Neurology最新文献

{"title":"Diagnosis and Management of Adult Status Epilepticus in Resource-Limited Settings: A Systematic Review.","authors":"Aayesha J Soni, R Grace Couper, M Pilar Vicuna, Jorge G Burneo","doi":"10.1212/WNL.0000000000213479","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213479","url":null,"abstract":"<p><strong>Background and objectives: </strong>Status epilepticus (SE) is the leading cause of death in patients with epilepsy, and it affects people in low/middle-income countries (LMICs) at a much higher rate. There is likely a significant gap between the recommended diagnosis and treatment of SE and current practices in resource-limited settings. We conducted a systematic literature review to determine how convulsive and nonconvulsive SE in adults is diagnosed and managed in LMICs.</p><p><strong>Methods: </strong>All relevant articles from Embase, Medline, PubMed, and the Virtual Health Library Regional Portal databases, published before September 16, 2024, were included. Studies needed to take place in LMICs and include treatment and outcomes of patients with SE. This review followed the Preferred Reporting Items for Systematic Review and Meta-Analyses guidelines. The risk of bias was assessed using the Risk of Bias in Randomized Trials and Risk of Bias in Non-randomized Studies of Interventions tools.</p><p><strong>Results: </strong>Our review included 23 studies from 3 continents including 1,526 patients, with most of the studies conducted in Asia. There is a lack of literature from Africa and surrounding the topic of nonconvulsive SE. The commonest etiology of SE was an acute symptomatic cause (21%-88%), with encephalitis predominating overall. Diagnostic and management practices varied greatly, dictated by local availability of drugs and expertise, rather than guidelines. First-line benzodiazepines were routinely underdosed while older and cheaper second-line antiseizure medications, such as valproic acid, phenytoin, and phenobarbital, were more frequently administered. In addition, there was a general lack of access to continuous EEG monitoring, with only 5 studies from tertiary-level centers in Asia reporting its usage. Mortality outcomes of up to 42.6% are higher in comparison with high-income countries.</p><p><strong>Discussion: </strong>The heterogeneity in management practices of SE in LMICs highlights the lack of consistent treatment, with very few studies from Africa and Latin America available in the literature. This contributed to the limitations of this review, with only a small region of countries (mostly from Asia) represented and retrospective review of clinical records predominantly used. The nonuniformity of diagnostic and management practices in SE has highlighted the need for clinically appropriate guidelines in LMICs.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 8","pages":"e213479"},"PeriodicalIF":7.7,"publicationDate":"2025-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143764354","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":"Ventral Subpial and Central Cord Enhancement in Spinal Neurosarcoidosis: The Reverse Trident Sign.","authors":"Amir M Jaafari, Eric Newman, Eoin P Flanagan, Sebastian A Lopez-Chiriboga, Sarosh R Irani","doi":"10.1212/WNL.0000000000213523","DOIUrl":"10.1212/WNL.0000000000213523","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 8","pages":"e213523"},"PeriodicalIF":7.7,"publicationDate":"2025-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11962045/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143764421","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":"Association of Cardiovascular Health With Brain Volume and White Matter Integrity: Results of the SOL-INCA MRI Study.","authors":"Gabriela Trifan, Ariana M Stickel, Jianwen Cai, Martha Daviglus, Mayra Estrella, Olga Garcia-Bedoya, Linda C Gallo, Carmen R Isasi, Robert Kaplan, Melissa Lamar, Gregory A Talavera, Wassim Tarraf, Hector M Gonzalez, Pauline Maillard, Charles S Decarli, Fernando D Testai","doi":"10.1212/WNL.0000000000213404","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213404","url":null,"abstract":"<p><strong>Background and objectives: </strong>Better cardiovascular health (CVH), measured using the Life's Simple 7 (LS7) score, is associated with improved cognitive function. However, its effect on brain imaging in Hispanics/Latinos has not been reported. We investigated the association of LS7 score with brain MRI outcomes in a diverse Hispanic/Latino population.