Journal of Neurology最新文献

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Management of cervical artery dissection: new evidence and future directions. 颈动脉夹层的治疗:新的证据和未来的方向。
IF 4.8 2区 医学
Journal of Neurology Pub Date : 2025-05-27 DOI: 10.1007/s00415-025-13166-1
Josefin E Kaufmann, Lukas Mayer-Suess, David Seiffge, Michael Knoflach, Stefan T Engelter, Christopher Traenka
{"title":"Management of cervical artery dissection: new evidence and future directions.","authors":"Josefin E Kaufmann, Lukas Mayer-Suess, David Seiffge, Michael Knoflach, Stefan T Engelter, Christopher Traenka","doi":"10.1007/s00415-025-13166-1","DOIUrl":"10.1007/s00415-025-13166-1","url":null,"abstract":"<p><p>Cervical artery dissection (CeAD) is a leading cause of ischemic stroke in young adults. Although its pathogenesis remains incompletely understood, advancements in CeAD patient care have been made in recent years. This review provides an updated overview of the latest evidence on hyperacute and (sub-)acute management of CeAD, highlighting aspects that have received limited attention, including vascular risk factors and mental health. Furthermore, we aim to outline future research directions to enhance patient outcomes and deepen our understanding of the disease.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 6","pages":"426"},"PeriodicalIF":4.8,"publicationDate":"2025-05-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12116624/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144150759","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Correction: Contribution of fatigue experienced by Parkinson's Disease patients on caregiver burden. 修正:帕金森病患者所经历的疲劳对照顾者负担的贡献。
IF 4.8 2区 医学
Journal of Neurology Pub Date : 2025-05-27 DOI: 10.1007/s00415-025-13109-w
Salvatore Landolfo, Daniele Urso, Carlo Santoro, Lucia Batzu, Valentina Gnoni, Alessia Giugno, Silvia Rota, Davide Vilella, Fabio Amati, Karolina Popławska-Domaszewicz, Stefano Giannoni-Luza, K Ray Chaudhuri, Giancarlo Logroscino
{"title":"Correction: Contribution of fatigue experienced by Parkinson's Disease patients on caregiver burden.","authors":"Salvatore Landolfo, Daniele Urso, Carlo Santoro, Lucia Batzu, Valentina Gnoni, Alessia Giugno, Silvia Rota, Davide Vilella, Fabio Amati, Karolina Popławska-Domaszewicz, Stefano Giannoni-Luza, K Ray Chaudhuri, Giancarlo Logroscino","doi":"10.1007/s00415-025-13109-w","DOIUrl":"https://doi.org/10.1007/s00415-025-13109-w","url":null,"abstract":"","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 6","pages":"424"},"PeriodicalIF":4.8,"publicationDate":"2025-05-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144150750","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
A practical guide to assessing functional motor weakness: a review of validated techniques. 评估功能性运动无力的实用指南:对经过验证的技术的回顾。
IF 4.8 2区 医学
Journal of Neurology Pub Date : 2025-05-27 DOI: 10.1007/s00415-025-13139-4
James Dolbow, Soheil El-Azzouni, Yiyi Zhang, Christopher Geiger
{"title":"A practical guide to assessing functional motor weakness: a review of validated techniques.","authors":"James Dolbow, Soheil El-Azzouni, Yiyi Zhang, Christopher Geiger","doi":"10.1007/s00415-025-13139-4","DOIUrl":"10.1007/s00415-025-13139-4","url":null,"abstract":"<p><p>Functional neurological symptom disorder, specifically, functional limb weakness, is a commonly seen condition in clinical neurological practice and requires careful examination for diagnosis. Specific and detailed examination techniques have been developed and validated over the past 100 years to help clinicians differentiate functional limb weakness from objective neurological weakness. These techniques vary in sensitivity, specificity, clinical application, and limitations. However, over time, the studied and/or validated forms of these exam techniques may have been lost and often not performed or taught in the way it was originally studied, thus decreasing the reliability of the examiner's findings. With many new examination techniques for functional limb weakness having been studied in recent years, it is important to not only review the form of each of these examination techniques, but also discuss the clinical applications, limitations, and utility of each. To date, there has been no comprehensive review demonstrating the exact form of all the studied and/or validated examination techniques for functional limb weakness and their proposed clinical utility. This review analyzes 9 examination techniques for functional limb weakness that have been studied for validity and provides readers with the exact technique of examination used to study each. It also outlines their sensitivity, specificity, clinical applications, and limitations to help clinicians accurately diagnose functional limb weakness.