Canadian Journal of Neurological Sciences最新文献_第10页

Adult-Onset PLA2G6-Associated Neurodegeneration (PLAN): A Clinical, Imaging and Genetic Profile Review. 成人发病pla2g6相关神经变性（PLAN）：临床、影像学和遗传概况综述

IF 2.2 4区医学

Canadian Journal of Neurological Sciences Pub Date : 2025-07-07 DOI: 10.1017/cjn.2025.10352

Rohan Mahale, Hansashree Padmanabha, Pooja Mailankody

{"title":"Adult-Onset PLA2G6-Associated Neurodegeneration (PLAN): A Clinical, Imaging and Genetic Profile Review.","authors":"Rohan Mahale, Hansashree Padmanabha, Pooja Mailankody","doi":"10.1017/cjn.2025.10352","DOIUrl":"10.1017/cjn.2025.10352","url":null,"abstract":"Background: Adult-onset PLA2G6-associated neurodegeneration (PLAN) has a diverse phenotypic presentation.Objective: Detailed description of the clinical, imaging and genetic profile of adult-onset PLAN; comparison of the Indian cohort with Asian, Middle East and European cohorts; genotype-phenotype correlations; and determination of ethnic phenotypic and genotypic differences of adult-onset PLAN.Methods: Report of two patients of adult-onset PLAN and review of reported cases of adult-onset PLAN since 2015 from Indian, Asian, Middle East and European cohorts.Results: There were 12 cases in the Indian cohort, 45 in the Asian cohort, 10 in the Middle East cohort and 17 in European cohort. Patients in the Indian and Asian cohorts had a later age at onset as compared to the Middle East and European cohorts. The median duration of disease was similar among all cohorts. Dystonia, myoclonus and gaze palsy were common in the Indian cohort; parkinsonism and tremor in the Asian cohort; parkinsonism, tremor, spasticity and cognitive impairment in the Middle East cohort; and parkinsonism and behavioural disturbances in the European cohort. Early-onset parkinsonism was common in all cohorts. Mineralisation on MRI was less frequent in the Asian and Middle East cohorts. Cerebral/cerebellar atrophy was less frequent in the Asian cohort. The homozygous missense variant (c.2222G > A,p.R741Q) was common in the Indian and Middle East cohorts, whereas the homozygous/compound heterozygous variant (c.991G > T, p.D331Y) was the most common variant in the Asian cohort. Milder clinical and neuroimaging phenotypes were noted with c.991G > T (p.D331Y) variant and a relatively severe phenotype in c.2222G > A,p.R741Q variant.Conclusion: Adult-onset PLAN has a variable phenotype. We found ethnic phenotypic and imaging differences among the cohorts.","PeriodicalId":56134,"journal":{"name":"Canadian Journal of Neurological Sciences","volume":" ","pages":"1-8"},"PeriodicalIF":2.2,"publicationDate":"2025-07-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144577058","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Corpora Amylacea Mimicking Diffuse Glioma: A Rare Case of Extensive Periventricular Accumulation. 淀粉样体模拟弥漫性胶质瘤：一例罕见的广泛脑室周围积聚。

IF 2.2 4区医学

Canadian Journal of Neurological Sciences Pub Date : 2025-07-07 DOI: 10.1017/cjn.2025.10115

Jung Youn Kim, Hye Jeong Choi, Sang Heum Kim, Kyung Gi Cho, Jin-Hyung Heo

引用次数: 0

Long-Term Tofersen and CSF Macrophage Inclusions in Superoxide Dismutase 1 Amyotrophic Lateral Sclerosis. 超氧化物歧化酶- 1型肌萎缩性侧索硬化症患者的长期凝血素和脑脊液巨噬细胞包裹体。

IF 2.9 4区医学

Canadian Journal of Neurological Sciences Pub Date : 2025-07-04 DOI: 10.1017/cjn.2025.10351

Michèle G DuVal, Tom Noga, Grayson Beecher

引用次数: 0

Orbital Myositis Preceding Presumed Giant Cell Myocarditis. 眼眶肌炎先于假定的巨细胞心肌炎。

IF 2.2 4区医学

Canadian Journal of Neurological Sciences Pub Date : 2025-07-03 DOI: 10.1017/cjn.2025.10086