</p><p><strong>Methods: </strong>Hispanic Community Health Study/Study of Latinos (HCHS/SOL) is the largest prospective cohort study of diverse Hispanic/Latino adults in the United States. LS7 score was assessed at baseline (2008-2011). The SOL-Investigation of Neurocognitive Aging (SOL-INCA) MRI ancillary study recruited HCHS/SOL participants who underwent neuroimaging approximately 10 years (2017-2022) later. Main outcomes include standardized residuals for total brain, total and lobar gray matter (frontal, temporal, parietal, and occipital), total white matter, total CSF, lateral ventricle (LV), and white matter hyperintensity (WMH) volumes and diffusion tensor imaging (mean free water fraction, peak width skeletonized mean diffusivity, and mean fractional anisotropy). MRI outcomes across continuous LS7 scores were investigated using adjusted linear regression models. We performed an interaction analysis by age and sex. All analyses accounted for complex sample design.</p><p><strong>Results: </strong>The study included 2,659 participants (weighted %female = 56%). The mean age (95% CI) was 54 (53-55) years, and the mean LS7 score was 7.2 (7.1-7.3). In the fully adjusted model, higher LS7 scores were associated with larger total and lobar brain volumes (β_{Total Brain} = 0.03 [0.002 to 0.06], β_{Total White} = 0.05 [0.03 to 0.08], β_{Total Gray} = 0.04 [0.01 to 0.06], β_{Frontal Gray} = 0.04 [0.02 to 0.07]); smaller total CSF (β = -0.06 [-0.08 to -0.03]), LV (β = -0.03 [-0.06 to -0.004]), and WMH (β = -0.05 [-0.07 to -0.03]) volumes; and better measures of microstructural integrity (β_FW = -0.002 [-0.003 to -0.001], β_PSMD = -0.01 [-0.01 to -0.003], and β_FA = 0.001 [0.001 to 0.002]). In interaction analysis by age, the benefits of CVH on magnetic resonance volumes and microstructural integrity were more pronounced in older patients. No interaction was observed by sex.</p><p><strong>Discussion: </strong>We found that better CVH is associated with better brain-based volumes and microstructural integrity and that the effects of LS7 score on brain health are age dependent. Additional studies are needed to assess the longitudinal effect of CVH on MR-based brain health outcomes.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 8","pages":"e213404"},"PeriodicalIF":7.7,"publicationDate":"2025-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143700849","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":"Comparison of the Clinical Spectrum of Juvenile- and Adult-Onset Huntington Disease: A National Cohort and Enroll-HD Observational Study.","authors":"Hannah S Bakels,Kasper F van der Zwaan,Erik Van Zwet,Robert Reijntjes,Gregory P Sprenger,Thijs A Knecht,Raymund A C Roos,Susanne T de Bot","doi":"10.1212/wnl.0000000000213525","DOIUrl":"https://doi.org/10.1212/wnl.0000000000213525","url":null,"abstract":"BACKGROUND AND OBJECTIVESDifferences in clinical characteristics between juvenile-onset Huntington disease (JHD) and adult-onset HD (AHD) are hypothesized but not directly compared. This study compares clinical characteristics occurrence and severity across age-at-onset (AO) subtypes.METHODSUsing the national juvenile-onset HD patient cohort and the international Enroll-HD registry (NCT01574053), we compared childhood-onset JHD (cJHD; AO 0-10), adolescent-onset JHD (aJHD; AO 11-20), and adult-onset HD (AHD; AO 21-65) on proportions of clinical characteristics at onset and psychiatric characteristics in pooled datasets. Kruskal-Wallis test was applied to UHDRS-Total Motor Score (UHDRS-TMS) items of the Enroll-HD dataset to compare the severity of motor disease characteristics 6-10 years after onset.RESULTSThe combined datasets provided data from 46 patients with cJHD (mean AO 6.70, 45% female), 243 patients with aJHD (mean AO 16.70, 46% female), and 9,504 patients with AHD (mean AO 44.70, 51% female). At onset, neurocognitive symptoms occurred in 47.50% of patients with cJHD (n = 46; 95% CI 31.80%-63.70%), significantly more often compared with 24.88% of patients with aJHD (n = 209; 19.30%-31.40%) and 15.02% of those with AHD (n = 8,177; 14.30%-15.80%). Psychiatric symptoms occurred in 47.12% of patients with aJHD (95% CI 40.20%-54.10%), significantly more compared with 31.04% of patients with AHD (30.10%-32.00%). Throughout the disease, aggressive behavior occurred in 73.91% of patients with cJHD (n = 46; 