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Prevalence of, and Disability Due to, Multiple Sclerosis and Neuromyelitis Optica Spectrum Disorder in Japan by the Fifth Nationwide Survey. 日本第五次全国调查中多发性硬化症和神经性脊髓炎谱系障碍的患病率和致残率。
IF 7.7 1区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2024-11-26 Epub Date: 2024-10-30 DOI: 10.1212/WNL.0000000000209992
Mitsuru Watanabe, Noriko Isobe, Masaaki Niino, Ichiro Nakashima, Takuya Matsushita, Yasunari Sakai, Jin Nakahara, Izumi Kawachi, Hirofumi Ochi, Yuji Nakatsuji, Yosikazu Nakamura, Koshi Nakamura, Kiyomi Sakata, Makoto Matsui, Satoshi Kuwabara, Jun-Ichi Kira

Background and objectives: All 4 previous nationwide surveys of multiple sclerosis (MS) in Japan were conducted before the discovery of anti-aquaporin-4 (AQP4) antibodies; thus, neuromyelitis optica spectrum disorder (NMOSD) was included in MS, as optic-spinal MS. We aimed to clarify the epidemiologic features and trends of MS and NMOSD in Japan separately using a fifth nationwide survey.

Methods: The primary survey, in which a questionnaire was sent to 3,799 selected departments (including neurology/internal medicine, pediatrics, and ophthalmology), explored the estimated number and prevalence of patients with MS or NMOSD in 2017, and the secondary survey collected detailed characteristics of the patients using a second questionnaire.

Results: The response rates for the primary and secondary surveys were 60.1% and 53.9%, respectively. The estimated total number of patients with MS or NMOSD was 24,800, 2.5-fold higher than that in the fourth survey in 2003. The crude prevalence was 19.6 per 100,000 patients (14.2 for MS and 5.4 for NMOSD), compared with 7.7 per 100,000 patients in the fourth survey. Patients with MS showed milder disability (median Expanded Disability Status Scale [EDSS] score: 2.0 [interquartile range 1.0-4.5] vs 2.5 [1.0-6.0]), decreased secondary progression (8.5% vs 15.2%), and increased usage of disease-modifying drugs (63.7% vs 37.2%) compared with those with conventional MS in the fourth survey. The proportions of oligoclonal bands and Barkhof criteria fulfillment on MRI, which are features of classical MS, increased with advancing year of birth. Patients with NMOSD also showed less disability and shorter disease duration than patients with optic-spinal MS in the fourth survey (EDSS score: 3.5 [2.0-5.5] vs 3.8 [2.0-6.0]; disease duration: 8.0 [3.9-14.8] vs 10.0 [5.0-16.0]). Among patients with NMOSD, disability was exacerbated by a history of longitudinally extensive spinal cord lesions and anti-AQP4 antibody positivity, which both decreased with advancing year of birth.

Discussion: The prevalences of MS (particularly with classical features) and NMOSD have been increasing in Japan, suggesting the contribution of environmental factors. However, disabilities in patients with MS and NMOSD have been mitigated. Extensive usage of various disease-modifying drugs could be a factor contributing to this disability mitigation in MS.

