Neurology最新文献

{"title":"Clinical Reasoning: A 68-Year-Old Man With Progressive Numbness, Vertigo, and Cognitive Decline.","authors":"Stephanie Marie Regan, Long F Davalos","doi":"10.1212/WNL.0000000000213437","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213437","url":null,"abstract":"<p><p>A 68-year-old man presented with subacute progressive numbness in his lower extremities and vertigo. Concurrently, he developed behavioral and cognitive changes. Initial examination demonstrated mild distal symmetric weakness in the lower extremities, allodynia in the feet, decreased proprioception at the ankles, and absent reflexes in the lower extremities. Electrodiagnostic studies showed a length-dependent, severe axonal sensorimotor polyneuropathy. He was hospitalized after experiencing daily falls and worsening executive dysfunction, during which time additional cognitive history and examination were completed. MRI brain revealed diffusion restriction in the bilateral caudate nuclei. Ultimately, CSF testing solidified the clinical diagnosis. This case highlights the differential diagnosis and diagnostic approach for rapidly progressive neuropathies associated with cognitive decline.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 5","pages":"e213437"},"PeriodicalIF":7.7,"publicationDate":"2025-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143365343","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":"Teaching Video NeuroImage: Unraveling the Enigma of Ocular Flutter in Anti-GD1b and Anti-GT1a Rhombencephalitis.","authors":"Yao Qin, Zhihong Xu, Xingyu Zhuang, Kunqian Ji","doi":"10.1212/WNL.0000000000213431","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213431","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 5","pages":"e213431"},"PeriodicalIF":7.7,"publicationDate":"2025-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143365366","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":"The Use of Blood-Based Proteomic Biomarkers for the Diagnosis of Sports-Related Concussion.","authors":"Daniel Whitehouse, Virginia Newcombe","doi":"10.1212/WNL.0000000000213397","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213397","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 5","pages":"e213397"},"PeriodicalIF":7.7,"publicationDate":"2025-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143370808","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":"Electrographic and Clinical Determinants of Good Outcome After Postanoxic Status Epilepticus.","authors":"Marjolein M Admiraal, Sofia Backman, Martin Annborn, Ola Borgquist, Josef Dankiewicz, Joachim Düring, Stephane Legriel, Gisela Lilja, Hans Lindehammer, Niklas Nielsen, Andrea O Rossetti, Johan Undén, Tobias Cronberg, Erik Westhall","doi":"10.1212/WNL.0000000000210304","DOIUrl":"https://doi.org/10.1212/WNL.0000000000210304","url":null,"abstract":"<p><strong>Background and objectives: </strong>Postanoxic electrographic status epilepticus (PSE) affects up to a third of all comatose patients after cardiac arrest (CA) and is associated with high mortality. Late PSE onset (>24 hours), from a restored continuous background pattern, and absence of established indicators of poor outcome at multimodal prognostication are described in survivors. We aimed to determine the increase in probability of good long-term outcome after PSE in patients presenting with this favorable PSE profile compared with all patients with PSE.</p><p><strong>Methods: </strong>This is a prospective observational substudy of the international Targeted Hypothermia vs Targeted Normothermia After Out-of-Hospital Cardiac Arrest trial (TTM2-trial, 2017-2020) including adult comatose patients resuscitated from CA with continuous EEG (cEEG) monitoring. EEG background pattern and type of PSE were determined using standardized EEG terminology of the American Clinical Neurophysiology Society, blinded to clinical data. On day 4, multimodal prognostication was performed according to the European postresuscitation guidelines. Good outcome was defined as a modified Rankin Scale score of 0-3 at 6 months. Detailed follow-up was performed at 6 and 24 months.</p><p><strong>Results: </strong>A total of 191 patients were monitored with cEEG, of whom 52 (27%) developed possible or definite PSE at a median of 42 hours [IQR 32-46] after CA. The median age was 70 (IQR 63-77) years, and 35% were female. Favorable PSE profile was present in 20 patients (38%), of whom 12 patients (60%) survived until 6 months and 8 (40%) had good outcome; thus, the probability of good outcome increased 2.7 times. All patients lacking a favorable PSE profile had poor outcome. All patients with good outcome obeyed commands within the first 7 days. At 24 months, all 12 survivors were still alive and 7 had good functional outcome. Detailed follow-up at 24 months showed that most had only mild cognitive impairment and overall life satisfaction was similar to the general population.</p><p><strong>Discussion: </strong>PSE is compatible with good outcome when onset is late and from a continuous background and no established indicators of poor outcome are present. One-third of patients with PSE had favorable PSE profile, of whom well over a third eventually had good outcome and showed improved level of consciousness within the first week.</p><p><strong>Trial registration information: </strong>ClinicalTrials.gov Identifier: NCT02908308.