Neurology最新文献

{"title":"Leukoencephalopathy With Calcifications and Cysts.","authors":"Felipe J Jones, Holly Elser, Adriana Mendez, Jamie L Fraser, Jennifer Orthmann-Murphy","doi":"10.1212/WNL.0000000000209936","DOIUrl":"https://doi.org/10.1212/WNL.0000000000209936","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"103 9","pages":"e209936"},"PeriodicalIF":7.7,"publicationDate":"2024-11-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142350943","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":"Thomas D. Sabin, MD, FAAN (1936-2024).","authors":"Peter R Bergethon, Allan Ropper, Thomas Swift","doi":"10.1212/WNL.0000000000209960","DOIUrl":"https://doi.org/10.1212/WNL.0000000000209960","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"103 9","pages":"e209960"},"PeriodicalIF":7.7,"publicationDate":"2024-11-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142350950","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":"End of Life in Neurodegenerative Diseases: An Unrecognized Opportunity for Better Care.","authors":"Jocelyn M Jiao, Delaram Safarpour","doi":"10.1212/WNL.0000000000210072","DOIUrl":"https://doi.org/10.1212/WNL.0000000000210072","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"103 9","pages":"e210072"},"PeriodicalIF":7.7,"publicationDate":"2024-11-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142406659","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: Teaching NeuroImage: Anti-NMDA Receptor Encephalitis Presenting With Cerebellitis in a Pediatric Patient.","authors":"James E Siegler, Steven L Galetta","doi":"10.1212/WNL.0000000000209934","DOIUrl":"10.1212/WNL.0000000000209934","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"103 9","pages":"e209934"},"PeriodicalIF":7.7,"publicationDate":"2024-11-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142375753","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":"Eculizumab Use in Neuromyelitis Optica Spectrum Disorders: Routine Clinical Care Data From a European Cohort.","authors":"Marius Ringelstein, Susanna Asseyer, Gero Lindenblatt, Katinka Fischer, Refik Pul, Jelena Skuljec, Lisa Revie, Katrin Giglhuber, Vivien Häußler, Michael Karenfort, Kerstin Hellwig, Friedemann Paul, Judith Bellmann-Strobl, Carolin Otto, Klemens Ruprecht, Tjalf Ziemssen, Alexander Emmer, Veit Rothhammer, Florian T Nickel, Klemens Angstwurm, Ralf Linker, Sarah A Laurent, Clemens Warnke, Sven Jarius, Mirjam Korporal-Kuhnke, Brigitte Wildemann, Stephanie Wolff, Maria Seipelt, Yavor Yalachkov, Nele Retzlaff, Uwe K Zettl, Paulus S Rommer, Markus C Kowarik, Jonathan Wickel, Christian Geis, Martin W Hümmert, Corinna Trebst, Makbule Senel, Ralf Gold, Luisa Klotz, Christoph Kleinschnitz, Sven G Meuth, Orhan Aktas, Achim Berthele, Ilya Ayzenberg","doi":"10.1212/WNL.0000000000209888","DOIUrl":"10.1212/WNL.0000000000209888","url":null,"abstract":"<p><strong>Background and objectives: </strong>Attack prevention is crucial in managing neuromyelitis optica spectrum disorders (NMOSDs). Eculizumab (ECU), an inhibitor of the terminal complement cascade, was highly effective in preventing attacks in a phase III trial of aquaporin-4 (AQP4)-IgG seropositive(+) NMOSDs. In this article, we evaluated effectiveness and safety of ECU in routine clinical care.</p><p><strong>Methods: </strong>We retrospectively evaluated patients with AQP4-IgG+ NMOSD treated with ECU between December 2014 and April 2022 at 20 German and 1 Austrian university center(s) of the Neuromyelitis Optica Study Group (NEMOS) by chart review. Primary outcomes were effectiveness (assessed using annualized attack rate [AAR], MRI activity, and disability changes [Expanded Disability Status Scale {EDSS}]) and safety (including adverse events, mortality, and attacks after meningococcal vaccinations), analyzed by descriptive statistics.</p><p><strong>Results: </strong>Fifty-two patients (87% female, age 55.0 ± 16.3 years) received ECU for 16.2 (interquartile range [IQR] 9.6 - 21.7) months. Forty-five patients (87%) received meningococcal vaccination before starting ECU, 9 with concomitant oral prednisone and 36 without. Seven of the latter (19%) experienced attacks shortly after vaccination (median: 9 days, IQR 6-10 days). No postvaccinal attack occurred in the 9 patients vaccinated while on oral prednisone before starting ECU and in 25 (re-)vaccinated while on ECU. During ECU therapy, 88% of patients were attack-free. The median AAR decreased from 1.0 (range 0-4) in the 2 years preceding ECU to 0 (range 0-0.8; <i>p</i> < 0.001). The EDSS score from start to the last follow-up was stable (median 6.0), and the proportion of patients with new T2-enhancing or gadolinium-enhancing MRI lesions in the brain and spinal cord decreased. Seven patients (13%) experienced serious infections. Five patients (10%; median age 53.7 years) died on ECU treatment (1 from myocardial infarction, 1 from ileus with secondary sepsis, and 3 from systemic infection, including 1 meningococcal sepsis), 4 were older than 60 years and severely disabled at ECU treatment start (EDSS score ≥ 7). The overall discontinuation rate was 19%.