Bhadra U Pandya, Niv Levy, Heather M McDonald, Edward A Margolin

引用次数: 0

Tofersen Treatment Normalizes Neurofilament Levels in Autosomal Recessive SOD1 Amyotrophic Lateral Sclerosis. 托佛森治疗可使常染色体隐性SOD1肌萎缩性侧索硬化症患者的神经丝水平正常化。

IF 2.2 4区医学

Canadian Journal of Neurological Sciences Pub Date : 2025-07-03 DOI: 10.1017/cjn.2025.10350

Bram De Wel, Theodore Mobach, Gerald Pfeffer, Gordon Jewett

引用次数: 0

Segmental Infarction of the Spinal Cord, Vertebral Body and Paraspinal Muscles. 脊髓、椎体和棘旁肌肉的节段性梗塞。

IF 2.9 4区医学

Canadian Journal of Neurological Sciences Pub Date : 2025-06-30 DOI: 10.1017/cjn.2025.10346

Mahsa Sepahvand, Helia Sepahvand, Ali Omidvari

引用次数: 0

Left-Sided Neurological Symptoms and Negative Diffusion-Weighted MRI in Suspected Minor Stroke Patients. 疑似轻微脑卒中患者的左侧神经症状和阴性弥散加权MRI。

IF 2.2 4区医学

Canadian Journal of Neurological Sciences Pub Date : 2025-06-27 DOI: 10.1017/cjn.2025.10125

Umberto Pensato, Nishita Singh, Raed A Joundi, Koji Tanaka, Andrew M William Penn, Kristine Votova, Maximilian Bibok, Robert Balshaw, Andrew M Demchuk, Michael D Hill, Shelagh B Coutts

{"title":"Left-Sided Neurological Symptoms and Negative Diffusion-Weighted MRI in Suspected Minor Stroke Patients.","authors":"Umberto Pensato, Nishita Singh, Raed A Joundi, Koji Tanaka, Andrew M William Penn, Kristine Votova, Maximilian Bibok, Robert Balshaw, Andrew M Demchuk, Michael D Hill, Shelagh B Coutts","doi":"10.1017/cjn.2025.10125","DOIUrl":"10.1017/cjn.2025.10125","url":null,"abstract":"Background: Historically, it has been proposed that functional neurological symptoms occur more frequently on the left side of the body due to a distinct body representation and emotional processing of the right hemisphere, yet objective imaging data to support this are lacking. We aimed to investigate whether patients with acute left-sided symptoms (right hemisphere) suspected of having a minor stroke are more likely to show negative diffusion-weighted imaging (DWI) compared to those with right-sided symptoms.Methods: Data are from the SpecTRA (Spectrometry for Transient Ischemic Attack Rapid Assessment) multicenter prospective cohort study conducted between 2013 and 2017. Patients with mild persistent unilateral hemiparesis and/or hemisensory symptoms (National Institute of Health Stroke Scale ≤ 3) and available DWI were included. The primary outcome was the proportion of patients with a negative DWI.Results: Of 1731 patients, 584 (30.8%) were included. Of these, 310 (53.1%) patients presented with left-sided symptoms and 274 (46.9%) with right-sided symptoms. Overall, 214 (36.6%) patients had a negative DWI, 126 (58.9%) with left-sided symptoms and 88 (41.1%) with right-sided symptoms: risk ratio (RR) 1.27 (95% CI = 1.02-1.57). Left-sided hemiparesis was associated with negative DWI (RR 1.42 [95% CI = 1.08-1.87]), while left-sided hemisensory symptoms were not (RR 1.11 [95% CI = 0.87-1.41]). There was no effect modification by age or sex on this association (Pinteraction 0.787 and 0.057, respectively).Conclusions: Unilateral left-sided neurological symptoms were more frequently associated with negative DWI compared to right-sided symptoms in suspected minor stroke patients. This observation is exploratory, as the final diagnosis in DWI-negative cases was not established.","PeriodicalId":56134,"journal":{"name":"Canadian Journal of Neurological Sciences","volume":" ","pages":"1-6"},"PeriodicalIF":2.2,"publicationDate":"2025-06-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144509588","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Safety and Real-World Effectiveness in the Transition from Alteplase to Tenecteplase for Stroke Treatment. 卒中治疗从阿替普酶过渡到替奈替普酶的安全性和实际有效性。