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 5","pages":"e210304"},"PeriodicalIF":7.7,"publicationDate":"2025-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143399691","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":"Reader Response: A Preponderance of Evidence: A Call for a Randomized Trial in Posterior Circulation Stroke.","authors":"Davide Strambo, Simon Nagel, Patrik Michel","doi":"10.1212/WNL.0000000000209827","DOIUrl":"https://doi.org/10.1212/WNL.0000000000209827","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 5","pages":"e209827"},"PeriodicalIF":7.7,"publicationDate":"2025-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143071050","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":"Teaching Video NeuroImage: Globe Retraction in Duane Syndrome.","authors":"Ruben Jauregui, Scott N Grossman","doi":"10.1212/WNL.0000000000213409","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213409","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 5","pages":"e213409"},"PeriodicalIF":7.7,"publicationDate":"2025-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143071055","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":"Severe Relapse After Switching From Eculizumab to Satralizumab in Neuromyelitis Optica Spectrum Disorder.","authors":"Elisabeth Maillart, Anne-Laure Dubessy, Natalia Shor, Mathilde Piljan, Rene Decombe, Catherine Lubetzki, Bruno Stankoff, Romain Marignier, Ysoline Beigneux","doi":"10.1212/WNL.0000000000213399","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213399","url":null,"abstract":"<p><strong>Objectives: </strong>Several treatments have been approved for the prevention of attacks in AQP4-IgG+ neuromyelitis optica spectrum disorder (NMOSD). However, because of the rarity of the disease, little is known concerning how to switch from one treatment to another in case of lack of effectiveness or side effects. In this article, we report a severe attack in a patient with NMOSD after switching from eculizumab to satralizumab.</p><p><strong>Methods: </strong>A 44-year-old woman with NMOSD was treated with azathioprine and then rituximab, without optimal control of the disease. Eculizumab was initiated with clinical efficacy. Two years later, after the onset of rheumatoid arthritis and because of difficult venous access, a switch to satralizumab was proposed.</p><p><strong>Results: </strong>After switching, a severe attack occurred 11 weeks after the last eculizumab infusion. Severe tetraplegia was related to a new extensive cervical lesion associated with a tumefactive lesion of the corpus callosum. The patient was treated with 10 infusions of methylprednisolone and 10 plasma exchanges. Eculizumab was reintroduced 20 days after symptom onset. Three months later, mild improvement was observed.</p><p><strong>Discussion: </strong>In clinical practice, in case of intolerance or side effects, anticomplement therapy should be switched to another NMOSD treatment with caution because of a high risk of relapse.</p><p><strong>Classification of evidence: </strong>This case report provides Class IV evidence that eculizumab should be stopped with caution and switched to another treatment immediately. This is a single observational study without controls.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 5","pages":"e213399"},"PeriodicalIF":7.7,"publicationDate":"2025-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143365365","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":"Association of Rheumatoid Arthritis With Progression of Cognitive Impairment and Risk of Mortality in People With Dementia.","authors":"Minjia Mo, Maria Eriksdotter, Sofia Ajeganova, Sumonto Mitra, Sara Garcia-Ptacek, Hong Xu","doi":"10.1212/WNL.0000000000213405","DOIUrl":"10.1212/WNL.0000000000213405","url":null,"abstract":"<p><strong>Background and objectives: </strong>Rheumatoid arthritis (RA) has been linked to an increased risk of dementia, yet little is known about how RA affects the progression of cognitive impairment and the risk of mortality in people with dementia. We aimed to investigate whether RA is linked to an accelerated cognitive decline and a higher risk of all-cause mortality in patients with dementia.</p><p><strong>Methods: </strong>We conducted a propensity score-matched register-based cohort study based on the Swedish Registry for Cognitive/Dementia Disorders-SveDem. Patients diagnosed with dementia and registered in SveDem between May 1, 2007, and October 16, 2018, were included. The main outcome for the study was cognitive decline, measured by Mini-Mental State Examination (MMSE) score changes over years. The secondary outcome was all-cause death. We used mixed-effects models to examine the association between RA and cognitive decline, and Cox proportional hazards models to investigate the risk of all-cause mortality. We also conducted subgroup analyses to explore the potential effects of sociodemographic, baseline MMSE, comorbidities, and the use of dementia medications on the association between RA and outcomes.