Pub Date : 2024-09-16DOI: 10.1001/jamaneurol.2024.3062
Le H Hua, Andrew J Solomon, Silvia Tenembaum, Antonio Scalfari, Àlex Rovira, Kevin Rostasy, Scott D Newsome, Ruth Ann Marrie, Melinda Magyari, Orhun Kantarci, Bernhard Hemmer, Cheryl Hemingway, Mary Pat Harnegie, Jennifer S Graves, Jeffrey A Cohen, Riley Bove, Brenda Banwell, John R Corboy, Emmanuelle Waubant
Importance: While the typical onset of multiple sclerosis (MS) occurs in early adulthood, 2% to 10% of cases initially present prior to age 18 years, and approximately 5% after age 50 years. Guidance on approaches to differential diagnosis in suspected MS specific to these 2 age groups is needed.
Observations: There are unique biological factors in children younger than 18 years and in adults older than age 50 years compared to typical adult-onset MS. These biological differences, particularly immunological and hormonal, may influence the clinical presentation of MS, resilience to neuronal injury, and differential diagnosis. While mimics of MS at the typical age at onset have been described, a comprehensive approach focused on the younger and older ends of the age spectrum has not been previously published.
Conclusions and relevance: An international committee of MS experts in pediatric and adult MS was formed to provide consensus guidance on diagnostic approaches and key clinical and paraclinical red flags for non-MS diagnosis in children and older adults.
{"title":"Differential Diagnosis of Suspected Multiple Sclerosis in Pediatric and Late-Onset Populations: A Review.","authors":"Le H Hua, Andrew J Solomon, Silvia Tenembaum, Antonio Scalfari, Àlex Rovira, Kevin Rostasy, Scott D Newsome, Ruth Ann Marrie, Melinda Magyari, Orhun Kantarci, Bernhard Hemmer, Cheryl Hemingway, Mary Pat Harnegie, Jennifer S Graves, Jeffrey A Cohen, Riley Bove, Brenda Banwell, John R Corboy, Emmanuelle Waubant","doi":"10.1001/jamaneurol.2024.3062","DOIUrl":"https://doi.org/10.1001/jamaneurol.2024.3062","url":null,"abstract":"<p><strong>Importance: </strong>While the typical onset of multiple sclerosis (MS) occurs in early adulthood, 2% to 10% of cases initially present prior to age 18 years, and approximately 5% after age 50 years. Guidance on approaches to differential diagnosis in suspected MS specific to these 2 age groups is needed.</p><p><strong>Observations: </strong>There are unique biological factors in children younger than 18 years and in adults older than age 50 years compared to typical adult-onset MS. These biological differences, particularly immunological and hormonal, may influence the clinical presentation of MS, resilience to neuronal injury, and differential diagnosis. While mimics of MS at the typical age at onset have been described, a comprehensive approach focused on the younger and older ends of the age spectrum has not been previously published.</p><p><strong>Conclusions and relevance: </strong>An international committee of MS experts in pediatric and adult MS was formed to provide consensus guidance on diagnostic approaches and key clinical and paraclinical red flags for non-MS diagnosis in children and older adults.</p>","PeriodicalId":14677,"journal":{"name":"JAMA neurology","volume":" ","pages":""},"PeriodicalIF":20.4,"publicationDate":"2024-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142287433","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 : 2024-09-16DOI: 10.1001/jamaneurol.2024.3018
James J Giordano, Michael S Okun
{"title":"The Nontrivial Ethics of Brain Biopsies for Research.","authors":"James J Giordano, Michael S Okun","doi":"10.1001/jamaneurol.2024.3018","DOIUrl":"https://doi.org/10.1001/jamaneurol.2024.3018","url":null,"abstract":"","PeriodicalId":14677,"journal":{"name":"JAMA neurology","volume":" ","pages":""},"PeriodicalIF":20.4,"publicationDate":"2024-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142287439","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 : 2024-09-03DOI: 10.1001/jamaneurol.2024.2780
