Neurology最新文献

{"title":"Editors' Note: A Preponderance of Evidence: A Call for a Randomized Trial in Posterior Circulation Stroke.","authors":"Ariane Lewis, Steven L Galetta","doi":"10.1212/WNL.0000000000213415","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213415","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 5","pages":"e213415"},"PeriodicalIF":7.7,"publicationDate":"2025-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143071003","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":"Editors' Note: Functional Outcome and Hemorrhage Rates After Bridging Therapy With Tenecteplase or Alteplase in Patients With Large Ischemic Core.","authors":"Aravind Ganesh, Steven L Galetta","doi":"10.1212/WNL.0000000000213450","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213450","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 5","pages":"e213450"},"PeriodicalIF":7.7,"publicationDate":"2025-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143256091","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 Burden of Multiple Sclerosis in Mexico.","authors":"Enrique Gómez-Figueroa, Carlos Javier Moreno-Bernardino, Andrea Margarita De Alba-Sánchez, Natali Guerrero-Udave, Patricia Orozco-Puga, Cynthia Patricia Corona-Vázquez, María Eugeni Briseño-Godínez, Omar Cárdenas-Sáenz, Amado Jímenez-Ruiz, Brenda Allison Verboonen-Salgado, Nayeli Sánchez-Rosales, Christian García Estrada, José Luis Ruíz-Sandoval","doi":"10.1212/WNL.0000000000213343","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213343","url":null,"abstract":"<p><strong>Background and objectives: </strong>Multiple sclerosis (MS) is a CNS disease causing significant disability, mainly in young and middle-aged individuals. Despite extensive global research, Mexico lacks comprehensive epidemiologic data on MS, complicating effective health care planning and intervention. This study analyzes the epidemiology of MS in Mexico using data from the Global Burden of Disease (GBD) study. It focuses on prevalence, incidence, disability-adjusted life years (DALYs), years lived with disability (YLDs), and years of life lost (YLLs) from 1990 to 2021. In addition, it examines the relationship between the sociodemographic index (SDI) and MS burden across Mexican states.</p><p><strong>Methods: </strong>Data were sourced from the GBD 2021 and analyzed using standard GBD methodologies. Key metrics included prevalence, DALYs, YLDs, and YLLs, standardized by age. Pearson correlation and linear regression were used to evaluate the association between SDI and MS prevalence. A locally weighted regression (LOESS) model was applied to compare observed and expected DALY rates based on SDI, identifying regional disparities in MS burden.</p><p><strong>Results: </strong>In 2021, an estimated 18,016 individuals (95% uncertainty interval [UI] 14,993-21,337) lived with MS in Mexico, with an age-standardized prevalence of 13.10 per 100,000 inhabitants (95% UI 10.91-15.50). The incidence rate was 0.65 per 100,000 inhabitants (95% UI 0.55-0.75). Total DALYs for MS in 2021 were 17,947 (95% UI 14,458-20,542), comprising 13.05 age-standardized DALYs per 100,000 inhabitants. YLLs accounted for 9.47 per 100,000 inhabitants (95% UI 8.24-10.85) and YLDs for 3.56 (95% UI 2.45-4.77). The LOESS model revealed significant regional discrepancies, with Northern Mexico exhibiting better-than-expected health outcomes while Central and Southern Mexico displaying higher observed DALYs than expected.</p><p><strong>Discussion: </strong>The findings highlight substantial regional disparities in the MS burden across Mexico. Northern Mexico showed better-than-expected health outcomes while Central and Southern Mexico exhibited higher disease burdens than anticipated. These discrepancies suggest that socioeconomic factors and health care accessibility significantly affect MS outcomes. The study's limitations include reliance on hospital records and potential underdiagnosis in less developed regions. Enhanced data collection and comprehensive health care strategies are essential to effectively address the growing MS burden in Mexico.