</p><p><strong>Results: </strong>We included 1,685 dementia patients with RA (mean [SD] age, 79.9 [6.7] years; 73.4% were women) and 5,055 dementia patients with non-RA (80.1 [7.5] years; 73.1% were women). The median follow-up was 2.9 years (interquartile range, 1.5-4.6 years) for non-RA and 2.6 years (interquartile range, 1.4-4.2 years) for RA. In total, 111,266 MMSE measurements were available for analysis. Compared with non-RA patients, patients with RA presented faster cognitive decline (β = -0.24 points/y; 95% CI -0.38 to -0.10) and an increased risk of death (hazard ratio 1.15; 95% CI 1.06-1.24). In subgroup analysis, significant interactions were observed between RA and baseline MMSE scores as well as living conditions regarding cognitive decline (<i>p</i> for interaction <0.05).</p><p><strong>Discussion: </strong>We identified a worse cognitive function and an increased mortality risk in dementia patients with RA compared with non-RA. However, we lacked information on the duration of RA before the onset of dementia and on disease activity, which could influence our findings. Further studies are needed to validate these results in comparable populations.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 5","pages":"e213405"},"PeriodicalIF":7.7,"publicationDate":"2025-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11810133/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143370805","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":"Reader Response: Functional Outcome and Hemorrhage Rates After Bridging Therapy With Tenecteplase or Alteplase in Patients With Large Ischemic Core.","authors":"Qian Feng","doi":"10.1212/WNL.0000000000209857","DOIUrl":"https://doi.org/10.1212/WNL.0000000000209857","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 5","pages":"e209857"},"PeriodicalIF":7.7,"publicationDate":"2025-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143256179","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":"Consensus Recommendations to Establish Reporting Standards in fMRI of Migraine: A Delphi Study.","authors":"Severin Schramm, Corinna Börner-Schröder, Miriam Reichert, Constanze Ramschütz, Xiao Michelle Androulakis, Messoud Ashina, Gianluca Coppola, Brett Cucchiara, Zhao Dong, Xiaoxia Du, Laura H Fischer-Schulte, Peter J Goadsby, Rune Häckert Christensen, Luke A Henderson, Anders Hougaard, Jian-Ren Liu, Gabriella Juhasz, Nazia Karsan, Jian Kong, Jeungchan Lee, Mi Ji Lee, Clas Linnman, Vani Mathur, Arne May, Jan Mehnert, Eric Moulton, David M Niddam, Jean Schoenen, David A Seminowicz, Anne Stankewitz, Yiheng Tu, Dániel Veréb, Tao Yin, Claus Zimmer, Florian Heinen, Thomas Baum, Michaela V Bonfert, Nico Sollmann","doi":"10.1212/WNL.0000000000210235","DOIUrl":"https://doi.org/10.1212/WNL.0000000000210235","url":null,"abstract":"<p><strong>Background and objectives: </strong>Migraine is a multifaceted primary headache disorder. In neuroimaging of migraine, fMRI has been used to elucidate pathophysiology or monitor treatment effects. The current literature, however, is highly heterogeneous regarding reported variables and methodologies. This begets a lack of comparability and complicates synthesis of results across studies. We developed a framework for standardized reporting of fMRI studies in migraine.</p><p><strong>Methods: </strong>Experts on fMRI in migraine were identified from the literature and subjected to structured questionnaires in 2 iterations of 3 rounds according to the DELPHI method. A total of 157 statements across 17 reporting domains were rated on 5-point Likert scales (strong support to strong opposition). The first iteration covered demographic data, migraine-specific factors, medication, scan timing, healthy controls (HCs), participant sampling/recruiting, standardized forms, study preregistration, region of interest (ROI) analyses, validation data sets, data sharing, preprocessing documentation, and analysis software. The second iteration of the questionnaire covered scanner-related factors, sequence-related factors, physiology monitoring, and stimulation-related factors. Items showing strong consensus/consensus (≥90%/≥75% of participants indicating scores 4 or 5) were included as standard reporting items.</p><p><strong>Results: </strong>All 3 rounds of the first/second iteration were completed by 29 and 26 researchers (age 46 ± 11 years; 38% female/age 46 ± 12 years; 44% female) from 23 and 21 institutions. Across both iterations, strong consensus and consensus was achieved for 34 (3 scanner-related factors, 9 sequence-related factors, 1 stimulation-related factor, 2 demographic factors, 7 migraine-specific factors, 2 medication-factors, 2 scan timing factors, 4 HC factors, 1 preregistration factor, 1 analysis software factor, and 2 ROI analyses factors) and 33 (1 scanner-related factors, 4 sequence related factors, 1 factor related to physiology monitoring, 1 stimulation-related factor, 3 demographic factors, 6 migraine-specific factors, 4 medication factors, 3 HC factors, 2 sampling factors, 1 standardized form, 1 preregistration factor, 1 data sharing factor, 2 analysis software factors, and 3 ROI analyses factors) items, respectively. From these, a checklist covering 63 items from 14 reporting domains was created.</p><p><strong>Discussion: </strong>We present an expert-based framework for reporting standards in fMRI studies of migraine, which can be used for future studies to homogenize cohort characterization, fMRI acquisitions, and analysis protocols.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 5","pages":"e210235"},"PeriodicalIF":7.7,"publicationDate":"2025-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143390875","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}