Augusto Rachão, Pedro Pereira, Miguel Grunho
{"title":"A Unique Case of Opioid-Induced Myoclonus.","authors":"Augusto Rachão, Pedro Pereira, Miguel Grunho","doi":"10.1001/jamaneurol.2024.2780","DOIUrl":"https://doi.org/10.1001/jamaneurol.2024.2780","url":null,"abstract":"","PeriodicalId":14677,"journal":{"name":"JAMA neurology","volume":" ","pages":""},"PeriodicalIF":20.4,"publicationDate":"2024-09-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142119818","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 : 2024-09-01DOI: 10.1001/jamaneurol.2024.1105
Maria Neimann Herskind, Jakob Bie Granild-Jensen, Mette Thorup Bendixen
{"title":"Distinct Magnetic Resonance Imaging in a Child With a TACO1 Variant.","authors":"Maria Neimann Herskind, Jakob Bie Granild-Jensen, Mette Thorup Bendixen","doi":"10.1001/jamaneurol.2024.1105","DOIUrl":"10.1001/jamaneurol.2024.1105","url":null,"abstract":"","PeriodicalId":14677,"journal":{"name":"JAMA neurology","volume":" ","pages":"1002-1003"},"PeriodicalIF":20.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140857122","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 : 2024-09-01DOI: 10.1001/jamaneurol.2024.2393
Daniel Macías-García, Marta Méndez-Del Barrio, Manuel Canal-Rivero, Laura Muñoz-Delgado, Astrid Adarmes-Gómez, Silvia Jesús, Elena Ojeda-Lepe, Fátima Carrillo-García, Francisco J Palomar, Francisco Javier Gómez-Campos, Juan Francisco Martin-Rodriguez, Benedicto Crespo-Facorro, Miguel Ruiz-Veguilla, Pablo Mir
<p><strong>Importance: </strong>Functional movement disorders (FMDs) are frequent and disabling neurological disorders with a substantial socioeconomic impact. Few randomized studies have analyzed the effectiveness of combined physiotherapy and psychotherapy in patients' quality of life.</p><p><strong>Objective: </strong>To assess the efficacy of multidisciplinary treatment (physiotherapy plus cognitive behavioral therapy) in FMDs.</p><p><strong>Design, setting, and participants: </strong>This was a parallel, rater-blinded, single-center, randomized clinical trial. Recruitment took place from June 2022 to April 2023, and follow-up visits were performed at months 3 and 5, concluding in October 2023. Participants were recruited from a national referral center for movement disorders: the Movement Disorders Unit from the Hospital Universitario Virgen Rocio in Seville, Spain. Patients had to be 18 years or older with a confirmed FMD diagnosis and capable of giving consent to participate. Patients who did not meet eligibility criteria or refused to participate were excluded. Any uncontrolled psychiatric disorder was considered an exclusion criterion.</p><p><strong>Interventions: </strong>Patients were randomly assigned, in a ratio of 1:1 to multidisciplinary treatment (physiotherapy plus cognitive behavioral therapy), or a control intervention (psychological support intervention).</p><p><strong>Main outcomes and measures: </strong>Primary outcomes: between-group differences in changes from baseline to month 3 and month 5 in patients' quality of life (EQ-5D-5L score: EQ Index and EQ visual analog scale [EQ VAS]; and 36-Item Short-Form Survey Physical Component Summary [SF-36 PCS] and SF-36 Mental Component Summary [MCS]). Linear mixed models were applied, controlling by baseline severity and applying Bonferroni correction.