IF 2.2 4区医学

Canadian Journal of Neurological Sciences Pub Date : 2025-06-27 DOI: 10.1017/cjn.2025.10121

Mary-Lou Halabi, Jillian Stang, Bijoy K Menon, Brian H Buck, Christiane Job-McIntosh, Stephen B Wilton, Shelley Valaire, Balraj Mann, Lisa Collins, Colleen Taralson, Lindsay Beaulieu, Elaine Shand, Corinna Hartley, Chelsey Dalgleish, Anthony Nickonchuk, Shannon Erfle, Oje Imoukhuede, Alejandro Manosalva, Katie Lin, Shelagh B Coutts, Thomas Jeerakathil, Ashfaq Shuaib, Andrew M Demchuk, Michael D Hill

{"title":"Safety and Real-World Effectiveness in the Transition from Alteplase to Tenecteplase for Stroke Treatment.","authors":"Mary-Lou Halabi, Jillian Stang, Bijoy K Menon, Brian H Buck, Christiane Job-McIntosh, Stephen B Wilton, Shelley Valaire, Balraj Mann, Lisa Collins, Colleen Taralson, Lindsay Beaulieu, Elaine Shand, Corinna Hartley, Chelsey Dalgleish, Anthony Nickonchuk, Shannon Erfle, Oje Imoukhuede, Alejandro Manosalva, Katie Lin, Shelagh B Coutts, Thomas Jeerakathil, Ashfaq Shuaib, Andrew M Demchuk, Michael D Hill","doi":"10.1017/cjn.2025.10121","DOIUrl":"10.1017/cjn.2025.10121","url":null,"abstract":"Background: Tenecteplase has been shown to be non-inferior to alteplase for the treatment of acute ischemic stroke within 4.5 hours of stroke onset. While not formally approved by regulatory authorities, many jurisdictions have transitioned to using tenecteplase for routine stroke treatment because it is simpler to use and has cost advantages.Methods: We report a three-phase time-series analysis over 2.5 years and the process for transition from use of alteplase to tenecteplase for the routine treatment of acute ischemic stroke from a system-wide perspective involving an entire province. The transition was planned and implemented centrally. Data were collected in clinical routine, arising from both administrative sources and a prospective stroke registry, and represent real-world outcome data. Data are reported using standard descriptive statistics.Results: A total of 1211 patients were treated with intravenous thrombolysis (477 pre-transition using alteplase, 180 transition period using both drugs, 554 post-transition using tenecteplase). Baseline characteristics, adverse events and outcomes were similar between epochs. There were four dosing errors with tenecteplase, including providing the cardiac dose to two patients. There were no instances of major hemorrhage associated with dosing errors.Discussion: The transition to using intravenous tenecteplase for stroke treatment was seamless and resulted in identical outcomes to intravenous alteplase.","PeriodicalId":56134,"journal":{"name":"Canadian Journal of Neurological Sciences","volume":" ","pages":"1-6"},"PeriodicalIF":2.2,"publicationDate":"2025-06-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144509589","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Measuring Migraine in Canada and the USA: An Online Survey of Emergency Room and Smartphone Application Use. 测量加拿大和美国的偏头痛：一项关于急诊室和智能手机应用程序使用的在线调查。

IF 2.2 4区医学

Canadian Journal of Neurological Sciences Pub Date : 2025-06-25 DOI: 10.1017/cjn.2025.10140