</p><p><strong>Discussion: </strong>Eculizumab proved to be effective in preventing NMOSD attacks. An increased risk of attacks after meningococcal vaccination before ECU start and potentially fatal systemic infections during ECU-particularly in patients with comorbidities-must be considered. Further research is necessary to explore optimal timing for meningococcal vaccinations.</p><p><strong>Classification of evidence: </strong>This study provides Class IV evidence that eculizumab reduces annualized attack rates and new MRI lesions in AQP4-IgG+ patients with NMOSD.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"103 9","pages":"e209888"},"PeriodicalIF":7.7,"publicationDate":"2024-11-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11449397/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142365834","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":"End-of-Life Health Care Service Use and Cost Among Medicare Decedents With Neurodegenerative Diseases.","authors":"Whitley W Aamodt, Chuxuan Sun, Nabila Dahodwala, Holly Elser, Andrea L C Schneider, John T Farrar, Norma B Coe, Allison W Willis","doi":"10.1212/WNL.0000000000209925","DOIUrl":"10.1212/WNL.0000000000209925","url":null,"abstract":"<p><strong>Background and objectives: </strong>Although neurodegenerative diseases are a leading cause of death, little is known about health care utilization and cost during the end-of-life (EoL) period or how it compares with that of other life-limiting conditions. We aimed to describe and compare resource utilization among US Medicare decedents with neurodegenerative diseases with decedents with cancer.</p><p><strong>Methods: </strong>We conducted a retrospective study of Medicare Part A and B beneficiaries with Alzheimer disease (AD), Parkinson disease (PD), or amyotrophic lateral sclerosis (ALS) who died in 2018. Decedents diagnosed with malignant brain tumors or pancreatic cancer served as non-neurodegenerative comparators. Descriptive analyses examined demographic and clinical characteristics in the last year of life. The probabilities and associated costs of emergency department (ED), inpatient, skilled nursing facility (SNF), and hospice utilization during the last 12 and 6 months of life were also compared between persons with neurodegenerative diseases and cancer, adjusting for sociodemographic factors and comorbidity burden.</p><p><strong>Results: </strong>A total of 1,126,799 Medicare beneficiaries died in 2018, of which 357,926 had a qualifying diagnosis. Persons with neurodegenerative diseases were older and more frequently received Medicaid assistance than persons with brain or pancreatic cancer. In all groups, health care service utilization increased over the last year of life, and total costs were predominantly attributable to inpatient care. In the last 6 months of life, neurologist care was infrequent among patients with neurodegenerative disease (AD: 1.5%; PD: 8.6%; ALS: 32.0%). Persons with neurodegenerative diseases as compared to persons with malignant brain tumors also had greater odds of ED use (AD: adjusted odds ratio [aOR] 1.17, 95% CI 1.11-1.23; PD: aOR 1.18, 95% CI 1.11-1.25; ALS: aOR 1.11, 95% CI 1.01-1.23), lower odds of hospitalization (AD: aOR 0.64, 95% CI 0.60-0.68; PD: aOR 0.65, 95% CI 0.61-0.69; ALS: aOR 0.33, 95% CI 0.30-0.37), and lower odds of hospice enrollment (AD: aOR 0.33, 95% CI 0.31-0.36; PD: aOR 0.33, 95% CI 0.31-0.36; ALS: aOR 0.41, 95% CI 0.36-0.46). The findings were similar in pancreatic cancer.</p><p><strong>Discussion: </strong>Persons with neurodegenerative diseases in the United States are more likely to visit the ED and less likely to use inpatient and hospice services at EoL than persons with brain or pancreatic cancer. These group differences may stem from prognostic uncertainty and reflect inadequate EoL care practices, requiring further investigation to ensure more timely palliative care and hospice referrals.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"103 9","pages":"e209925"},"PeriodicalIF":7.7,"publicationDate":"2024-11-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11469682/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142406660","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":"Cancer Risk Among Patients With Multiple Sclerosis: A 10-Year Nationwide Retrospective Cohort Study.","authors":"Chloe Pierret, Aurelien Mulliez, Christine Le Bihan-Benjamin, Xavier Moisset, Philippe-Jean Bousquet, Emmanuelle Leray","doi":"10.1212/WNL.0000000000209885","DOIUrl":"10.1212/WNL.0000000000209885","url":null,"abstract":"<p><strong>Background and objectives: </strong>Previous literature has been diverging on cancer risk in people with multiple sclerosis (PwMS). Therefore, this study compared the risk of cancer in PwMS and a matched sample from the French general population.