Annals of Neurology最新文献

{"title":"From Topography to Therapy: Rethinking Cognitive Monitoring in GBA1-Linked Parkinson's Disease.","authors":"Meijun Liu","doi":"10.1002/ana.70007","DOIUrl":"https://doi.org/10.1002/ana.70007","url":null,"abstract":"","PeriodicalId":127,"journal":{"name":"Annals of Neurology","volume":" ","pages":""},"PeriodicalIF":7.7,"publicationDate":"2025-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144726180","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"From Data to Decision: Reinventing Hypoxic–Ischemic Encephalopathy Prognosis with Magnetic Resonance Imaging and Machine Learning","authors":"Yuyang Zheng MSc, Tao Fang MSc, Zhiqi Zhao PhD, Wei Peng PhD","doi":"10.1002/ana.70008","DOIUrl":"10.1002/ana.70008","url":null,"abstract":"","PeriodicalId":127,"journal":{"name":"Annals of Neurology","volume":"98 3","pages":"640-641"},"PeriodicalIF":7.7,"publicationDate":"2025-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144726179","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Volumetric MRI Comparing Longitudinal Change in MOGAD to NMOSD, MS and Healthy Controls, and Disability Associations.","authors":"Amy Kunchok, Moein Amin, Tatchaporn Ongphichetmetha, Mengke Du, Robert Bermel, Tucker Harvey, Justin R Abbatemarco, Stephen E Jones, Jeffrey A Cohen, Daniel Ontaneda, Kunio Nakamura","doi":"10.1002/ana.27309","DOIUrl":"https://doi.org/10.1002/ana.27309","url":null,"abstract":"<p><strong>Objectives: </strong>To examine volumetric magnetic resonance imaging (vMRI) in myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), compared to multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), and healthy controls (HC).</p><p><strong>Methods: </strong>Standardized vMRI in MOGAD were compared to age, sex, and disease duration matched MS (5:1), and non-matched NMOSD and HC, in mixed linear models with time and group interactions. Disability assessments included; patient determined disease steps (PDDS), manual dexterity (MDT), walking speed (WST), processing speed (PST), and contrast sensitivity tests (CST). Correlations between vMRI and disability in MOGAD were examined.</p><p><strong>Results: </strong>A total of 293 patients were included; 32 MOGAD, 160 MS, 49 NMOSD, and 52 HC. MOGAD had a faster rate of volume loss in all brain regions compared to HC (p < 0.05). At baseline, MOGAD had preserved thalamic (0.000507, p = 0.005), caudate (0.000287, p = 0.004), and putamen (0.000341, p = 0.007) fractions than MS. Longitudinally, MOGAD had increased lateral ventricle fraction (LVF) (-0.000645, p = 0.032), but less hippocampal (0.000031, p = 0.044) and upper cervical cord area (UCCA) loss (1.482887, p = 0.005), compared to NMOSD. MOGAD had increased LVF (0.00529, p < 0.001), but less UCCA loss (0.538656, p = 0.042) longitudinally compared to MS. Among MOGAD, UCCA was associated with PDDS (-0.72, p = 0.004) and CST (0.60, p = 0.014). Whole brain fraction (WBF) was associated with PDDS (-0.66, p = 0.01) and PST (0.52, p = 0.019). LVF was associated with PDDS (0.78, p < 0.001).</p><p><strong>Interpretation: </strong>MOGAD showed longitudinal brain volume loss compared to HC. Deep gray matter was relatively preserved compared to MS, while MS and NMOSD had greater UCCA loss, indicating regional differences in atrophy. Despite less overall atrophy, volume loss in MOGAD was associated with disability. ANN NEUROL 2025.</p>","PeriodicalId":127,"journal":{"name":"Annals of Neurology","volume":" ","pages":""},"PeriodicalIF":7.7,"publicationDate":"2025-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144726181","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Subthalamic Electrophysiological Mapping of Gait Initiation Dynamics and Freezing in Parkinson's Disease.","authors":"Antoine Collomb-Clerc, Mathieu Yeche, Adèle Demain, Angèle Van Hamme, Claire Olivier, Hayat Belaid, Déborah Ziri, Stéphane Derrey, Sara Fernandez-Vidal, Katia Lehongre, Carine Karachi, Brian Lau, Marie-Laure Welter","doi":"10.1002/ana.70003","DOIUrl":"https://doi.org/10.1002/ana.70003","url":null,"abstract":"<p><strong>Objective: </strong>The objective of this study was to investigate the relationships among subthalamic nucleus (STN) activity, gait initiation (GI), and freezing of gait (FOG) in patients with Parkinson's disease (PD).