: Sleep deprivation has been linked to higher dementia risk, but the role of weekend recovery sleep (WRS) in mitigating this risk remains unclear. This study aims to evaluate the association between WRS and dementia risk.Methods: This prospective cohort study followed 88,592 dementia-free adults aged 40-79 years from the UK Biobank, using wrist accelerometers to measure average weekday and weekend sleep durations. Incident dementia (all-cause dementia, Alzheimer's disease [AD], vascular dementia [VaD], and nonspecific dementia) was determined using medical records. Associations were estimated using Cox proportional hazards models and restricted cubic splines (RCS).Results: Among 88,592 participants (mean [SD] age, 61.9 [7.9] years), 735 (0.83%) developed dementia, including 308 (0.35%) cases of AD, 137 (0.15%) cases of VaD, and 319 (0.36%) cases of nonspecific dementia. RCS analyses revealed optimal weekday sleep durations associated with the lowest dementia risk: 8.38 h (HR, 0.73; 95% CI 0.64-0.84) for all-cause dementia, 8.33 h (HR, 0.72; 95% CI 0.58-0.89) for AD, and 9.07 h (HR, 0.59; 95% CI 0.40-0.88) for VaD. In the suboptimal sleep group (weekday sleep less than optimal duration), longer WRS was associated with reduced risks of all-cause dementia (HR, 0.801; 95% CI 0.717-0.893) and VaD (HR, 0.747; 95% CI 0.612-0.91). However, in the prolonged sleep group (weekday sleep exceeding optimal duration), longer WRS was linked to an increased nonspecific dementia risk (HR, 1.291; 95% CI 1.087-1.533).Conclusion: Adequate WRS can reduce dementia risk, particularly for VaD, after insufficient weekday sleep, highlighting the importance of appropriate WRS for cognitive health.","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 9","pages":"612"},"PeriodicalIF":4.6,"publicationDate":"2025-09-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145000780","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"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

Syllable-based speech characteristics as potential biomarker for differential diagnosis of Parkinson's disease, multiple system atrophy, and cerebellar ataxia. 基于音节的言语特征作为帕金森病、多系统萎缩和小脑共济失调鉴别诊断的潜在生物标志物。

IF 4.6 2区医学

Journal of Neurology Pub Date : 2025-09-05 DOI: 10.1007/s00415-025-13352-1

Hyunsun Ham, Bora Jin, Kwang Su Cha, Kyung Ah Woo, Jung Hwan Shin, Han-Joon Kim

{"title":"Syllable-based speech characteristics as potential biomarker for differential diagnosis of Parkinson's disease, multiple system atrophy, and cerebellar ataxia.","authors":"Hyunsun Ham, Bora Jin, Kwang Su Cha, Kyung Ah Woo, Jung Hwan Shin, Han-Joon Kim","doi":"10.1007/s00415-025-13352-1","DOIUrl":"https://doi.org/10.1007/s00415-025-13352-1","url":null,"abstract":"Speech disorders differ between Parkinson's disease (PD) and multiple system atrophy (MSA), but studies focusing on group differences based on syllables or including cerebellar ataxia (CA) are lacking until now. This cross-sectional study aimed to analyze syllable-based speech characteristics in patients with PD, MSA, and CA, as well as healthy controls, to determine their diagnostic utility. Speech samples were collected from 68 PD, 52 MSA, 23 CA, and 70 healthy controls. Participants performed four speech tasks: producing high- and low-pitched sounds for five Korean vowels, repeating 14 Korean consonants with the vowel /a/, raising and lowering pitch of the vowel /a/, and continuously repeating /pa-ta-ka/ for 5 s. Acoustic analysis and artificial intelligence-based exploratory analysis were conducted to identify the syllable combinations that best distinguished between disease groups. Among the four speech tasks, the sequential motion rate task (/pa-ta-ka/ repetition) demonstrated the highest classification accuracy in distinguishing PD, MSA, and CA from the other groups, with accuracies of 68.90%, 77.42%, and 73.39%, respectively. For single syllable sequence, the /ka-ka-ka/ sequence achieved the highest accuracy, distinguishing CA from other groups with an accuracy of 78.92%. Among combined syllable sequence, the /aaa-hahaha/ sequence exhibited accuracies of 78.63% and 83.33% in differentiating PD and CA, respectively, while the /dadada-aaa/ sequence showed an accuracy of 80.24% in distinguishing MSA from other groups. These findings suggest that syllable-based speech characteristics, along with acoustic parameters, can discriminate among parkinsonian disorders and CA, highlighting their potential as a promising diagnostic tool.","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 9","pages":"613"},"PeriodicalIF":4.6,"publicationDate":"2025-09-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145006295","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"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