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 6","pages":"427"},"PeriodicalIF":4.8,"publicationDate":"2025-05-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12116663/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144150705","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Clinical course and long-term outcomes in autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy. 自身免疫胶质原纤维酸性蛋白(GFAP)星形细胞病的临床病程和长期预后。
IF 4.8 2区 医学
Journal of Neurology Pub Date : 2025-05-26 DOI: 10.1007/s00415-025-13159-0
Joaquín Arzalluz-Luque, Pauline Dumez, Géraldine Picard, Marie Benaiteau, Maxime Bonjour, Pierre Lardeux, Julian Theuriet, Florian Lamblin, Marie Rafiq, Jerome Honnorat, Romain Marignier
{"title":"Clinical course and long-term outcomes in autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy.","authors":"Joaquín Arzalluz-Luque, Pauline Dumez, Géraldine Picard, Marie Benaiteau, Maxime Bonjour, Pierre Lardeux, Julian Theuriet, Florian Lamblin, Marie Rafiq, Jerome Honnorat, Romain Marignier","doi":"10.1007/s00415-025-13159-0","DOIUrl":"10.1007/s00415-025-13159-0","url":null,"abstract":"<p><strong>Background: </strong>The aim was to describe the clinical course and long-term outcomes of the French cohort of patients with glial fibrillary acidic protein (GFAP) astrocytopathy.</p><p><strong>Methods: </strong>Patients with positive CSF GFAP antibody test were identified between May 2017 and February 2023. Those whose clinical presentation occurred < 2 years before the initiation of the study, with other diagnosis than GFAP astrocytopathy, and with missing clinical information were excluded.</p><p><strong>Results: </strong>Among the 74 patients included, 71 were alive at last follow-up. The median age at onset was 43 years (range 6-84), 44 patients were male (62%), and 11 (15%) had a neoplasia. The main initial syndrome was meningo-encephalitis (n = 41, 58%). The median follow-up was 28 months (range 1-129). The median mRS at presentation was 4 (range 1-5) and at last follow-up was 1 (range 0-4). Forty patients reported disability at last follow-up (56%). The most frequent sequelae were cognitive complaints (20/40, 50%) and gait disorder (19/40, 48%). 38/55 patients (69%) returned to school/work. Long-term immunoactive treatment was introduced in 40 patients (56%); the most commonly administered were oral corticosteroids (n = 35, 49%). Relapses were documented in 10 patients (14%) and occurred after a median follow-up of 9 months (range 3-46). The presence of concomitant tumor at onset was associated with relapse (HR 4.55, 95% CI 1.28-16.14, p = 0.03).</p><p><strong>Conclusions: </strong>This study suggests a greater impact than previously described in long-term outcomes of patients with GFAP astrocytopathy and reports concomitant tumor at presentation as a risk factor for relapse.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 6","pages":"421"},"PeriodicalIF":4.8,"publicationDate":"2025-05-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12106124/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144150749","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Acute peripheral unilateral vestibulopathy of the whole nerve causes increased impairment of spatial orientation and poorer long-term outcome. 急性周围单侧整个神经前庭病变导致空间定向损伤增加和远期预后较差。
IF 4.8 2区 医学
Journal of Neurology Pub Date : 2025-05-26 DOI: 10.1007/s00415-025-13160-7
Christoph Best, Heidrun H Krämer, Marianne Dieterich