</p><p><strong>Methods: </strong>We recorded GI and STN local field potentials (LFPs) via externalized cables in 38 patients with PD (35 reporting FOG in daily life), both OFF- and ON-dopamine (DOPA). GI was also recorded in 24 age-matched controls. GI scores related to pace, rhythm, and balance were derived from kinetics, and FOG was identified using kinematics. We compared GI performance related to FOG and DOPA, and GI-LFP relationships between posterior-sensorimotor versus central-associative STN regions.</p><p><strong>Results: </strong>DOPA^OFF, 12 patients with PD had FOG observed (FOG-OBS, 263 episodes), occurring at a mean of 8 steps after GI, and 26 had no FOG observed (NOBS; FOG-NOBS). GI pace scores were worse in FOG-OBS than in FOG-NOBS patients with PD, even when not followed by FOG, with weaker association with alpha/low-beta activity in the posterior STN. Although rhythm/balance scores were similar between groups, their association with low-beta activity was stronger in FOG-OBS patients, with rhythm correlating more in the central STN and balance in the posterior STN. GI was worse preceding imminent FOG, with disrupted low-beta GI-LFP associations and emergent high-beta correlations with reversed spatial distribution (pace/rhythm-posterior STN, balance-central). Dopamine improved pace, rhythm, and FOG, and partially restored STN activity.</p><p><strong>Interpretation: </strong>Our results reveal 3 distinct GI patterns in patients with PD associated with absence, predisposition to, or imminent occurrence of FOG, with STN neuronal modulations differing dynamically between the posterior and central subregions. These markers could support developing adaptive DBS strategies tailored for episodic gait impairments. ANN NEUROL 2025.</p>","PeriodicalId":127,"journal":{"name":"Annals of Neurology","volume":" ","pages":""},"PeriodicalIF":8.1,"publicationDate":"2025-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144705873","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Reversible Aphasia from Tentorial DAVF with Thalamic Edema.","authors":"Xinrui Yu, Xi Yang, Xiaofeng Qu","doi":"10.1002/ana.70000","DOIUrl":"https://doi.org/10.1002/ana.70000","url":null,"abstract":"","PeriodicalId":127,"journal":{"name":"Annals of Neurology","volume":" ","pages":""},"PeriodicalIF":7.7,"publicationDate":"2025-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144937141","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Randomized, Double-Blind, Placebo-Controlled, Multiple-Dose Studies to Assess the Safety and Efficacy of Elezanumab when Added to Standard of Care in Relapsing and Progressive Forms of Multiple Sclerosis","authors":"Bruce A.C. Cree MD, PhD, MAS,&nbsp;Mark S. Freedman MD,&nbsp;Michael Gold MD,&nbsp;Kimberly Pfleeger PhD,&nbsp;Brittany Schwefel PhD,&nbsp;Annette Wundes MD,&nbsp;Adam Ziemann MD","doi":"10.1002/ana.27262","DOIUrl":"10.1002/ana.27262","url":null,"abstract":"<div>\u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>Elezanumab is a monoclonal antibody that binds repulsive guidance molecule a (RGMa), an inhibitor of central nervous system regeneration after inflammation or injury. The aim was to assess the safety and efficacy of elezanumab in relapsing and progressive forms of multiple sclerosis (MS).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>RADIUS-R and RADIUS-P were phase 2 trials in relapsing (RADIUS-R) or progressive (RADIUS-P) MS. Participants were randomized to intravenous elezanumab 400mg, 1800mg, or placebo every 4 weeks through week 48. The primary endpoint was the mean Overall Response Score (ORS).