Pub Date : 2024-11-12Epub Date: 2024-09-30DOI: 10.1212/WNL.0000000000209967
Rogan Magee, Yombe Fonkeu, Lamya Ibrahim, Jae W Song, Steven R Messe
{"title":"Teaching NeuroImage: Basilar Artery Septation With Nodularity.","authors":"Rogan Magee, Yombe Fonkeu, Lamya Ibrahim, Jae W Song, Steven R Messe","doi":"10.1212/WNL.0000000000209967","DOIUrl":"https://doi.org/10.1212/WNL.0000000000209967","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":null,"pages":null},"PeriodicalIF":7.7,"publicationDate":"2024-11-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142350947","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}
Pub Date : 2024-11-12Epub Date: 2024-09-30DOI: 10.1212/WNL.0000000000209936
Felipe J Jones, Holly Elser, Adriana Mendez, Jamie L Fraser, Jennifer Orthmann-Murphy
{"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":null,"pages":null},"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}
Pub Date : 2024-11-12Epub Date: 2024-09-30DOI: 10.1212/WNL.0000000000209960
Peter R Bergethon, Allan Ropper, Thomas Swift
{"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":null,"pages":null},"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}
Pub Date : 2024-11-12Epub Date: 2024-10-01DOI: 10.1212/WNL.0000000000209888
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
Background and objectives: 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.
Methods: 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.
Results: 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; p < 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%.
Discussion: 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.
Classification of evidence: This study provides Class IV evidence that eculizumab reduces annualized attack rates and new MRI lesions in AQP4-IgG+ patients with NMOSD.
{"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":null,"pages":null},"PeriodicalIF":7.7,"publicationDate":"2024-11-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","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":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-11-12Epub Date: 2024-09-27DOI: 10.1212/WNL.0000000000209987
Elane Kim
{"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":null,"pages":null},"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}
Pub Date : 2024-11-12Epub Date: 2024-10-01DOI: 10.1212/WNL.0000000000209995
William J Jagust
{"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":null,"pages":null},"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}
Pub Date : 2024-11-12Epub Date: 2024-10-01DOI: 10.1212/WNL.0000000000209884
Jemma Hazan, Kathy Y Liu, Harry Costello, Jeremy D Isaacs, Madhav Thambisetty, Robert Howard
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.
有观点认为，阿尔茨海默病（AD）的诊断方式正在经历一场深刻的变革，其标志是向基于生物标志物的方法转变，正如《阿尔茨海默病诊断和分期修订标准》所提出的那样。这些标准主张仅根据生物标志物诊断阿尔茨海默病，而无需临床症状。本文探讨了这一转变背后的驱动因素，主要是受美国食品药品管理局批准降低淀粉样蛋白治疗的影响。我们对提出的标准进行了评估，这些标准允许通过替代淀粉样蛋白 PET 成像、脑脊液或血浆生物标记物，根据淀粉样蛋白 "A "或磷酸化 tau "T1 "阳性来诊断 AD，并考虑了支持和反对使用这些标准的论点。新标准的优点包括对AD的定义更加明确，目前AD的定义被交替使用，既指神经病理学的存在，也指临床综合征。我们认为，纯生物学定义有可能造成分类错误，并强调需要纵向数据来确定淀粉样蛋白阳性和 tau 阳性个体终生罹患痴呆症的风险。我们还告诫不要将基于生物标志物的AD诊断范围仅仅局限于淀粉样蛋白和tau。总之，我们建议目前仍将该标准应用于研究领域，同时提倡在临床实践中考虑采用血浆生物标记物。
{"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":null,"pages":null},"PeriodicalIF":7.7,"publicationDate":"2024-11-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","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":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-11-12Epub Date: 2024-09-30DOI: 10.1212/WNL.0000000000209968
{"title":"Predicting Outcome in Guillain-Barré Syndrome: International Validation of the Modified Erasmus GBS Outcome Score.","authors":"","doi":"10.1212/WNL.0000000000209968","DOIUrl":"https://doi.org/10.1212/WNL.0000000000209968","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":null,"pages":null},"PeriodicalIF":7.7,"publicationDate":"2024-11-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142350945","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}
Pub Date : 2024-11-12Epub Date: 2024-09-27DOI: 10.1212/WNL.0000000000209931
Aaron S Zelikovich, Safa Kaleem, Gary Kocharian, Nalini Tata, Alison Seitz, Alexander E Merkler
{"title":"Teaching NeuroImage: Hemorrhagic Arachnoid Cyst.","authors":"Aaron S Zelikovich, Safa Kaleem, Gary Kocharian, Nalini Tata, Alison Seitz, Alexander E Merkler","doi":"10.1212/WNL.0000000000209931","DOIUrl":"https://doi.org/10.1212/WNL.0000000000209931","url":null,"abstract":"","PeriodicalId":19256,"journal":{"name":"Neurology","volume":null,"pages":null},"PeriodicalIF":7.7,"publicationDate":"2024-11-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142350948","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}
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