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 5","pages":"e213343"},"PeriodicalIF":7.7,"publicationDate":"2025-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143256181","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":"Disease Burden in Female Patients With X-Linked Adrenoleukodystrophy.","authors":"Natalie R Grant, Yedda Li, Lizbeth De La Rosa Abreu, Catherine Becker, Brian D Wishart, Amanda Nagy, Florian S Eichler, Reza Sadjadi","doi":"10.1212/WNL.0000000000213370","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213370","url":null,"abstract":"<p><strong>Background and objectives: </strong>X-linked adrenoleukodystrophy (ALD) is a neurodegenerative disease primarily affecting male patients. Female patients with ALD are also affected in adulthood, yet their disease course and symptom burden remain poorly defined. In this single-site study, we set out to characterize disease burden in female individuals with ALD and identify barriers faced by this patient population.</p><p><strong>Methods: </strong>Adult female individuals with genetically or biochemically confirmed ALD were recruited through an outpatient specialty clinic and a patient advocacy group. We performed a retrospective chart review and conducted prospective telephone interviews to assess symptom presence and onset, interventions and management strategies, injuries, comorbidities, and quality of life (QOL). For comparison, we retrospectively gathered data from ALD diagnosis and symptom onset for adult male patients with ALD seen in our clinic.</p><p><strong>Results: </strong>We included 127 female (median [interquartile range] age = 50.2 [39.2, 59.9]) and 82 male individuals with ALD (median [interquartile range] age = 37.5 [24.2, 43.9] years). Among our female cohort, 115 (91%) reported neurologic symptoms. The most common symptoms were urinary symptoms (74%), walking difficulty (66%), and spasticity (65%). Mental health symptoms were also common (64%). Of interest, 70 (55%) reported a history of falls, 61 (48%) had sustained injuries from falling, and 54 (43%) had a history of fractures. Compared with the male cohort, our female cohort had a significantly later age at symptom onset and diagnosis. In addition, symptom presentation was less likely to prompt a diagnosis in female individuals. Of 46 female individuals who sought clinical care for symptoms before diagnosis, 22 were initially misdiagnosed. Fifty-one (90%) of 57 female interviewees reported encountering challenges with health care access, and 49 (86%) reported a reduction in different aspects of QOL. Activities of daily living beyond walking were affected in 25 (44%) participants.</p><p><strong>Discussion: </strong>We conclude that symptoms related to myelopathy and neuropathy are common in female individuals with ALD and that their disease burden is aggravated by the high rates of mental health problems, barriers to health care access, and injuries and complications requiring treatment. Limitations of our study include a risk for recall bias and selection bias.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 5","pages":"e213370"},"PeriodicalIF":7.7,"publicationDate":"2025-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143370806","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":"Pearls & Oy-sters: Isolated Acquired Amusia in a Patient With Right Temporal Stroke.","authors":"Mark Dibbs, Jeremy J Moeller","doi":"10.1212/WNL.0000000000213410","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213410","url":null,"abstract":"<p><p>Strokes in the right temporal lobe are known to cause acquired amusia, or deficits in music processing, which can be formally assessed using the online version of the Montreal Battery of Evaluation of Amusia (MBEA). Patients with acquired amusia most often present with not only amusia but also other neurologic symptoms, such as aphasia, neglect, or memory issues. We report a case of a 39-year-old man who initially presented for follow-up after a single seizure episode. Two years before the seizure, the patient experienced an episode of headache, nausea, and vomiting, after which he developed difficulty appreciating music and carrying a tune, something he had never experienced before as a competent trumpet player and singer. An MRI scan performed after his seizure revealed encephalomalacia and gliosis within the right lateral temporal lobe with areas of hemosiderin deposition, suggesting that the episode 2 years ago was a stroke. His standard neurologic examination was normal including a score of 30/30 on the Montreal Cognitive Assessment. On the online version of the MBEA, he scored 66.7% on the off-tune test, 87.5% on the off-beat test, and 70.8% on the out-of-key test, consistent with a diagnosis of amusia. This case highlights the importance of eliciting less common isolated neurologic symptoms in patients with an otherwise normal examination, including musical symptoms. We also highlight the utility of tools such as the MBEA to document the severity of amusia and potentially to follow patients' progress as they recover.