95% CI 58.60%-85.20%) and 55.88% of those with aJHD (n = 238; 49.30%-62.30%), significantly more compared with 40.65% of patients with AHD (n = 9,501; 39.70%-41.70%). Psychosis occurred in 23.53% of patients with aJHD (95% CI 18.40%-29.50%), significantly more compared with 12.77% of those with AHD (12.10%-13.50%). The Kruskal-Wallis test revealed significantly higher median UHDRS-TMS scores in one or both JHD subtypes compared with AHD for dysarthria (AHD: n = 4,163, median 1.00, interquartile range (IQR) 0.70; cJHD: n = 12, 2.20, 2.00, p = 0.039; aJHD: n = 93, 1.00, 1.00, p = 0.031), parkinsonism (AHD: n = 4,158, median 6.00, IQR 4.70; cJHD: n = 12, 11.00, 9.40, p = 0.008; aJHD: n = 93, 8.50, 6.80, p < 0.001), and dystonia (AHD: n = 4,161, median 2.00, IQR 5.20; cJHD: n = 12, 6.50, 8.20, p = 0.141; aJHD: n = 93, 4.00, 7.20, p = 0.015) and significantly lower median scores for chorea (AHD: n = 4,163, median 9.20, IQR 7.00; cJHD: n = 12, 5.00, 4.20, p = <0.001; aJHD: n = 93, 6.30, 9.50, p < 0.001).DISCUSSIONThis study highlights distinct clinical patterns in JHD subtypes compared with AHD. Stratification by age at onset-defined HD subtypes is needed in future studies.","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"13 1","pages":"e213525"},"PeriodicalIF":9.9,"publicationDate":"2025-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143866386","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: Multipsychiatric Comorbidity in People With Epilepsy Compared With People Without Epilepsy: A Systematic Review and Meta-Analysis.","authors":"Aravind Ganesh, Steven L Galetta","doi":"10.1212/WNL.0000000000213561","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213561","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 8","pages":"e213561"},"PeriodicalIF":7.7,"publicationDate":"2025-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143674070","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":"Author Response: Multipsychiatric Comorbidity in People With Epilepsy Compared With People Without Epilepsy: A Systematic Review and Meta-Analysis.","authors":"Churl-Su Kwon, Charles R Newton, Nathalie Jette","doi":"10.1212/WNL.0000000000209942","DOIUrl":"https://doi.org/10.1212/WNL.0000000000209942","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 8","pages":"e209942"},"PeriodicalIF":7.7,"publicationDate":"2025-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143674146","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":"How a Concussion Affects Intrinsic Brain Networks in Children: Time Will Tell.","authors":"Harm J van der Horn, Andrew R Mayer","doi":"10.1212/WNL.0000000000213575","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213575","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 8","pages":"e213575"},"PeriodicalIF":7.7,"publicationDate":"2025-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143764416","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":"Modifiable Vascular Risk Factors and Subsequent Brain Metrics in Hispanic/Latino Populations.","authors":"Josephine Barnes, Lloyd Prosser","doi":"10.1212/WNL.0000000000213547","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213547","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 8","pages":"e213547"},"PeriodicalIF":7.7,"publicationDate":"2025-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143700955","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":"Neuroimaging in Children With Inherited Metabolic Epilepsies.","authors":"Itay Tokatly Latzer, Edward Yang, Vitor Lauar Pimenta de Figueiredo, Susie Y Huang, Teppei Matsubara, Phillip L Pearl","doi":"10.1212/WNL.0000000000213485","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213485","url":null,"abstract":"<p><p>Inherited metabolic epilepsies (IMEs) represent inherited metabolic disorders in which seizures, various in types and typically intractable, are one of the predominant manifestations and primary determinants of the overall outcome. A metabolic etiology should be suspected in any case of refractory seizures stemming from an undetermined cause. While the occurrence rate of individual IMEs is rare, their cumulative prevalence is high enough to pose a significant clinical challenge. Concurrently with genetic and metabolic tests, neuroimaging is considered part of the first tier of their investigation, attained with an aim to affirm or counter the phenotype. It may be normal or nonspecific; however, as described by this overview, there is an appreciable number of IMEs associated with distinct MRI or magnetic resonance spectroscopy patterns. Apart from narrowing or supporting their diagnoses, these neuroimaging patterns may serve as biomarkers to monitor treatment efficacy and outcome. Other advanced modalities and techniques, such as PET, fMRI, diffusion tensor imaging, MRI g-ratio mapping, and functional near-infrared spectroscopy, broaden our insights relating to their pathophysiology, clinical course, targeted treatment development, and prognosis.