{"title":"Acute peripheral unilateral vestibulopathy of the whole nerve causes increased impairment of spatial orientation and poorer long-term outcome.","authors":"Christoph Best, Heidrun H Krämer, Marianne Dieterich","doi":"10.1007/s00415-025-13160-7","DOIUrl":"10.1007/s00415-025-13160-7","url":null,"abstract":"<p><strong>Background: </strong>Acute peripheral unilateral vestibulopathy (UVP) is the third most common cause of peripheral vestibular vertigo. Etiologically, a viral inflammation is assumed. In most cases, an isolated dysfunction of the superior part of the vestibular nerve can be found (superior part UVP = sUVP), but an additional involvement of the inferior part has also been shown (whole nerve UVP = s+iUVP). The aim of the study was (a) to determine the prevalence of an additional inferior part involvement, (b) to quantify the extent of vestibular deficit comparing sUVP vs. s+iUVP and (c) to examine the long-term outcome focusing on psychological distress as well as long-lasting symptoms associated with dizziness.</p><p><strong>Methods: </strong>96 UVP patients were enrolled. They underwent a neuro-otological examination including measurements of cervical vestibular evoked myogenic potentials (cVEMP), subjective visual vertical (SVV), ocular torsion (OT), caloric testing and the clinical head impulse test (HIT) in the acute phase. The Symptom Checklist-90 R and the Vertigo Symptom Scale were examined at a mean follow-up interval of 4.0 years (± 0.4 years) after disease onset.</p><p><strong>Results: </strong>Among the 96 patients (47 female; mean age 58 ± 14 years), additional involvement of the inferior nerve part was found in 35 cases (36%). These patients showed a significantly greater tilt of SVV (6.3° ± 4.4° vs. 4.2° ± 3.7°; F = 5.581, p = 0.020) and a more pronounced OT (15.1° ± 8.2° vs. 11.3° ± 7.4°; F = 4.770, p = 0.032) in the acute stage of the disease. The proportion of pathological HIT was significantly higher in the s+iUVP group (82.9% vs. 67.2%; Chi-Square = 20.167, p < 0.001). cVEMPs showed significantly decreased amplitude on the affected side (124.8 µV (± 10.3 µV) vs. 408.4 µV (± 26.6 µV); F = 61.911; p < 0.001). At long-term follow-up, the patients with s+iUVP had significantly increased anxiety scores as compared to patients with isolated sUVP (SCL-90 score for anxiety: 48.4 ± 3.8 vs. 41.6 ± 0.5; F = 4.231, p = 0.026).</p><p><strong>Discussion: </strong>An additional lesion of the inferior part of the vestibular nerve led to increased vestibular dysfunction in acute UVP and might trigger long-lasting symptom persistence. Identifying these patients early might improve the clinical outcome, lead to a faster improvement and prevent secondary psychosomatic symptoms.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 6","pages":"422"},"PeriodicalIF":4.8,"publicationDate":"2025-05-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12106580/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144150707","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
The topography of infratentorial lesions in depression and anxiety in multiple sclerosis. 多发性硬化症伴抑郁和焦虑的幕下病变的地形图。
IF 4.8 2区 医学
Journal of Neurology Pub Date : 2025-05-26 DOI: 10.1007/s00415-025-13167-0
Federica Giofrè, Alessandra Lugaresi, Flavia Baccari, Elaine Lui, Stefanie Roberts, Charles Malpas, Tomas Kalincik
{"title":"The topography of infratentorial lesions in depression and anxiety in multiple sclerosis.","authors":"Federica Giofrè, Alessandra Lugaresi, Flavia Baccari, Elaine Lui, Stefanie Roberts, Charles Malpas, Tomas Kalincik","doi":"10.1007/s00415-025-13167-0","DOIUrl":"10.1007/s00415-025-13167-0","url":null,"abstract":"<p><strong>Background: </strong>Depression and anxiety are highly prevalent in multiple sclerosis and significantly impact patient outcomes. However, their link to specific areas of demyelination remains unclear.</p><p><strong>Objectives: </strong>This study examines the association between infratentorial lesions and depression or anxiety, focusing on three regions of interest: raphe nuclei, locus coeruleus, and cerebellar lobule VIIA.</p><p><strong>Methods: </strong>Patients were recruited from the cognitive neuroimmunology clinic at the Royal Melbourne Hospital. Participants were categorised as belonging to the groups 'depression'/'no depression' and 'anxiety'/'no anxiety' based on SPECTRA Indices of Psychopathology. MRI scans were examined for lesion presence in regions of interest. Association analyses were performed using multivariable logistic regression, adjusting for demographics, clinical parameters, and therapy.</p><p><strong>Results: </strong>Of 73 patients, 21 (29%) had clinically relevant depressive symptoms, and 18 (25%) had anxiety. Depression was significantly associated with lesions in the raphe nuclei (47.6% vs. 17.3%, OR 10.5, 95%CI 1.9-57.5, p=0.007) and locus coeruleus (38.1% vs. 15.4%, OR 20.5, 95%CI 2.3-184, p=0.007). Anxiety showed a potential association with locus coeruleus lesions (38.9% vs. 16.4%, OR 8.62, 95%CI 0.9-79.2, p=0.057).