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 5","pages":"e213410"},"PeriodicalIF":7.7,"publicationDate":"2025-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143071047","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":"Perspectives Toward the Development of Quantitative Seed Amplification Assays for α-Synuclein.","authors":"Simone Baiardi, Piero Parchi","doi":"10.1212/WNL.0000000000213384","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213384","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 5","pages":"e213384"},"PeriodicalIF":7.7,"publicationDate":"2025-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143365311","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":"Influence of Risk Factor Combinations on Incidence Rates of SUDEP: A Population-Based Study.","authors":"Torbjörn Tomson, Tomas Andersson, Sofia Carlsson, Olafur Sveinsson","doi":"10.1212/WNL.0000000000213372","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213372","url":null,"abstract":"<p><strong>Background and objective: </strong>Information on absolute risks of sudden unexpected death in epilepsy (SUDEP) in individual patients with epilepsy is scarce. Our main objective was therefore to explore the range in incidence rates of SUDEP to provide a more solid basis for individualized counseling and to characterize patients with high and very high SUDEP incidence for future intervention studies aiming at prevention of SUDEP.</p><p><strong>Methods: </strong>We used data on everyone in Sweden diagnosed with epilepsy from 1998 to 2005 (n = 60,952), followed until 2011 and identified SUDEP cases through adjudication of deceased patients who had epilepsy. We conducted a nested case-control study and retrieved detailed information on clinical characteristics for the SUDEP cases and matched living epilepsy controls (5:1). Estimates of the strengths of associations from the case-control study were used to estimate the incidence rate of SUDEP (per 100,000 person-years) in the full cohort by SUDEP risk factors.</p><p><strong>Results: </strong>Two hundred fifty-five SUDEP cases (median age 48 years; 154 males) were identified. The lowest incidence, 8 (95% CI 3-17), was observed among patients without a history of tonic-clonic seizures (TCS), whereas patients with 1 or more TCS the preceding year had an incidence of 287 (95% CI 192-428). Incidence rates above 200 were also found among patients with a history of nocturnal TCS, substance abuse or alcohol dependence, and nonadherence with antiseizure medication (ASM) treatment. Considering combination of risk factors, the incidence rate was very low, 5 (95% CI 2-12), for patients who share bedroom and are free from TCS the preceding year as well as adherent with the prescribed ASM treatment. By contrast, patients living alone who are nonadherent have a history of nocturnal TCS and at least 1 TCS the preceding year have an incidence at 1808 (95% CI 594-5,504), more than 350 times higher than the low-risk patient.</p><p><strong>Discussion: </strong>In this analysis of Swedish population-based SUDEP data, we have identified a 350-fold difference in the SUDEP incidence depending on individual circumstances and epilepsy characteristics. Although somewhat old, our data should be useful for patient counseling about individual SUDEP risks amenable to modification and for case stratifications for intervention studies aiming at prevention of SUDEP.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 5","pages":"e213372"},"PeriodicalIF":7.7,"publicationDate":"2025-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143256178","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":"Clinical Reasoning: A 32-Year-Old Woman With Recurrent Intracerebral Hemorrhages.","authors":"Mette Foldager Hindsholm, David Gaist, Benedicte Parm Ulhøi, Claus Ziegler Simonsen","doi":"10.1212/WNL.0000000000213422","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213422","url":null,"abstract":"<p><p>Evaluating patients with intracerebral hemorrhage is common practice in the field of stroke neurology but can be complicated and may require extensive evaluation in younger patients with no history of hypertension. In this case, a healthy 32-year-old woman presented with an acute spontaneous lobar intracerebral hemorrhage. Neurologic workup required extensive imaging evaluation, genetic testing, and a thorough evaluation of patient and family medical history to identify the final diagnosis. This case highlights the diagnostic approach and importance of thorough clinical evaluation of young patients with intracerebral hemorrhages. Readers will walk through the stepwise diagnostic approach to arrive at the leading diagnosis with a review of the possible differential diagnoses and a discussion of this rare condition.