</p><p><strong>Results: </strong>Of 70 patients screened with an FMD, 40 were enrolled (mean [SD] age, 43.5 [12.8] years; age range, 18-66 years; 32 female [80%]; mean [SD] age at FMD onset, 38.4 [12.1] years), and 38 completed all the follow-up visits and were included in the analysis for primary outcomes. Multidisciplinary treatment improved SF-36 PCS with a mean between-group difference at 3 months of 4.23 points (95% CI, -0.9 to 9.4 points; P = .11) and a significant mean between-group difference at 5 months of 5.62 points (95% CI, 2.3-8.9 points; P < .001), after multiple-comparisons adjustment. There were no significant differences in other quality-of-life outcomes such as SF-36 MCS (mean between-group difference at 3 and 5 months: 0.72 points; 95% CI, -5.5 to 7.0 points; P = .82 and 0.69 points; 95% CI, 2.3-8.9 points; P = .83, respectively), EQ VAS (9.34 points; 95% CI, -0.6 to 19.3 points; P = .07 and 13.7 points; 95% CI, -1.7 to 29.0 points; P = .09, respectively) and EQ Index (0.001 point; 95% CI, -0.1 to 0.1 point; P = .98 and 0.08 points; 95% CI, 0-0.2 points; P = .13, respectively). At months 3 and 5, 42% and 47% of patie
{"title":"Combined Physiotherapy and Cognitive Behavioral Therapy for Functional Movement Disorders: A Randomized Clinical Trial.","authors":"Daniel Macías-García, Marta Méndez-Del Barrio, Manuel Canal-Rivero, Laura Muñoz-Delgado, Astrid Adarmes-Gómez, Silvia Jesús, Elena Ojeda-Lepe, Fátima Carrillo-García, Francisco J Palomar, Francisco Javier Gómez-Campos, Juan Francisco Martin-Rodriguez, Benedicto Crespo-Facorro, Miguel Ruiz-Veguilla, Pablo Mir","doi":"10.1001/jamaneurol.2024.2393","DOIUrl":"10.1001/jamaneurol.2024.2393","url":null,"abstract":"<p><strong>Importance: </strong>Functional movement disorders (FMDs) are frequent and disabling neurological disorders with a substantial socioeconomic impact. Few randomized studies have analyzed the effectiveness of combined physiotherapy and psychotherapy in patients' quality of life.</p><p><strong>Objective: </strong>To assess the efficacy of multidisciplinary treatment (physiotherapy plus cognitive behavioral therapy) in FMDs.</p><p><strong>Design, setting, and participants: </strong>This was a parallel, rater-blinded, single-center, randomized clinical trial. Recruitment took place from June 2022 to April 2023, and follow-up visits were performed at months 3 and 5, concluding in October 2023. Participants were recruited from a national referral center for movement disorders: the Movement Disorders Unit from the Hospital Universitario Virgen Rocio in Seville, Spain. Patients had to be 18 years or older with a confirmed FMD diagnosis and capable of giving consent to participate. Patients who did not meet eligibility criteria or refused to participate were excluded. Any uncontrolled psychiatric disorder was considered an exclusion criterion.</p><p><strong>Interventions: </strong>Patients were randomly assigned, in a ratio of 1:1 to multidisciplinary treatment (physiotherapy plus cognitive behavioral therapy), or a control intervention (psychological support intervention).</p><p><strong>Main outcomes and measures: </strong>Primary outcomes: between-group differences in changes from baseline to month 3 and month 5 in patients' quality of life (EQ-5D-5L score: EQ Index and EQ visual analog scale [EQ VAS]; and 36-Item Short-Form Survey Physical Component Summary [SF-36 PCS] and SF-36 Mental Component Summary [MCS]). Linear mixed models were applied, controlling by baseline severity and applying Bonferroni correction.</p><p><strong>Results: </strong>Of 70 patients screened with an FMD, 40 were enrolled (mean [SD] age, 43.5 [12.8] years; age range, 18-66 years; 32 female [80%]; mean [SD] age at FMD onset, 38.4 [12.1] years), and 38 completed all the follow-up visits and were included in the analysis for primary outcomes. Multidisciplinary treatment improved SF-36 PCS with a mean between-group difference at 3 months of 4.23 points (95% CI, -0.9 to 9.4 points; P = .11) and a significant mean between-group difference at 5 months of 5.62 points (95% CI, 2.3-8.9 points; P < .001), after multiple-comparisons adjustment. There were no significant differences in other quality-of-life outcomes such as SF-36 MCS (mean between-group difference at 3 and 5 months: 0.72 points; 95% CI, -5.5 to 7.0 points; P = .82 and 0.69 points; 95% CI, 2.3-8.9 points; P = .83, respectively), EQ VAS (9.34 points; 95% CI, -0.6 to 19.3 points; P = .07 and 13.7 points; 95% CI, -1.7 to 29.0 points; P = .09, respectively) and EQ Index (0.001 point; 95% CI, -0.1 to 0.1 point; P = .98 and 0.08 points; 95% CI, 0-0.2 points; P = .13, respectively). At months 3 and 5, 42% and 47% of patie","PeriodicalId":14677,"journal":{"name":"JAMA neurology","volume":" ","pages":"966-976"},"PeriodicalIF":20.