</p><p><strong>Methods: </strong>This 10-year nationwide retrospective matched cohort study (2012-2021) used data from the national French administrative health care database (99% coverage of the French population) to determine the time to the first incident cancer. PwMS were identified using their long-term disease (LTD) status, hospitalizations, and multiple sclerosis (MS)-specific drug reimbursements. The control population was matched 4:1 on age, sex, residence, insurance scheme, and cohort entry date. Participants were included if they had no history of cancer in the 3 years before inclusion. Patients with cancer were identified through LTD status, hospitalizations, chemotherapy, radiotherapy, or prostate cancer-specific drug reimbursements. Overall and cancer location-specific hazard ratios (HRs) for the first incident cancer were obtained from Fine and Gray models, and age- and sex-stratified estimates were reported. Participation in cancer screening through the 3 national programs (breast, colorectal, and cervical) were compared between groups.</p><p><strong>Results: </strong>Cancer incidence was 799 per 100,000 person-years (PYs) (n = 8,368) among the 140,649 PwMS and 736 per 100,000 PYs (n = 31,796) among the 562,596 matched controls (70.8% of women; follow-up: 7.6 ± 3.2 years). A small overall risk increase was observed for PwMS (HR 1.06, 95% CI 1.03-1.08), mostly in women (HR 1.08, 95% CI 1.05-1.11). Risk varied by cancer types and was lower for prostate (HR 0.80, 95% CI 0.73-0.88), breast (HR 0.91, 95% CI 0.86-0.95), and colorectal (HR 0.90, 95% CI 0.84-0.97) cancer and higher for bladder (HR 1.71, 95% CI 1.54-1.89), brain (HR 1.68, 95% CI 1.42-1.98), and cervical (HR 1.24, 95% CI 1.12-1.38) cancer in PwMS. Cancer risk was higher in PwMS younger than 55 years (HR 1.20, 95% CI 1.15-1.24) but decreased in PwMS aged 65 years and older (HR 0.89, 95% CI 0.85-0.94). This trend was found in all cancer locations. There were fewer PwMS getting screened than controls (all programs), with a particularly pronounced difference among those aged 65 years and older.</p><p><strong>Discussion: </strong>Cancer risk was slightly increased in PwMS, particularly for urogenital cancers, possibly due to surveillance bias. Risk fluctuated depending on age, perhaps due to varying generational screening practices (i.e., diagnosis neglect in the older PwMS) and risk factors.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"103 9","pages":"e209885"},"PeriodicalIF":7.7,"publicationDate":"2024-11-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11464044/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142392120","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":"Litany of Things Left Unsaid.","authors":"Elane Kim","doi":"10.1212/WNL.0000000000209987","DOIUrl":"https://doi.org/10.1212/WNL.0000000000209987","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"103 9","pages":"e209987"},"PeriodicalIF":7.7,"publicationDate":"2024-11-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142350944","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":"Alzheimer Disease Is a Specific Disorder Defined by Neuropathology Detectable During Life.","authors":"William J Jagust","doi":"10.1212/WNL.0000000000209995","DOIUrl":"https://doi.org/10.1212/WNL.0000000000209995","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"103 9","pages":"e209995"},"PeriodicalIF":7.7,"publicationDate":"2024-11-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142365931","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":"Challenges in a Biological Definition of Alzheimer Disease.","authors":"Jemma Hazan, Kathy Y Liu, Harry Costello, Jeremy D Isaacs, Madhav Thambisetty, Robert Howard","doi":"10.1212/WNL.0000000000209884","DOIUrl":"10.1212/WNL.0000000000209884","url":null,"abstract":"<p><p>It has been suggested that the diagnostic landscape of Alzheimer disease (AD) is undergoing a profound transformation, marked by a shift toward a biomarker-based approach, as proposed by the Revised Criteria for Diagnosis and Staging of Alzheimer's Disease. These criteria advocate for diagnosing AD solely on biomarkers, without requiring clinical symptoms. This article explores the drivers behind this transition, primarily influenced by the Food and Drug Administration's approval of amyloid-lowering treatments. We evaluate the proposed criteria, which allow for an AD diagnosis based on amyloid \"A\" or phosphorylated tau \"T1\" positivity through surrogate amyloid PET imaging, CSF, or plasma biomarkers, and consider the arguments for and against their use. The merits of the new criteria include a clearer definition of AD, which is currently used interchangeably to refer to both the presence of neuropathology and the clinical syndrome. We argue that a purely biological definition risks a category error and emphasize the need for longitudinal data to establish the lifetime risk of dementia in amyloid-positive and tau-positive individuals. We also caution against limiting the scope of biomarker-based AD diagnosis to amyloid and tau alone. In conclusion, we recommend that the criteria remain within the research domain for the present while advocating for the considered adoption of plasma biomarkers in clinical practice.</p>","PeriodicalId":19256,"journal":{"name":"Neurology","volume":"103 9","pages":"e209884"},"PeriodicalIF":7.7,"publicationDate":"2024-11-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11449396/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142365932","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}