Neurology最新文献

{"title":"Effect of Hemoglobin and Blood Glucose Levels on CT Perfusion Ischemic Core Estimation: A Post Hoc Analysis of the ESCAPE-NA1 Trial.","authors":"Umberto Pensato, Salome Bosshart, Alexander Stebner, Axel Rohr, Timothy J Kleinig, Rishi Gupta, Götz Thomalla, Ji Hoe Heo, Mayank Goyal, Andrew M Demchuk, Michael D Hill, Johanna M Ospel","doi":"10.1212/WNL.0000000000209939","DOIUrl":"10.1212/WNL.0000000000209939","url":null,"abstract":"<p><strong>Background and objectives: </strong>CT perfusion (CTP) maps can estimate the ischemic core in acute ischemic stroke based on distinctive cerebral blood flow thresholds. However, metabolic factors beyond perfusion influence the tissue tolerance to ischemia and the infarct growth rate. Underestimating the ischemic core volume (ICV) might result in overestimating the salvageable cerebral tissue and, consequently, overestimating the potential clinical benefits of reperfusion therapies. We aim to evaluate whether baseline hemoglobin and blood glucose levels influence the accuracy of baseline CTP ICV estimations.</p><p><strong>Methods: </strong>Large vessel occlusion stroke patients investigated with baseline CTP undergoing thrombectomy with near-complete reperfusion and without parenchymal hemorrhage from the ESCAPE-NA1 trial were included. Patients were subdivided into anemic (hemoglobin <130 g/L for men and <120 g/L for women) and nonanemic groups, and hyperglycemic (blood glucose level >7 mmol/L) and normoglycemic groups. Ischemic core underestimated volume (ICuV) was calculated: final infarct volume minus CTP-based ICV. The primary outcome was the presence of \"perfusion scotoma\" defined as ICuV ≥10 mL. Presence of \"perfusion scotoma\" and median ICuV were compared between anemic vs nonanemic and hyperglycemic vs normoglycemic patients using nonparametric tests and multivariable binary logistic regression with adjustment for baseline variables.</p><p><strong>Results: </strong>One hundred sixty-two of 1,105 (15%) patients were included (median age 70.5 [interquartile range (IQR) 61-80.4], 50.6% women). The median ICuV was 7.26 mL (IQR 0-25.63). Seventy-eight (48%) patients demonstrated perfusion scotoma. Forty-two (25.7%) patients were anemic, and 65 (40.1%) were hyperglycemic. In univariable analysis, the hyperglycemic group had a higher prevalence of perfusion scotoma (65% [n = 40] vs 39% [n = 38], <i>p</i> = 0.006) and larger ICuV (17.79 mL [IQR 1.57-42.75] vs 6 mL [-0.31 to 12.51], <i>p</i> = 0.003) compared to normoglycemic patients. No significant ICuV differences between patients with and without anemia were seen. Multivariable regression analysis revealed an association between perfusion scotoma and hyperglycemia, adjusted odds ratio (OR) 2.48 (95% CI 1.25-4.92), and between perfusion scotoma and blood glucose levels, adjusted OR 1.19 (95% CI 1.03-1.39) per 1 mmol/L increase.</p><p><strong>Discussion: </strong>In our study, CTP-based ischemic core underestimation was common and associated with higher baseline blood glucose levels. Individual metabolic factors beyond perfusion that critically influence the infarct growth rate should be considered when interpreting baseline CTP estimations of ischemic core.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"103 10","pages":"e209939"},"PeriodicalIF":4.4,"publicationDate":"2024-11-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142471046","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":"Immune-Mediated Megaconial Myopathy: A Novel Subtype of Autoimmune Myopathy.","authors":"Ashley R Santilli, Oliver Ni, Margherita Milone, Duygu Selcen, Anahit C Mehrabyan, Arjun Seth, Christine Hsieh, Wasim F Raslan, Moayd M Alkhalifah, Raed M Alenezi, Stefan Nicolau, Pannathat Soontrapa, Teerin Liewluck","doi":"10.1212/WNL.0000000000210001","DOIUrl":"https://doi.org/10.1212/WNL.0000000000210001","url":null,"abstract":"<p><strong>Objectives: </strong>To describe a novel subtype of autoimmune myopathy, immune-mediated megaconial myopathy (IMMM), myopathologically characterized by giant mitochondria (megaconia).</p><p><strong>Methods: </strong>In this case series, we reviewed the Mayo Clinic Muscle Pathology database, between 2018 and 2023, to identify patients with megaconial pathology, subacute progressive weakness, and hyperCKemia, clinically resembling myositis. We recruited 1 patient from another institute, who had similar clinicopathologic features.