背景和目的:日本此前进行的四次全国性多发性硬化症(MS)调查都是在发现抗quaporin-4(AQP4)抗体之前进行的;因此,神经脊髓炎视神经谱系障碍(NMOSD)被作为视脊髓多发性硬化症纳入MS。我们的目的是通过第五次全国调查,分别阐明日本多发性硬化症和 NMOSD 的流行病学特征和发展趋势:初级调查向 3799 个选定科室(包括神经内科/内科、儿科和眼科)发送了调查问卷,调查了 2017 年多发性硬化症或 NMOSD 患者的估计人数和患病率,次级调查使用第二份调查问卷收集了患者的详细特征:一级调查和二级调查的回复率分别为 60.1%和 53.9%。据估计,多发性硬化症或非多发性硬化症患者的总人数为 24,800 人,比 2003 年第四次调查时增加了 2.5 倍。粗发病率为每 10 万名患者中有 19.6 人(多发性硬化症患者为 14.2 人,非肢体缺损症患者为 5.4 人),而第四次调查的粗发病率为每 10 万名患者中有 7.7 人。与第四次调查中的传统多发性硬化症患者相比,多发性硬化症患者的残疾程度较轻(扩展残疾状况量表[EDSS]评分中位数:2.0[四分位间范围1.0-4.5] vs 2.5 [1.0-6.0]),继发性进展较少(8.5% vs 15.2%),使用疾病调节药物的比例较高(63.7% vs 37.2%)。随着出生年份的增加,核磁共振成像中出现寡克隆带和符合巴克霍夫标准(这是典型多发性硬化症的特征)的比例也在增加。在第四次调查中,与视神经脊髓多发性硬化症患者相比,NMOSD 患者的残疾程度更轻,病程更短(EDSS 评分:3.5 [2.0-5.5] vs 3.8 [2.0-6.0];病程:8.0 [3.9-14.8] vs 10.0 [5.0-16.0])。在NMOSD患者中,纵向广泛脊髓病变史和抗AQP4抗体阳性会加重残疾,而这两种情况都会随着出生年份的增加而减少:讨论:多发性硬化症(尤其是具有典型特征的多发性硬化症)和 NMOSD 的发病率在日本一直呈上升趋势,这表明与环境因素有关。然而,多发性硬化症和 NMOSD 患者的残疾程度有所减轻。广泛使用各种改变病情的药物可能是导致多发性硬化症残疾减轻的一个因素。
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引用次数: 0
Person-Directed Ictal Pointing Gesture During a Focal Seizure: Intracranial EEG Correlation. 局灶性癫痫发作时的人指向躯干手势:颅内脑电图相关性。
IF 7.7 1区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2024-11-26 Epub Date: 2024-10-28 DOI: 10.1212/WNL.0000000000210024
Fawad Khan, Evan Nelson, Colin Curtis
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引用次数: 0
Diagnosis of Incident Cancer After Cryptogenic Stroke: An Exploratory Analysis of the ARCADIA Randomized Trial. 隐源性卒中后的癌症诊断:ARCADIA 随机试验的探索性分析。
IF 7.7 1区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2024-11-26 Epub Date: 2024-10-31 DOI: 10.1212/WNL.0000000000210027
Babak B Navi, Cenai Zhang, Benjamin R Miller, Anokhi Pawar, Mary Cushman, Scott E Kasner, David Tirschwell, W T Longstreth, Richard Kronmal, Jordan Elm, Richard M Zweifler, Joseph Tarsia, Joseph P Broderick, David J Gladstone, Morin Beyeler, Hooman Kamel, Mitchell S V Elkind, Christopher Streib

Objectives: The objective of this study was to estimate the incidence, timing, and type of new cancer diagnosis among patients with cryptogenic stroke.

Methods: We used data from the ARCADIA trial, which enrolled patients with cryptogenic stroke and atrial cardiopathy. Participants were prospectively followed, and serious adverse events were assessed every 3 months or sooner if investigators were alerted between visits to an event. Kaplan-Meier statistics were used to estimate the cumulative incidence of a cancer diagnosis within the first year after randomization.

Results: Among 878 participants without baseline history of cancer, 13 (1.5%) were diagnosed with incident cancer in the year after randomization, comprising 12 solid cancers (3 prostate, 2 breast, 2 gastrointestinal, and 5 other primary sites) and 1 hematologic cancer (non-Hodgkin lymphoma). The cumulative incidences of a cancer diagnosis were 0% at 3 months, 0.6% (95% CI 0.2%-1.5%) at 6 months, and 2.0% (95 CI 1.1%-3.4%) at 1 year. The median time from index stroke to cancer diagnosis was 261 days (interquartile range 183-358).

Discussion: In a multicenter cryptogenic stroke cohort with prospective follow-up, the 1-year cumulative incidence of a cancer diagnosis was 2%. This rate may be an underestimation because of the clinical trial population and exclusion of cancers diagnosed immediately after stroke.