Andrea E Portt, Christine Lay, Hong Chen, Erjia Ge, Peter M Smith

{"title":"Measuring Migraine in Canada and the USA: An Online Survey of Emergency Room and Smartphone Application Use.","authors":"Andrea E Portt, Christine Lay, Hong Chen, Erjia Ge, Peter M Smith","doi":"10.1017/cjn.2025.10140","DOIUrl":"10.1017/cjn.2025.10140","url":null,"abstract":"Background: Knowledge of environmental triggers for migraine attacks is limited and has mostly been acquired by studies using emergency room (ER) visits. However, it is unlikely that ER visits are a random sample of migraine events, even within strata of migraine severity. Additionally, time lags between attack onset and ER visits may vary across the population, posing challenges for assessing causal links of migraine with community-level or ecologic exposures.Objective: Our objective was to assess the relationship between demographic and geographic measures and self-reported migraine-related ER visits.Methods: We analyzed a targeted non-probability survey of ER use related to migraine in Canada and the USA. The 18-question online survey addressed ER use and behaviors related to recording attacks.Results: The final dataset included 389 respondents (Canada = 164 [42.2%], USA = 225 [57.8%]); 51 (13.1%) were Migraine Buddy app users who shared their diaries. In both countries, participants reported similar migraine symptoms. Barriers to attending the ER included cost and wait times. There was more variability in delays between attack onset and arrival to the ER than between onset and recording in the smartphone app. Younger participants and participants living in Canada were significantly more likely to present to the ER.Conclusion: The sample of patients presenting to the ER for migraine may be biased toward younger patients and depend on the jurisdiction. Smartphone app records may have fewer barriers to creation and more consistent time lags compared to ER visit records.","PeriodicalId":56134,"journal":{"name":"Canadian Journal of Neurological Sciences","volume":" ","pages":"1-9"},"PeriodicalIF":2.2,"publicationDate":"2025-06-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144487245","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Evaluating the Role of Post-Thrombectomy MRI in Predicting Functional Recovery in Basilar Artery Occlusion. 评价取栓后MRI在预测基底动脉闭塞患者功能恢复中的作用。

IF 2.2 4区医学

Canadian Journal of Neurological Sciences Pub Date : 2025-06-24 DOI: 10.1017/cjn.2025.10347

Nahal Farhani, Marco Ayroso, Susan Alcock, Jai Shankar

{"title":"Evaluating the Role of Post-Thrombectomy MRI in Predicting Functional Recovery in Basilar Artery Occlusion.","authors":"Nahal Farhani, Marco Ayroso, Susan Alcock, Jai Shankar","doi":"10.1017/cjn.2025.10347","DOIUrl":"10.1017/cjn.2025.10347","url":null,"abstract":"Background: Stroke due to basilar artery occlusion (BAO) carries poor outcomes despite advancements in endovascular therapy (EVT). Predictors of recovery remain underexplored. This study evaluates clinical and imaging predictors of outcomes following EVT, including a Critical Area Diffusion Score (CADS), assessing infarct location and extent in critical brainstem regions on post-EVT MRI.Methods: This retrospective study analyzed 48 BAO patients treated with EVT at a provincial stroke center (2015-2021). Patients were categorized by outcomes (favorable: modified Rankin Scale [mRS] 0-3; unfavorable: mRS 4-6). Clinical, demographic and imaging data - age, baseline National Institutes of Health Stroke Scale (NIHSS), reperfusion success (thrombolysis in cerebral infarction [TICI] 2b-3) and post-EVT CADS from diffusion-weighted MRI - were assessed using univariate and multivariate logistic regression.Results: Patients with favorable outcomes were younger (median 64.0 vs. 73.0 years, p = 0.031), had lower NIHSS scores at presentation (median 8 vs. 16, p = 0.018) and achieved higher successful reperfusion rates (81.0% vs. 48.1%, p = 0.020). CADS ≤ 3 was linked to better outcomes (median mRS 3 vs. 5, p = 0.026) and higher odds of recovery in univariate analysis (OR = 10.89, p = 0.038). However, in multivariate analysis, CADS was not an independent predictor, with successful reperfusion (OR = 18.8, p = 0.044) as the strongest factor.Conclusion: Age, NIHSS scores and successful reperfusion predict recovery in BAO patients undergoing EVT. Although CADS ≤ 3 is linked to favorable outcomes, it is not independently predictive. CADS holds promise as a prognostic tool, but its utility should be considered alongside clinical markers to enhance outcome prediction in BAO.","PeriodicalId":56134,"journal":{"name":"Canadian Journal of Neurological Sciences","volume":" ","pages":"1-8"},"PeriodicalIF":2.2,"publicationDate":"2025-06-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144477988","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0