</p><p><strong>Conclusions: </strong>Depression in multiple sclerosis is associated with lesions within serotoninergic and noradrenergic brainstem nuclei. No definitive anatomical substrate for anxiety was identified. These findings suggest that inflammatory structural changes may underlie mood disorders in multiple sclerosis, potentially serving as early imaging markers of susceptibility to depression.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 6","pages":"420"},"PeriodicalIF":4.8,"publicationDate":"2025-05-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12106590/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144142795","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Cognitive manifestations and brain integrity in hereditary transthyretin amyloidosis: a systematic review. 遗传性甲状腺转蛋白淀粉样变的认知表现和脑完整性:一项系统综述。
IF 4.8 2区 医学
Journal of Neurology Pub Date : 2025-05-25 DOI: 10.1007/s00415-025-13120-1
Iara Senem, Rodrigo Melo Conde, Maria Paula Foss, Jan Axelsson, Jonas Wixner, Wilson Marques
{"title":"Cognitive manifestations and brain integrity in hereditary transthyretin amyloidosis: a systematic review.","authors":"Iara Senem, Rodrigo Melo Conde, Maria Paula Foss, Jan Axelsson, Jonas Wixner, Wilson Marques","doi":"10.1007/s00415-025-13120-1","DOIUrl":"https://doi.org/10.1007/s00415-025-13120-1","url":null,"abstract":"<p><strong>Background: </strong>Central Nervous System involvement in hereditary transthyretin amyloidosis (ATTRv) is present in liver transplanted patients with longstanding ATTRV30M amyloidosis, and in some rarer variants. The pathophysiology of brain involvement and its relationship with cognitive disturbances is unknown. This systematic review summarized the literature on brain and cognitive involvement in ATTRv amyloidosis and aimed to elucidate the reasons for such involvement.</p><p><strong>Methods: </strong>The literature search was performed using the following databases: Medline/PubMed, Embase via Elsevier, Scopus, and Web of Science. Two assessors independently screened titles and abstracts, examined full texts, extracted data, and assessed the risk of bias. The risk of bias assessment was carried out using the JBI critical appraisal tools. This review included studies that applied any neuroimaging exam or cognitive assessment in humans with genetic confirmation of any TTR mutation.</p><p><strong>Results: </strong>59 studies met the inclusion criteria. Overall, the studies were of good quality. 57 studies reported at least one brain MRI technique. Only six studies reported a formal neuropsychological assessment. The studies included 1218 ATTRv patients (mean 45.7 ± 11.8 years) and 169 asymptomatic TTR variant carriers (mean 30.6 ± 7.5 years). The most common TTR variant was V30M (n = 936), followed by V122I (n = 74). 42.4% of ATTRv patients presented abnormalities in the neuroimaging exam and 19.7% presented cognitive dysfunction.</p><p><strong>Conclusion: </strong>Based on the available evidence, brain involvement and cognitive symptoms can be present in ATTRv amyloidosis. Further research should explore the relationship of these symptoms with other complications (autonomic and cardiologic).</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 6","pages":"419"},"PeriodicalIF":4.8,"publicationDate":"2025-05-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144142793","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
Association between vascular risk factors burden and neurodegenerative diseases: results from ONDRI. 血管危险因素负担与神经退行性疾病之间的关系:来自ONDRI的结果。
IF 4.8 2区 医学
Journal of Neurology Pub Date : 2025-05-25 DOI: 10.1007/s00415-025-13152-7
Manuel Montero-Odasso, Frederico Pieruccini-Faria, Sandra E Black, Malcolm A Binns, Morris Freedman, David A Grimes, Robert A Hegele, Seyyed Hassan Haddad, Anthony E Lang, Mario Masellis, Jennifer Mandzia, Derek Beaton, Joel Ramirez, Angela C Roberts, William McIlroy, Stephen H Pasternak, Lorne Zinman, Agessandro Abrahao, Rick H Swartz, Sean Symons, Brian Tan, Carmela Tartaglia, Surim Son, Ryota Sakurai, Allison Dilliott, Benjamin F Cornish, Areej Hezam, Michael J Strong, Robert Bartha