</p><p><strong>Results: </strong>Five patients were identified. Age at onset of weakness ranged from 19 to 45.5 years. All patients had proximal weakness, elevated creatine kinase levels (1,214 to 5,920 U/L), negative myositis antibodies, necrotizing myopathology, and nonnecrotic myofibers harboring giant mitochondria. Immunohistochemical studies conducted in 4 patients showed sarcolemmal MHC-1 and C5b9 immunoreactivities. Megaconial pathology was considered pathognomonic of congenital muscular dystrophy due to biallelic pathogenic variants in <i>CHKB</i>. Sequencing of <i>CHKB</i> in 4/5 patients was unrevealing. Immunomodulatory therapy improved weakness and hyperCKemia in 4 treated patients. Of interest, all patients had coexisting pancreatic diseases (3 cystic fibrosis-related exocrine pancreatic insufficiency, 1 pancreatic cancer, and 1 pancreatitis).</p><p><strong>Discussion: </strong>In addition to incurable <i>CHKB</i>-congenital muscular dystrophy, giant mitochondria can also occur in this new subtype of treatable autoimmune myopathy, IMMM. The association between IMMM and pancreatic disorders remains to be elucidated.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"103 10","pages":"e210001"},"PeriodicalIF":7.7,"publicationDate":"2024-11-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142546546","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":"Hughlings Jackson's Second Thoughts on Mental States in Epilepsy.","authors":"Andrew J Larner, Michael Swash","doi":"10.1212/WNL.0000000000209959","DOIUrl":"https://doi.org/10.1212/WNL.0000000000209959","url":null,"abstract":"<p><p>John Hughlings Jackson (1835-1911) was the pre-eminent British neurologist of the last 3 decades of the 19th century whose most seminal contributions related to the understanding of epileptic seizures. Jackson instructed that his personal papers should be destroyed at his death, and consequently, few examples of his handwriting now survive. We discovered a series of marginalia in Jackson's handwriting annotating one of his papers, \"On temporary mental disorders after epileptic paroxysms,\" first published in 1875 in the <i>West Riding Lunatic Asylum Medical Reports</i>. Two of the most extensive annotations indicate Jackson's later understanding of \"epileptic vertigo\" and of \"mental automatisms.\" We contextualize the changes in Jackson's thinking suggested by these emendations. These marginalia give insights into Jackson's continuing effort to understand epilepsy and its implications for brain function, an issue that was then, as now, one of the fundamental problems in neurology.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"103 10","pages":"e209959"},"PeriodicalIF":7.7,"publicationDate":"2024-11-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142471049","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: Fine Particulate Matter and Parkinson Disease Risk Among Medicare Beneficiaries.","authors":"","doi":"10.1212/WNL.0000000000210092","DOIUrl":"https://doi.org/10.1212/WNL.0000000000210092","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"103 10","pages":"e210092"},"PeriodicalIF":7.7,"publicationDate":"2024-11-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142504907","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: Breaking Bad CIDP: Recognition of Anti-NF155 Autoimmune Nodopathy in Refractory CIDP.","authors":"Roopa Sharma, Nicholas J Bellacicco, Walter G Husar, James H Park, Eric Lancaster, Madeline Singer","doi":"10.1212/WNL.0000000000209848","DOIUrl":"10.1212/WNL.0000000000209848","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"103 10","pages":"e209848"},"PeriodicalIF":7.7,"publicationDate":"2024-11-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11503473/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142522525","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":"What Are the Central Mechanisms of Cough and Their Neurologic Implications?","authors":"Reece M Hass, Eduardo E Benarroch","doi":"10.1212/WNL.0000000000210064","DOIUrl":"https://doi.org/10.1212/WNL.0000000000210064","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"103 10","pages":"e210064"},"PeriodicalIF":7.7,"publicationDate":"2024-11-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142605806","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":"Health-Related Behaviors and Risk of Common Age-Related Brain Diseases Across Severities of Genetic Risk.","authors":"Sandro Marini, Tamara N Kimball, Ernst Mayerhofer, Reinier W P Tack, Jasper R Senff, Savvina Prapiadou, Cyprien A Rivier, Jonathan Duskin, Christina Kourkoulis, Guido J Falcone, Nirupama Yechoor, Rudolph E Tanzi, Jonathan Rosand, Sanjula Singh, Livia Parodi, Christopher D Anderson","doi":"10.1212/WNL.0000000000210014","DOIUrl":"https://doi.org/10.1212/WNL.0000000000210014","url":null,"abstract":"<p><strong>Background and objectives: </strong>The 21-point Brain Care Score (BCS) is an index that ranks behaviors and clinical measurements with the aim of encouraging lifestyle adjustments to lower the incidence of age-related brain disease, including stroke, late-life depression (LLD), and dementia. A higher BCS at baseline is associated with a lower risk of these outcomes. We aimed to investigate whether the associations between BCS and stroke, LLD, and dementia risks are independent of genetic predisposition for these conditions and quantify the effect of healthy lifestyle across genetic risk distributions for these outcomes.