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11385055/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141889264","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}
Pub Date : 2024-09-01DOI: 10.1001/jamaneurol.2024.2404
Babak B Navi, Cenai Zhang, Benjamin Miller, Mary Cushman, Scott E Kasner, Mitchell S V Elkind, David L Tirschwell, W T Longstreth, Richard A Kronmal, Morin Beyeler, Jordan Elm, Richard M Zweifler, Joseph Tarsia, Carlo W Cereda, Giovanni Bianco, Gianluca Costamagna, Patrik Michel, Joseph P Broderick, David J Gladstone, Hooman Kamel, Christopher Streib
Importance: Approximately 10% to 15% of ischemic strokes are associated with cancer; cancer-associated stroke, particularly when cryptogenic, is associated with high rates of recurrent stroke and major bleeding. Limited data exist on the safety and efficacy of different antithrombotic strategies in patients with cancer and cryptogenic stroke.
Objective: To compare apixaban vs aspirin for the prevention of adverse clinical outcomes in patients with history of cancer and cryptogenic stroke.
Design, setting, and participants: Post hoc analysis of data from 1015 patients with a recent cryptogenic stroke and biomarker evidence of atrial cardiopathy in the Atrial Cardiopathy and Antithrombotic Drugs in Prevention After Cryptogenic Stroke (ARCADIA) trial, a multicenter, randomized, double-blind clinical trial conducted from 2018 to 2023 at 185 stroke centers in North America. Data analysis was performed from October 15, 2023, to May 23, 2024.
Exposures: Oral apixaban, 5 mg (or 2.5 mg if criteria met), twice daily vs oral aspirin, 81 mg, once daily. Subgroups of patients with and without cancer at baseline were examined.
Main outcomes and measures: The primary outcome for this post hoc analysis was a composite of major ischemic or major hemorrhagic events. Major ischemic events were recurrent ischemic stroke, myocardial infarction, systemic embolism, and symptomatic deep vein thrombosis or pulmonary embolism. Major hemorrhagic events included symptomatic intracranial hemorrhage and any major extracranial hemorrhage.
Results: Among 1015 participants (median [IQR] age, 68 [60-76] years; 551 [54.3%] female), 137 (13.5%) had a history of cancer. The median (IQR) follow-up was 1.5 (0.6-2.5) years for patients with history of cancer and 1.5 (0.6-3.0) years for those without history of cancer. Participants with history of cancer, compared with those without history of cancer, had a higher risk of major ischemic or major hemorrhagic events (hazard ratio [HR], 1.73; 95% CI, 1.10-2.71). Among those with history of cancer, 8 of 61 participants (13.1%) randomized to apixaban and 16 of 76 participants (21.1%) randomized to aspirin had a major ischemic or major hemorrhagic event; however, the risk was not significantly different between groups (HR, 0.61; 95% CI, 0.26-1.43). Comparing participants randomized to apixaban vs aspirin among those with cancer, events included recurrent stroke (5 [8.2%] vs 9 [11.8%]), major ischemic events (7 [11.5%] vs 14 [18.4%]), and major hemorrhagic events (1 [1.6%] vs 2 [2.6%]).
Conclusions and relevance: Among participants in the ARCADIA trial with history of cancer, the risk of major ischemic and hemorrhagic events did not differ significantly with apixaban compared with aspirin.