{"title":"Association between vascular risk factors burden and neurodegenerative diseases: results from ONDRI.","authors":"Manuel Montero-Odasso, Frederico Pieruccini-Faria, Sandra E Black, Malcolm A Binns, Morris Freedman, David A Grimes, Robert A Hegele, Seyyed Hassan Haddad, Anthony E Lang, Mario Masellis, Jennifer Mandzia, Derek Beaton, Joel Ramirez, Angela C Roberts, William McIlroy, Stephen H Pasternak, Lorne Zinman, Agessandro Abrahao, Rick H Swartz, Sean Symons, Brian Tan, Carmela Tartaglia, Surim Son, Ryota Sakurai, Allison Dilliott, Benjamin F Cornish, Areej Hezam, Michael J Strong, Robert Bartha","doi":"10.1007/s00415-025-13152-7","DOIUrl":"https://doi.org/10.1007/s00415-025-13152-7","url":null,"abstract":"<p><strong>Background: </strong>Vascular risk factors are common in older adults and contribute to brain damage, can manifest as increased white matter hyperintensities (WMH), and associated with future risk of stroke and dementia. However, their prevalence, effect across different neurodegenerative diseases, and association with WMH remains underexplored.</p><p><strong>Objective: </strong>To investigate the association between vascular risk burden, and brain white matter integrity, across five neurodegenerative conditions.</p><p><strong>Methods: </strong>Cross-sectional study including 520 participants from the Ontario Neurodegenerative Disease Research Initiative (ONDRI) cohorts: 126 with amnestic Mild Cognitive Impairment/Alzheimer's Disease (MCI/AD), 53 with Frontotemporal Dementia (FTD), 161 with Cerebrovascular Disease (CVD), 140 with Parkinson's Disease (PD), and 40 with Amyotrophic Lateral Sclerosis (ALS), along with 41 cognitively healthy controls. A vascular risk index (VRI, range 0-5) assessed hypertension, diabetes, dyslipidemia, obesity (BMI ≥ 30), and smoking history. Macro (WMH volume) and micro (Diffusion tensor imaging) white matter integrity were evaluated using 3-Tesla MRI. Associations were analyzed using multinomial logistic regression and ANCOVA, adjusting for age, sex, education, and APOE ε4 allele status.</p><p><strong>Results: </strong>Vascular risk factors, particularly hypertension and hypercholesterolemia, were more prevalent in the disease cohorts than controls. A higher VRI was significantly associated with MCI/AD (1.5-fold, p = 0.05), FTD (1.7-fold, p =0 .02), and CVD (2.6-fold, p < 0.005) cohorts. High VRI was associated with reduced macro and microstructural white matter integrity in the pooled sample (macro: p = 0.005; micro: p = 0.003), and separately in CVD (macro: p = 0.04; micro: p = 0.002). APOE ε4 status only mildly attenuated these associations.</p><p><strong>Conclusion: </strong>Vascular risk burden is prevalent in neurocognitive syndromes including MCI/AD, FTD and CVD, and impacts white matter integrity. Future studies are needed to explore if vascular risk management may mitigate the consequences of neurodegeneration in these clinical groups.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 6","pages":"418"},"PeriodicalIF":4.8,"publicationDate":"2025-05-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144142792","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
Rituximab in stiff-person syndrome with glutamic acid decarboxylase 65 autoantibody: a systematic review. 利妥昔单抗治疗伴有谷氨酸脱羧酶65自身抗体的僵硬人综合征:一项系统综述。
IF 4.8 2区 医学
Journal of Neurology Pub Date : 2025-05-24 DOI: 10.1007/s00415-025-13157-2
Antonia Pignolo, Claudia Vinciguerra, Roberto Monastero, Nicasio Rini, Angelo Torrente, Carmela Rita Balistreri, Filippo Brighina, Vincenzo Di Stefano
{"title":"Rituximab in stiff-person syndrome with glutamic acid decarboxylase 65 autoantibody: a systematic review.","authors":"Antonia Pignolo, Claudia Vinciguerra, Roberto Monastero, Nicasio Rini, Angelo Torrente, Carmela Rita Balistreri, Filippo Brighina, Vincenzo Di Stefano","doi":"10.1007/s00415-025-13157-2","DOIUrl":"10.1007/s00415-025-13157-2","url":null,"abstract":"<p><strong>Background: </strong>Stiff-person syndrome (SPS) is a rare autoimmune neurological disorder characterized by muscle rigidity and painful spasms, predominantly affecting young women. It is often associated with high titers of anti-glutamic acid decarboxylase (GAD) 65 antibodies. Current treatments for SPS include symptomatic therapies and immunomodulatory approaches, but there is a need for more effective therapies because many patients show incomplete responses and disease progression.