Trial registration information: ClinicalTrials.gov Identifier: NCT03192215. Registered June 20, 2017. First patient enrolled February 1, 2018.

研究目的本研究旨在估算隐源性中风患者新诊断癌症的发生率、时间和类型:我们使用了 ARCADIA 试验的数据,该试验招募了隐源性中风和心房性心脏病患者。我们对参与者进行了前瞻性随访,每 3 个月对严重不良事件进行一次评估,如果调查人员在两次随访之间接到事件警报,则提前进行评估。采用 Kaplan-Meier 统计法估算随机化后第一年内癌症诊断的累积发生率:在基线无癌症病史的 878 名参与者中,有 13 人(1.5%)在随机化后一年内被诊断为癌症,其中包括 12 例实体癌(3 例前列腺癌、2 例乳腺癌、2 例胃肠道癌症和 5 例其他原发部位癌症)和 1 例血液系统癌症(非霍奇金淋巴瘤)。癌症诊断的累计发生率为:3 个月时 0%,6 个月时 0.6%(95% CI 0.2%-1.5%),1 年时 2.0%(95% CI 1.1%-3.4%)。从指数中风到癌症确诊的中位时间为 261 天(四分位间范围 183-358):讨论:在具有前瞻性随访的多中心隐源性卒中队列中,癌症诊断的 1 年累积发生率为 2%。讨论:在多中心隐匿性中风队列的前瞻性随访中,1 年的累积癌症发病率为 2%,由于临床试验人群以及排除了中风后立即确诊的癌症患者,该发病率可能被低估了:试验注册信息:ClinicalTrials.gov Identifier:NCT03192215。2017年6月20日注册。2018年2月1日首例患者入组。
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引用次数: 0
Efficacy of Atogepant in Chronic Migraine With and Without Acute Medication Overuse in the Randomized, Double-Blind, Phase 3 PROGRESS Trial. 随机、双盲、3 期 PROGRESS 试验中,Atogepant 对伴有或不伴有急性药物过度使用的慢性偏头痛的疗效。
IF 7.7 1区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2024-11-26 Epub Date: 2024-10-21 DOI: 10.1212/WNL.0000000000209868
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引用次数: 0
"Perfusion Scotoma" As an Imaging Marker for the No-Reflow Phenomenon After Thrombectomy? 血栓切除术后无回流现象的成像标记 "灌注斯科特瘤"?
IF 7.7 1区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2024-11-26 Epub Date: 2024-10-21 DOI: 10.1212/WNL.0000000000210089
Michelle P Lin, Saikrishna Vallamchetla
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引用次数: 0
Editor's Note: Predictors of Seizure Recurrence in Women With Idiopathic Generalized Epilepsy Who Switch From Valproate to Another Medication. 编者按:从丙戊酸钠转用另一种药物的特发性全身性癫痫女性患者癫痫复发的预测因素。
IF 7.7 1区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2024-11-26 Epub Date: 2024-10-28 DOI: 10.1212/WNL.0000000000210082
Ariane Lewis, Steven L Galetta
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引用次数: 0
Reader Response: Predictors of Seizure Recurrence in Women With Idiopathic Generalized Epilepsy Who Switch From Valproate to Another Medication. 读者回复:从丙戊酸钠转用另一种药物的特发性全身性癫痫女性患者癫痫复发的预测因素》(Predictors of Seizure Recurrence in Women With Idiopathic Generalized Epilepsy who Switch From Valproate to Another Medication)。
IF 7.7 1区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2024-11-26 Epub Date: 2024-10-28 DOI: 10.1212/WNL.0000000000209589
Nitin K Sethi
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引用次数: 0
Energy Associated With Dynamic Network Changes in Patients With Multiple Sclerosis and Cognitive Impairment. 与多发性硬化症和认知障碍患者动态网络变化相关的能量。
IF 7.7 1区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2024-11-12 Epub Date: 2024-10-11 DOI: 10.1212/WNL.0000000000209952
Tommy A A Broeders, Maureen van Dam, Giuseppe Pontillo, Vasco Rauh, Linda Douw, Ysbrand D van der Werf, Joep Killestein, Frederik Barkhof, Christiaan H Vinkers, Menno M Schoonheim