重要性:约 10%-15%的缺血性脑卒中与癌症有关;癌症相关脑卒中,尤其是隐源性脑卒中,复发率和大出血率很高。关于癌症和隐源性中风患者不同抗血栓策略的安全性和有效性的数据有限:比较阿哌沙班与阿司匹林在预防癌症史和隐源性卒中患者不良临床结局方面的疗效:对近期发生隐源性卒中且有心房性心脏病生物标志物证据的1015名患者的数据进行事后分析,这些患者参加了心房性心脏病和抗血栓药物预防隐源性卒中(ARCADIA)试验,该试验是一项多中心、随机、双盲临床试验,于2018年至2023年在北美185个卒中中心进行。数据分析从2023年10月15日开始,至2024年5月23日结束:口服阿哌沙班,5 毫克(或 2.5 毫克,如果符合标准),每天两次 vs 口服阿司匹林,81 毫克,每天一次。主要结果和测量指标:这项事后分析的主要结果是重大缺血或重大出血事件的复合结果。主要缺血性事件包括复发性缺血性中风、心肌梗死、全身性栓塞、症状性深静脉血栓或肺栓塞。主要出血事件包括症状性颅内出血和任何主要颅外出血:1015名参与者(中位数[IQR]年龄为68[60-76]岁;551[54.3%]名女性)中,137人(13.5%)有癌症病史。有癌症病史患者的随访中位数(IQR)为 1.5(0.6-2.5)年,无癌症病史患者的随访中位数(IQR)为 1.5(0.6-3.0)年。与无癌症病史者相比,有癌症病史者发生重大缺血或大出血事件的风险更高(危险比 [HR],1.73;95% CI,1.10-2.71)。在有癌症病史的参与者中,随机接受阿哌沙班治疗的61人中有8人(13.1%)和随机接受阿司匹林治疗的76人中有16人(21.1%)发生了严重缺血或大出血事件;但是,两组之间的风险没有显著差异(HR,0.61;95% CI,0.26-1.43)。在随机接受阿哌沙班与阿司匹林治疗的癌症患者中,发生的事件包括复发性中风(5 [8.2%] vs 9 [11.8%])、重大缺血性事件(7 [11.5%] vs 14 [18.4%])和重大出血事件(1 [1.6%] vs 2 [2.6%]):结论和相关性:在ARCADIA试验的参与者中,与阿司匹林相比,有癌症病史的阿哌沙班患者发生重大缺血性和出血性事件的风险没有显著差异:试验注册:ClinicalTrials.gov Identifier:试验注册:ClinicalTrials.gov Identifier:NCT03192215。
{"title":"Apixaban vs Aspirin in Patients With Cancer and Cryptogenic Stroke: A Post Hoc Analysis of the ARCADIA Randomized Clinical Trial.","authors":"Babak B Navi, Cenai Zhang, Benjamin Miller, Mary Cushman, Scott E Kasner, Mitchell S V Elkind, David L Tirschwell, W T Longstreth, Richard A Kronmal, Morin Beyeler, Jordan Elm, Richard M Zweifler, Joseph Tarsia, Carlo W Cereda, Giovanni Bianco, Gianluca Costamagna, Patrik Michel, Joseph P Broderick, David J Gladstone, Hooman Kamel, Christopher Streib","doi":"10.1001/jamaneurol.2024.2404","DOIUrl":"10.1001/jamaneurol.2024.2404","url":null,"abstract":"<p><strong>Importance: </strong>Approximately 10% to 15% of ischemic strokes are associated with cancer; cancer-associated stroke, particularly when cryptogenic, is associated with high rates of recurrent stroke and major bleeding. Limited data exist on the safety and efficacy of different antithrombotic strategies in patients with cancer and cryptogenic stroke.</p><p><strong>Objective: </strong>To compare apixaban vs aspirin for the prevention of adverse clinical outcomes in patients with history of cancer and cryptogenic stroke.</p><p><strong>Design, setting, and participants: </strong>Post hoc analysis of data from 1015 patients with a recent cryptogenic stroke and biomarker evidence of atrial cardiopathy in the Atrial Cardiopathy and Antithrombotic Drugs in Prevention After Cryptogenic Stroke (ARCADIA) trial, a multicenter, randomized, double-blind clinical trial conducted from 2018 to 2023 at 185 stroke centers in North America. Data analysis was performed from October 15, 2023, to May 23, 2024.</p><p><strong>Exposures: </strong>Oral apixaban, 5 mg (or 2.5 mg if criteria met), twice daily vs oral aspirin, 81 mg, once daily. Subgroups of patients with and without cancer at baseline were examined.</p><p><strong>Main outcomes and measures: </strong>The primary outcome for this post hoc analysis was a composite of major ischemic or major hemorrhagic events. Major ischemic events were recurrent ischemic stroke, myocardial infarction, systemic embolism, and symptomatic deep vein thrombosis or pulmonary embolism. Major hemorrhagic events included symptomatic intracranial hemorrhage and any major extracranial hemorrhage.