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>In RADIUS-R, 208 participants received elezanumab 400mg (n = 69), elezanumab 1800mg (n = 69), or placebo (n = 70). In RADIUS-P, 123 participants received elezanumab 400mg (n = 40), elezanumab 1800mg (n = 40), or placebo (n = 43). The primary endpoint of ORS was not met in either study. For RADIUS-R, mean ORS was −0.2 with effect size of −0.2 for elezanumab 400mg and −0.2 with effect size of −0.2 for elezanumab 1800mg. For RADIUS-P, mean ORS was 0.0 with effect size of 0.0 for elezanumab 400mg and 0.1 with effect size of 0.1 for elezanumab 1800mg.</p>\u0000 \u0000 <p>Elezanumab was well tolerated; the rate of serious adverse events was similar across treatment groups in both studies. Adverse events with ≥10% of elezanumab population were falls, urinary tract infections, headaches in RADIUS-R and RADIUS-P, and also fatigue, infusion-related reactions, and muscular weakness in RADIUS-P.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>Elezanumab was safe and well tolerated, but did not meet the primary endpoint in either study. ANN NEUROL 2025;98:590–602</p>\u0000 </section>\u0000 </div>","PeriodicalId":127,"journal":{"name":"Annals of Neurology","volume":"98 3","pages":"590-602"},"PeriodicalIF":7.7,"publicationDate":"2025-07-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/ana.27262","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144688457","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Soap Bubble Appearance in Cerebral Mucormycosis","authors":"Meiling Hu MM,&nbsp;Yu Tong MM,&nbsp;Kunyi Li MD,&nbsp;Lan Wen MD","doi":"10.1002/ana.27275","DOIUrl":"https://doi.org/10.1002/ana.27275","url":null,"abstract":"","PeriodicalId":127,"journal":{"name":"Annals of Neurology","volume":"98 3","pages":"636-637"},"PeriodicalIF":7.7,"publicationDate":"2025-07-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144910062","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Annals of Neurology: Volume 98, Number 2, August 2025","authors":"","doi":"10.1002/ana.26987","DOIUrl":"https://doi.org/10.1002/ana.26987","url":null,"abstract":"","PeriodicalId":127,"journal":{"name":"Annals of Neurology","volume":"98 2","pages":""},"PeriodicalIF":8.1,"publicationDate":"2025-07-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/ana.26987","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144666572","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Sternocleidomastoid Muscle Overactivity: A Potentially Critical Contributor to Postural Abnormalities in Parkinson's Disease.","authors":"Sha Zhu, Ronghua Hong, Zhuang Wu, Yunjun Bao, Yunping Song, Guojiong Hu, Zhongfei Bai, Feifei Zhu, Zhenhua Liao, Lizhen Pan, Qiang Guan, Zhuoyu Zhang, Lingjing Jin","doi":"10.1002/ana.27310","DOIUrl":"https://doi.org/10.1002/ana.27310","url":null,"abstract":"<p><strong>Objective: </strong>Overactivity of muscles is thought to be involved in postural abnormalities (PA) in Parkinson's disease (PD). Here, we investigated the relationship between muscle activity and postural parameters to explore the peripheral mechanisms of PA.</p><p><strong>Methods: </strong>A total of 90 PD patients and 19 healthy controls were enrolled. Posture features (F1-F8) and the index for PA were collected. Surface electromyography was acquired from cervical and thoracolumbar muscles during lying, sitting, standing, and walking, respectively. Follow-up was completed for a subset of PD patients.