</p><p><strong>Methods: </strong>The systematic review followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, with a literature search of PubMed, Web of Knowledge, Google Scholar, and Science Direct. Studies evaluating efficacy, safety, dosage, and impact on concomitant treatments of Rituximab (RTX) in SPS were selected. Data on anti-GAD titers were also analyzed.</p><p><strong>Results: </strong>Fourteen studies published between July 2005 and October 2022 were selected. The studies included 30 SPS patients treated with RTX. Data were heterogeneous regarding dosage, administration schedule, and patient assessment. RTX was generally well-tolerated, with rare side effects, including infusion reactions or infections. Significant clinical improvement occurred in most patients, with a small proportion achieving complete remission. Anti-GAD antibody titers decreased in some studies, with no consistent correlation with clinical outcomes.</p><p><strong>Conclusions: </strong>Evidence supporting the efficacy of RTX in SPS is limited by the small sample sizes of the included studies and the variability in treatment protocols. However, RTX has shown efficacy for clinical improvement. Correlation with anti-GAD titers remains still unclear. Further randomized controlled trials are needed to confirm RTX as an established treatment for SPS.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 6","pages":"417"},"PeriodicalIF":4.8,"publicationDate":"2025-05-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12103359/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144142794","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Real-world observational study of infections in people treated with ocrelizumab for multiple sclerosis. ocrelizumab治疗多发性硬化症患者感染的真实世界观察性研究。
IF 4.8 2区 医学
Journal of Neurology Pub Date : 2025-05-22 DOI: 10.1007/s00415-025-13133-w
Laura Davies, Rasheed Shehadeh, W John Watkins, Stephen Jolles, Neil P Robertson, Emma C Tallantyre
{"title":"Real-world observational study of infections in people treated with ocrelizumab for multiple sclerosis.","authors":"Laura Davies, Rasheed Shehadeh, W John Watkins, Stephen Jolles, Neil P Robertson, Emma C Tallantyre","doi":"10.1007/s00415-025-13133-w","DOIUrl":"10.1007/s00415-025-13133-w","url":null,"abstract":"<p><strong>Background: </strong>Anti-CD20 monoclonal antibodies are now a common first-line treatment for multiple sclerosis (MS). Rituximab, ocrelizumab and ofatumumab have all been associated with a dose-dependent risk of hypogammaglobulinaemia, but its relevance in clinical practice remains uncertain.</p><p><strong>Objectives: </strong>To study infection rates over time in a real-world cohort of people treated with ocrelizumab for MS, and their relationship to serum immunoglobulin.</p><p><strong>Design: </strong>Observational study of 152 people receiving ocrelizumab for MS followed for up to 5.6 years (mean 2.7 years).</p><p><strong>Results: </strong>Mean (SD) annualized changes in immunoglobulins during ocrelizumab treatment were IgM - 0.22 g/L/year (0.4), IgG - 0.38 g/L/year (0.9), IgA - 0.03 g/L/year. Rates of self-reported infection increased significantly during the first 4 years of treatment. Infection rates were not only associated with total immunoglobulin levels but also independently associated with age, comorbidity and female sex. We demonstrated for the first time that 29 out of 34 (87%) people on ocrelizumab with IgG in the lower normal range had sub-protective antibody responses to pneumococcus / haemophilus influenzae.</p><p><strong>Conclusions: </strong>Real-world observational studies complement open label extensions of clinical trials, often by having a more representative cohort and more complete follow-up. Our data suggest that while serious infections are rare in people on ocrelizumab, non-serious infections become increasingly burdensome. We offer practical suggestions on mitigating the risk of infection on ocrelizumab and other anti-CD20 medications.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 6","pages":"415"},"PeriodicalIF":4.8,"publicationDate":"2025-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12098501/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144119902","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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