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 5","pages":"e213422"},"PeriodicalIF":7.7,"publicationDate":"2025-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143070994","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 Race and Ethnicity With Emergency Room Rate of Migraine Diagnosis, Testing, and Management in Children With Headache.","authors":"Danielle Kellier, Marissa Maliakal Anto, Matt Hall, Jennifer Marin, Kendall Nash, Elizabeth M Wells, Nicholas S Abend, Melissa L Hutchinson, Mahendranath Moharir, Ricka Denise Messer, Jamie Lee Palaganas, Juan Piantino, Christina Szperka, Craig Press","doi":"10.1212/WNL.0000000000213351","DOIUrl":"10.1212/WNL.0000000000213351","url":null,"abstract":"<p><strong>Background and objectives: </strong>Headache evaluation and treatment are believed to be influenced by race and ethnicity. Specific headache diagnosis assigned in the pediatric emergency department (ED) may compound disparities. We sought to investigate racial and ethnic disparities in the diagnosis, testing, and treatment of pediatric patients with headache presenting to the ED.</p><p><strong>Methods: </strong>We performed a cross-sectional analysis of ED visits from 49 children's hospitals between 2016 and 2022 from the Pediatric Health Information System, an administrative database of ED and hospitalized encounters within children's hospitals in the United States. Index encounters in the ED from patients (aged 5-21 years, median age 13 [10-15]) with a primary diagnosis of migraine, headache, new daily persistent headache, or tension-type headache were included. Encounters with trauma, infection, and malignancy where secondary headache was possible were excluded. The primary outcomes were the rates of migraine diagnosis, testing, and treatment. We used generalized estimating equations to estimate associations between race and ethnicity and outcomes after adjusting for demographic factors, medical complexity, visit timing, and final headache diagnosis.</p><p><strong>Results: </strong>A total of 309,678 encounters were included while 61,677 repeat visits, 81,821 visits with diagnoses suggestive of secondary headache, and 5,714 visits from 3 hospitals with sparse data on patient race/ethnicity were excluded. Of 160,466 eligible visits (59.8% female), 41% were by non-Hispanic White (NHW) children, 24.8% non-Hispanic Black (NHB), and 26.0% Hispanic/Latino (HL). NHW children were more frequently diagnosed with migraine (45.5% vs NHB 28.2% and HL 28.3%, <i>p</i> < 0.001). NHB and HL children compared with NHW children received less testing including brain MRI scans (adjusted odds ratio [aOR]: NHB 0.56 [95% CI 0.46-0.69] and HL 0.54 [0.36-0.82]). There was no difference in the proportion of visits without administration of headache-related medications (NHW 23.3% vs NHB 24.6% and HL 23.4%, <i>p</i> = 0.64). NHB and HL children were more likely to receive only oral medications (aOR: NHB 1.37 [1.2-1.56] and HL 1.54 [1.34-1.76]) and less likely to be admitted inpatient (aOR: NHB 0.8 [0.66-0.97] and HL 0.65 [0.44-0.94]).</p><p><strong>Discussion: </strong>NHB and HL children in the pediatric ED with headache receive fewer migraine diagnoses, less testing, and less intensive treatment compared with NHW children. Beyond affecting headache management, this inequity in migraine diagnosis requires further consideration to include children from marginalized racial and ethnic groups in future migraine research.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 5","pages":"e213351"},"PeriodicalIF":7.7,"publicationDate":"2025-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11795614/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143255980","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":"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}