Alternating vertical nystagmus associated with Wernicke encephalopathy. 与韦尼克脑病相关的交替垂直眼震。

IF 4.6 2区医学

Journal of Neurology Pub Date : 2025-09-05 DOI: 10.1007/s00415-025-13370-z

Chenghao Li, Hyo-Jung Kim, Jeong-Yoon Choi, Ji-Soo Kim

引用次数: 0

Wilhelm Kleine (1897-1968). 威廉·克莱恩（1897-1968）。

IF 4.6 2区医学

Journal of Neurology Pub Date : 2025-09-05 DOI: 10.1007/s00415-025-13290-y

Axel Karenberg, Gilles Fénelon

引用次数: 0

Anti-AK5 limbic encephalitis in a patient with pleural mesothelioma. 胸膜间皮瘤患者的抗ak5边缘脑炎。

IF 4.6 2区医学

Journal of Neurology Pub Date : 2025-09-04 DOI: 10.1007/s00415-025-13340-5

Giulia Sofia Cereda, Antonio Farina, Nolwenn Billet, Macarena Villagrán-García, Anne-Laurie Pinto, Marie Benaiteau, Dorine Templement-Grangerat, Isabelle Treilleux, David Meyronet, David Laville, Géraldine Picard, Virginie Desestret, Jérôme Honnorat

引用次数: 0

IF 4.6 2区医学

Journal of Neurology Pub Date : 2025-09-04 DOI: 10.1007/s00415-025-13330-7

David Ellenberger, Niklas Frahm, Alexander Stahmann, Clemens Warnke, Kerstin Hellwig, Christoph Kleinschnitz, Peter Flachenecker, Michaela Mai, Matthias Grothe, Uwe K Zettl

{"title":"Age-related differences in switching highly effective disease-modifying therapy in patients with multiple sclerosis.","authors":"David Ellenberger, Niklas Frahm, Alexander Stahmann, Clemens Warnke, Kerstin Hellwig, Christoph Kleinschnitz, Peter Flachenecker, Michaela Mai, Matthias Grothe, Uwe K Zettl","doi":"10.1007/s00415-025-13330-7","DOIUrl":"https://doi.org/10.1007/s00415-025-13330-7","url":null,"abstract":"Background: High efficacy therapies (HET) play a crucial role in multiple sclerosis (MS) management. HET discontinuation/de-escalation is a critical decision, especially in different age groups, due to potential changes in relapse rates. We aimed at evaluating the impact of HET discontinuation on annualized relapse rates (ARRs) in people with MS (pwMS) aged ≥ 50 or < 50 years.Methods: We retrospectively analyzed data of 1,091 pwMS (German MS Register). ARR before and 12 months after the HET washout period were compared between older and younger patients for switching from HET to HET (H-H), HET to mild/moderate efficacy therapies (H-M) or HET to discontinuation (H-D). Reasons for therapy switches were assessed for all subgroups.Results: Most treatment switches continued with another HET (H-H n = 786), while de-escalation (H-M n = 86) or discontinuation (H-D n = 219) occurred less frequently. The minority within each switching group were ≥ 50 years of age (H-H 29%, H-M 22%, H-D 32%). ARR in H-H decreased after switching in both age groups (< 50: 0.19-0.12; ≥ 50: 0.17 to 0.09), increased in H-M < 50 (0.13-0.63) and remained stable in ≥ 50 (0.11-0.08), and increased in H-D < 50 (0.05-0.13) and remained stable in ≥ 50 (0.14-0.11). Main reason for therapy switch was lack of efficacy in H-H, adverse events in H-M regardless of age, patient's choice (23%) in H-D < 50, and lack of efficacy (26%) in H-D ≥ 50.Conclusions: Discontinuation strategies should be individualized, considering age-related changes in disease activity, adverse events and patient's choice.","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 9","pages":"609"},"PeriodicalIF":4.6,"publicationDate":"2025-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144992669","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"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