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 8","pages":"e213485"},"PeriodicalIF":7.7,"publicationDate":"2025-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143710868","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":"Autonomic Dysfunction in Patients With Dementia With Lewy Bodies and Its Relationship With Nigrostriatal Denervation.","authors":"Chiara Giuseppina Bonomi, Alessandro Martorana, Caterina Motta, Chiara Serafini, Agostino Chiaravalloti, Benedetta Lauretti, Orazio Schillaci, Nicola Biagio Mercuri, Camilla Rocchi","doi":"10.1212/WNL.0000000000213463","DOIUrl":"https://doi.org/10.1212/WNL.0000000000213463","url":null,"abstract":"<p><strong>Background and objectives: </strong>Diagnosing dementia with Lewy bodies (DLBs) is challenging because of symptom overlap with other neurodegenerative diseases. Although dysautonomia is a recognized supportive diagnostic criterion, its prevalence and extent remain underexplored. We aimed to evaluate autonomic dysfunction in patients with DLB using a comprehensive battery of autonomic function tests (AFTs) and to investigate whether this dysfunction differs between patients with and without nigrostriatal denervation.</p><p><strong>Methods: </strong>This prospective cohort study was performed at a Memory Clinic in Rome, Italy. Patients meeting diagnostic criteria for possible DLB were enrolled and underwent AFTs including head-up tilt test (HUTT), Valsalva maneuver, deep breathing, cold face, hand grip (HG), and electrochemical skin conductance. Dopamine transporter SPECT (DaT-SPECT) was performed to assess nigrostriatal transmission. We compared results from AFTs in (1) patients with DLB vs healthy controls (HCs) and (2) patients with DLB with pathologic vs normal DaT-SPECT.</p><p><strong>Results: </strong>Twenty-two patients with DLB (median age: 72.00 [10.00] years, %female: 21.75) and 20 HCs (median age: 69.00 [5.25] years, %female: 40) were enrolled. Only 1 patient (4.5%) showed neurogenic orthostatic hypotension (nOH) at HUTT. However, patients with DLB showed cardiovascular adrenergic dysfunction, represented by lower Valsalva overshoot (<i>r</i> = -0.553, 95% CI -0.773 to -0.214, <i>p</i> = 0.008) and HG Δdiastolic blood pressure (<i>r</i> = -0.703, 95% CI -0.844 to -0.470, <i>p</i> < 0.0001); parasympathetic cardiovagal dysfunction, reflected in the lower Valsalva ratio (<i>r</i> = -0.812, 95% CI -0.912 to -0.622, <i>p</i> < 0.0001) and sinus arrhythmia at deep breathing (<i>r</i> = -0.682, 95% CI -0.837 to -0.426, <i>p</i> < 0.001); and reduced sudomotor function in hands (<i>r</i> = -0.648, 95% CI -0.809 to -0.395, <i>p</i> < 0.001) and feet (<i>r</i> = -0.600, 95% CI -0.781 to -0.327, <i>p</i> < 0.001). Multivariable analyses found that age and sex were not associated with AFTs, but a higher Mini-Mental State Examination score was associated with better Valsalva ratio (<i>B</i> = 0.038, 95% CI 0.010-0.066, <i>p</i> = 0.010). Patients with normal DaT-SPECT had worse HG responses than those with pathologic DaT-SPECT (<i>r</i> = -0.686, 95% CI -0.895 to -0.231, <i>p</i> = 0.029).</p><p><strong>Discussion: </strong>Despite the absence of overt nOH, patients with DLB show covert dysautonomia encompassing adrenergic, parasympathetic, and sudomotor dysfunction, highlighting the importance of standardized autonomic evaluation. Patients with normal DaT-SPECT exhibited greater peripheral autonomic impairment, reflected by lower HG responses, suggesting diverse α-synuclein pathology trajectories within DLB. Further research is needed to explore autonomic nervous system dysfunctions across different DLB subtypes and stages.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"104 8","pages":"e213463"},"PeriodicalIF":7.7,"publicationDate":"2025-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143670422","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}