</p><p><strong>Results: </strong>Among 1015 participants (median [IQR] age, 68 [60-76] years; 551 [54.3%] female), 137 (13.5%) had a history of cancer. The median (IQR) follow-up was 1.5 (0.6-2.5) years for patients with history of cancer and 1.5 (0.6-3.0) years for those without history of cancer. Participants with history of cancer, compared with those without history of cancer, had a higher risk of major ischemic or major hemorrhagic events (hazard ratio [HR], 1.73; 95% CI, 1.10-2.71). Among those with history of cancer, 8 of 61 participants (13.1%) randomized to apixaban and 16 of 76 participants (21.1%) randomized to aspirin had a major ischemic or major hemorrhagic event; however, the risk was not significantly different between groups (HR, 0.61; 95% CI, 0.26-1.43). Comparing participants randomized to apixaban vs aspirin among those with cancer, events included recurrent stroke (5 [8.2%] vs 9 [11.8%]), major ischemic events (7 [11.5%] vs 14 [18.4%]), and major hemorrhagic events (1 [1.6%] vs 2 [2.6%]).</p><p><strong>Conclusions and relevance: </strong>Among participants in the ARCADIA trial with history of cancer, the risk of major ischemic and hemorrhagic events did not differ significantly with apixaban compared with aspirin.</p><p><strong>Trial registration: </strong>ClinicalTrials.gov Identifier: NCT03192215.</p>","PeriodicalId":14677,"journal":{"name":"JAMA neurology","volume":" ","pages":"958-965"},"PeriodicalIF":20.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11320331/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141916691","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}
Pub Date : 2024-09-01DOI: 10.1001/jamaneurol.2024.2166
Jason W Adams, Daniel Kirsch, Samantha M Calderazzo, Fatima Tuz-Zahra, Yorghos Tripodis, Jesse Mez, Michael L Alosco, Victor E Alvarez, Bertrand R Huber, Caroline Kubilus, Kerry A Cormier, Raymond Nicks, Madeline Uretsky, Evan Nair, Eva Kuzyk, Nurgul Aytan, Jonathan D Cherry, John F Crary, Daniel H Daneshvar, Christopher J Nowinski, Lee E Goldstein, Brigid Dwyer, Douglas I Katz, Robert C Cantu, Robert A Stern, Ann C McKee, Thor D Stein
<p><strong>Importance: </strong>Parkinsonism is associated with traumatic brain injury and chronic traumatic encephalopathy (CTE), a neurodegenerative disease associated with repetitive head impact (RHI) exposure, but the neuropathologic substrates that underlie parkinsonism in individuals with CTE are yet to be defined.</p><p><strong>Objective: </strong>To evaluate the frequency of parkinsonism in individuals with CTE and the association of RHI and neuropathologic substrates with parkinsonism in these individuals.</p><p><strong>Design, setting, and participants: </strong>This cross-sectional study included brain donors with neuropathologically diagnosed CTE without other significant neurodegenerative disease and with information on parkinsonism from the Understanding Neurologic Injury and Traumatic Encephalopathy brain bank between July 2015 and May 2022.</p><p><strong>Exposure: </strong>Years of contact sports participation as a proxy for RHI.</p><p><strong>Main outcomes and measures: </strong>The main outcomes were frequency of parkinsonism in individuals with CTE and associations between (1) RHI with substantia nigra (SN) Lewy bodies (LBs) and neurofibrillary tangles (NFTs); (2) LBs, NFTs, and arteriolosclerosis with SN neuronal loss; and (3) SN neuronal loss, LBs, NFTs, and arteriolosclerosis with parkinsonism, tested by age-adjusted logistic regressions.