</p><p><strong>Results: </strong>Root mean square (RMS) amplitudes of the sternocleidomastoid muscle (SCM) and external oblique muscle, were higher in PD patients with PA (PD_PA) than PD patients without PA (PD_NPA) and healthy controls during lying and standing. Extensor activity in PD_PA patients increased only in the antigravity position compared with PD_NPA patients. Spearman's correlation showed that RMS amplitude of SCM in the lying position was associated with index for PA and forward flexion angles including F3-F8, whereas RMS amplitude of the external oblique muscle in the lying position was correlated with F5 alone. Longitudinal analysis showed that changes in F3, F4, and index for PA were significantly correlated with changes in RMS amplitude of the SCM in the lying position. Additionally, PD_NPA patients with SCM overactivity had a significantly higher risk of progressing to PA than those with normal SCM activity.</p><p><strong>Interpretation: </strong>The SCM and external oblique muscle are involved in forward trunk flexion in PD patients, while extensors may play a compensatory role. SCM may be the key muscle in PA for PD patients, participating in the pathogenesis of PA. ANN NEUROL 2025.</p>","PeriodicalId":127,"journal":{"name":"Annals of Neurology","volume":" ","pages":""},"PeriodicalIF":8.1,"publicationDate":"2025-07-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144641318","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Sex Differences in Associations of Lewy Body Disease with Alzheimer's Disease and Cognitive Decline.","authors":"Madeline Wood Alexander, D Luke Fischer, Lawren VandeVrede, Emma Nichols, Lei Yu, James R Pike, Walter Swardfager, Che-Yuan Wu, Mario Masellis, Liisa A M Galea, Katherine Zukotynski, Gillian Einstein, Sandra E Black, Lisa L Barnes, Julie A Schneider, Zoe Arvanitakis, Kaitlin B Casaletto, Jennifer S Rabin","doi":"10.1002/ana.27308","DOIUrl":"10.1002/ana.27308","url":null,"abstract":"<p><strong>Objective: </strong>To investigate how sex and age at menopause influence the interplay between Alzheimer's disease (AD) and Lewy body disease (LBD) neuropathologies, and their associations with cognitive decline.</p><p><strong>Methods: </strong>We analyzed data from: (1) three Rush Alzheimer's Disease Center cohorts (i.e., the Religious Orders Study, Rush Memory and Aging Project, and Minority Aging Research Study), and (2) the National Alzheimer's Coordinating Center Neuropathology Data Set. Neuropathological evaluation assessed LBD (neocortical/limbic-type vs none) and AD, including neuritic plaques (β-amyloid plaques surrounded by dystrophic neurites) and neurofibrillary tangles. In each dataset, we tested interactive associations between LBD and sex on neuritic plaques, neurofibrillary tangles, and cognitive decline. Additionally, in the Rush dataset, we tested whether age at spontaneous menopause modified the associations of LBD with neuritic plaques, neurofibrillary tangles, and cognitive decline in women.</p><p><strong>Results: </strong>In the Rush dataset, we included 1,277 women and 579 men. In the National Alzheimer's Coordinating Center dataset, we included 3,283 women and 3,563 men. Across both datasets, men were more likely to have LBD, whereas women showed greater neuritic plaque and neurofibrillary tangle burdens. Sex modified the associations of LBD with neurofibrillary tangles (but not neuritic plaques), whereby LBD was more strongly associated with greater neurofibrillary tangle burden in women than men. Men showed faster LBD-related cognitive decline, whereas women showed faster neurofibrillary tangle-related decline, after adjusting for copathologies (neuritic plaques, neurofibrillary tangles, and LBD, as appropriate). In women, earlier age at menopause exacerbated the associations of LBD with neurofibrillary tangle burden and episodic memory decline.</p><p><strong>Interpretation: </strong>Sex may influence AD and LBD neuropathologies, highlighting the need for precision approaches to dementia prevention and intervention. ANN NEUROL 2025.</p>","PeriodicalId":127,"journal":{"name":"Annals of Neurology","volume":" ","pages":""},"PeriodicalIF":7.7,"publicationDate":"2025-07-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12341763/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144615592","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}