</p><p><strong>Methods: </strong>Using the UK Biobank (UKB) prospective cohort study, we computed baseline BCSs and polygenic scores to estimate genetic predisposition for stroke and LLD and <i>APOE</i> ε allele status to stratify dementia risk. As for outcomes again in UKB, we measured incidence of stroke, LLD, and dementia. We used multivariate Cox proportional hazard models to assess associations between BCS, genetic predisposition, and these outcomes. We also conducted stratified and interaction analyses to estimate the incidence of these outcomes across quartiles of genetic risk and BCS.</p><p><strong>Results: </strong>We included 368,340 UKB participants (median age 58 years (interquartile range 51-63 years), 46.3% male). Independent of genetic risk, a 5-point increase in BCS corresponded to lowered hazards of stroke (hazard ratio [HR] 0.70, 95% CI 0.68-0.73), LLD (HR 0.65, 95% CI 0.63-0.67), and dementia (HR 0.82, 95% CI 0.78-0.85). Incidences of all 3 outcomes were higher among participants with high genetic risk of these outcomes. However, these increased risks were offset for individuals with a higher BCS (incidence rates per 1,000 person-years were 2.76 vs 1.19 for stroke, 7.34 vs 4.46 for LLD, and 3.64 vs 2.05 for dementia, when comparing low and high BCS).</p><p><strong>Discussion: </strong>Across different genetic predispositions for stroke, LLD, and dementia, healthier lifestyle behaviors are protective for brain health, demonstrating the nondeterminism of genetic risk. Furthermore, differences in BCS behave as aggregate risk estimators of all 3 outcomes. Further work is needed to prospectively investigate the utility and performance of the BCS as a targeted intervention in populations at elevated genetic risk of age-related brain disease.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"103 10","pages":"e210014"},"PeriodicalIF":7.7,"publicationDate":"2024-11-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142591260","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":"Acute Postradiation Lumbosacral Plexopathy.","authors":"Eleftheria Koropouli, Charalampos Leloudas, Nikolaos-Achilleas Arkoudis, Ariadne Daponte, Georgios Velonakis, Dimitra Tzavella, Panagiotis Kokotis, Vasiliki Zouvelou, Michail Rentzos","doi":"10.1212/WNL.0000000000209972","DOIUrl":"https://doi.org/10.1212/WNL.0000000000209972","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"103 10","pages":"e209972"},"PeriodicalIF":7.7,"publicationDate":"2024-11-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142504906","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":"No Man's Land.","authors":"Eunice Cho","doi":"10.1212/WNL.0000000000210028","DOIUrl":"https://doi.org/10.1212/WNL.0000000000210028","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"103 10","pages":"e210028"},"PeriodicalIF":7.7,"publicationDate":"2024-11-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142504908","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":"Costs Are Still on the Rise for Commonly Prescribed Branded Neurologic Medications.","authors":"Amanda V Gusovsky, Chun Chieh Lin, Kevin Kerber, Evan L Reynolds, Brian C Callaghan, James F Burke","doi":"10.1212/WNL.0000000000210029","DOIUrl":"https://doi.org/10.1212/WNL.0000000000210029","url":null,"abstract":"<p><strong>Objectives: </strong>To observe medication cost trends for 5 common neurologic conditions.</p><p><strong>Methods: </strong>We quantified annual out-of-pocket (OOP) and total medication costs for patients seen by a neurologist with epilepsy, multiple sclerosis (MS), Parkinson disease (PD), peripheral neuropathy (PN), and dementia/Alzheimer's disease in a commercial claims database cross-sectionally from 2012 to 2021.</p><p><strong>Results: </strong>We identified 186,144 patients with epilepsy, 54,676 with MS, 45,909 with PD, 169,127 with PN, and 60,861 with dementia/Alzheimer. OOP costs for MS medications increased each year, by 217% on average. Branded epilepsy medications had higher OOP costs than generics. Decreases ranging from 48% to 80% in annual OOP costs of duloxetine, pregabalin, rasagiline, rivastigmine, and memantine were observed in the years after generic introduction.</p><p><strong>Discussion: </strong>Preferentially selecting generic medications reduces OOP costs, other than for MS where costs continue to increase. Policy solutions, such as cost caps, are needed.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"103 10","pages":"e210029"},"PeriodicalIF":7.7,"publicationDate":"2024-11-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142546545","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}