</p><p><strong>Results: </strong>Of 481 male brain donors with neuropathologically diagnosed CTE, parkinsonism occurred frequently in individuals with CTE (119 [24.7%]; 362 [75.3%] did not have parkinsonism). Participants with parkinsonism had a higher mean (SD) age at death (71.5 [13.0] years) than participants without parkinsonism (54.1 [19.3] years) (P < .001) and higher rates of dementia (104 [87.4%] vs 105 [29.0%]), visual hallucinations (45 [37.8%] vs 51 [14.1%]), and probable rapid eye movement sleep behavior disorder (52 [43.7%] vs 58 [16.0%]) (P < .001 for all). Participants with parkinsonism had a more severe CTE stage (eg, stage IV: 35 [29.4%] vs 39 [10.8%]) and nigral pathology than those without parkinsonism (NFTs: 50 of 117 [42.7%] vs 103 of 344 [29.9%]; P = .01; neuronal loss: 61 of 117 [52.1%] vs 59 of 344 [17.1%]; P < .001; and LBs: 28 of 116 [24.1%] vs 20 of 342 [5.8%]; P < .001). Years of contact sports participation were associated with SN NFTs (adjusted odds ratio [AOR], 1.04; 95% CI, 1.00-1.07; P = .03) and neuronal loss (AOR, 1.05; 95% CI, 1.01-1.08; P = .02). Nigral neuronal loss (AOR, 2.61; 95% CI, 1.52-4.47; P < .001) and LBs (AOR, 2.29; 95% CI, 1.15-4.57; P = .02) were associated with parkinsonism. However, SN neuronal loss was associated with SN LBs (AOR, 4.48; 95% CI, 2.25-8.92; P < .001), SN NFTs (AOR, 2.51; 95% CI, 1.52-4.15; P < .001), and arteriolosclerosis (AOR, 2.27; 95% CI, 1.33-3.85; P = .002). In American football players, regression analysis demonstrated that SN NFTs and neuronal loss mediated the association between years of play and parkinsonism in th
{"title":"Substantia Nigra Pathology, Contact Sports Play, and Parkinsonism in Chronic Traumatic Encephalopathy.","authors":"Jason W Adams, Daniel Kirsch, Samantha M Calderazzo, Fatima Tuz-Zahra, Yorghos Tripodis, Jesse Mez, Michael L Alosco, Victor E Alvarez, Bertrand R Huber, Caroline Kubilus, Kerry A Cormier, Raymond Nicks, Madeline Uretsky, Evan Nair, Eva Kuzyk, Nurgul Aytan, Jonathan D Cherry, John F Crary, Daniel H Daneshvar, Christopher J Nowinski, Lee E Goldstein, Brigid Dwyer, Douglas I Katz, Robert C Cantu, Robert A Stern, Ann C McKee, Thor D Stein","doi":"10.1001/jamaneurol.2024.2166","DOIUrl":"10.1001/jamaneurol.2024.2166","url":null,"abstract":"<p><strong>Importance: </strong>Parkinsonism is associated with traumatic brain injury and chronic traumatic encephalopathy (CTE), a neurodegenerative disease associated with repetitive head impact (RHI) exposure, but the neuropathologic substrates that underlie parkinsonism in individuals with CTE are yet to be defined.</p><p><strong>Objective: </strong>To evaluate the frequency of parkinsonism in individuals with CTE and the association of RHI and neuropathologic substrates with parkinsonism in these individuals.</p><p><strong>Design, setting, and participants: </strong>This cross-sectional study included brain donors with neuropathologically diagnosed CTE without other significant neurodegenerative disease and with information on parkinsonism from the Understanding Neurologic Injury and Traumatic Encephalopathy brain bank between July 2015 and May 2022.</p><p><strong>Exposure: </strong>Years of contact sports participation as a proxy for RHI.</p><p><strong>Main outcomes and measures: </strong>The main outcomes were frequency of parkinsonism in individuals with CTE and associations between (1) RHI with substantia nigra (SN) Lewy bodies (LBs) and neurofibrillary tangles (NFTs); (2) LBs, NFTs, and arteriolosclerosis with SN neuronal loss; and (3) SN neuronal loss, LBs, NFTs, and arteriolosclerosis with parkinsonism, tested by age-adjusted logistic regressions.</p><p><strong>Results: </strong>Of 481 male brain donors with neuropathologically diagnosed CTE, parkinsonism occurred frequently in individuals with CTE (119 [24.7%]; 362 [75.3%] did not have parkinsonism). Participants with parkinsonism had a higher mean (SD) age at death (71.5 [13.0] years) than participants without parkinsonism (54.1 [19.3] years) (P < .001) and higher rates of dementia (104 [87.4%] vs 105 [29.0%]), visual hallucinations (45 [37.8%] vs 51 [14.1%]), and probable rapid eye movement sleep behavior disorder (52 [43.7%] vs 58 [16.0%]) (P < .001 for all). Participants with parkinsonism had a more severe CTE stage (eg, stage IV: 35 [29.4%] vs 39 [10.8%]) and nigral pathology than those without parkinsonism (NFTs: 50 of 117 [42.7%] vs 103 of 344 [29.9%]; P = .01; neuronal loss: 61 of 117 [52.1%] vs 59 of 344 [17.1%]; P < .001; and LBs: 28 of 116 [24.1%] vs 20 of 342 [5.8%]; P < .001). Years of contact sports participation were associated with SN NFTs (adjusted odds ratio [AOR], 1.04; 95% CI, 1.00-1.07; P = .03) and neuronal loss (AOR, 1.05; 95% CI, 1.01-1.08; P = .02). Nigral neuronal loss (AOR, 2.61; 95% CI, 1.52-4.47; P < .001) and LBs (AOR, 2.29; 95% CI, 1.15-4.57; P = .02) were associated with parkinsonism. However, SN neuronal loss was associated with SN LBs (AOR, 4.48; 95% CI, 2.25-8.92; P < .001), SN NFTs (AOR, 2.51; 95% CI, 1.52-4.15; P < .001), and arteriolosclerosis (AOR, 2.27; 95% CI, 1.33-3.85; P = .002). In American football players, regression analysis demonstrated that SN NFTs and neuronal loss mediated the association between years of play and parkinsonism in th","PeriodicalId":14677,"journal":{"name":"JAMA neurology","volume":" ","pages":"916-924"},"PeriodicalIF":20.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11250391/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141616467","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}
Pub Date : 2024-09-01DOI: 10.1001/jamaneurol.2024.1922
Andrea M Shamaskin-Garroway, Joel Shamaskin
{"title":"Slowing Down-A Family's Experience With ALS.","authors":"Andrea M Shamaskin-Garroway, Joel Shamaskin","doi":"10.1001/jamaneurol.2024.1922","DOIUrl":"10.1001/jamaneurol.2024.1922","url":null,"abstract":"","PeriodicalId":14677,"journal":{"name":"JAMA neurology","volume":" ","pages":"907-908"},"PeriodicalIF":20.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141442681","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 : 2024-09-01DOI: 10.1001/jamaneurol.2024.2100
Joshua D Grill, Jennifer H Lingler
{"title":"Centers for Medicare and Medicaid Services Coverage of Amyloid PET.","authors":"Joshua D Grill, Jennifer H Lingler","doi":"10.1001/jamaneurol.2024.2100","DOIUrl":"10.1001/jamaneurol.2024.2100","url":null,"abstract":"","PeriodicalId":14677,"journal":{"name":"JAMA neurology","volume":" ","pages":"903-904"},"PeriodicalIF":20.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141616466","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}