Background and objectives: Patients with multiple sclerosis (MS) often experience cognitive impairment, and this is related to structural disconnection and subsequent functional reorganization. It is unclear how specific patterns of functional reorganization might make it harder for cognitively impaired (CI) patients with MS to dynamically adapt how brain regions communicate, which is crucial for normal cognition. We aimed to identify dynamic functional network patterns that are relevant to cognitive impairment in MS and investigate whether these patterns can be explained by altered energy costs.

Methods: Resting-state functional and diffusion MRI was acquired in a cross-sectional design, as part of the Amsterdam MS cohort. Patients with clinically definitive MS (relapse-free) were classified as CI (≥2/7 domains Z < -2), mildly CI (MCI) (≥2/7 domains Z < -1.5), or cognitively preserved (CP) based on an expanded Brief Repeatable Battery of Neuropsychological Tests. Functional connectivity states were determined using k-means clustering of moment-to-moment cofluctuations (i.e., edge time series), and the resulting state sequence was used to characterize the frequency of transitions. Control energy of the state transitions was calculated using the structural network with network control theory.

Results: Imaging and cognitive data were available for 95 controls and 330 patients (disease duration: 15 years; 179 CP, 65 MCI, and 86 CI). We identified a "visual network state," "sensorimotor network state," "ventral attention network state," and "default mode network state." CI patients transitioned less frequently between connectivity states compared with CP (β = -5.78; p = 0.038). Relative to the time spent in a state, CI patients transitioned less from a "default mode network state" to a "visual network state" (β = -0.02; p = 0.004). The CI patients required more control energy to transition between states (β = 0.32; p = 0.007), particularly for the same transition (β = 0.34; p = 0.049).

Discussion: This study showed that it costs more energy for MS patients with cognitive impairment to dynamically change the functional network, possibly explaining why these transitions occur less frequently. In particular, transitions from a default mode network state to a visual network state were relevant for cognition in these patients. To further study the order of events leading to these network disturbances, future work should include longitudinal data across different disease stages.

背景和目的:多发性硬化症(MS)患者经常会出现认知障碍,这与结构断裂和随后的功能重组有关。目前尚不清楚特定的功能重组模式会如何使认知功能受损(CI)的多发性硬化症患者更难动态调整大脑区域的交流方式,而这对正常认知至关重要。我们旨在确定与多发性硬化症认知障碍相关的动态功能网络模式,并研究这些模式是否可以用能量成本的改变来解释:方法:作为阿姆斯特丹多发性硬化症队列的一部分,我们采用横断面设计采集了静息态功能和弥散磁共振成像。临床确诊的多发性硬化症患者(无复发)被分为CI(≥2/7 domains Z <-2)、轻度CI (MCI)(≥2/7 domains Z <-1.5)或认知功能保留(CP)。功能连接状态是通过对瞬间到瞬间的共波动(即边缘时间序列)进行k-means聚类确定的,由此产生的状态序列用于描述状态转换的频率。利用网络控制理论的结构网络计算状态转换的控制能量:我们获得了 95 名对照组和 330 名患者(病程:15 年;179 名 CP、65 名 MCI 和 86 名 CI)的成像和认知数据。我们确定了 "视觉网络状态"、"感觉运动网络状态"、"腹侧注意网络状态 "和 "默认模式网络状态"。与 CP 相比,CI 患者在连接状态之间转换的频率较低(β = -5.78; p = 0.038)。相对于在一种状态下所花费的时间,CI 患者从 "默认模式网络状态 "过渡到 "视觉网络状态 "的时间较少(β = -0.02;p = 0.004)。CI患者在不同状态之间转换时需要更多的控制能量(β = 0.32; p = 0.007),尤其是在相同的转换过程中(β = 0.34; p = 0.049):本研究表明,认知障碍多发性硬化症患者动态改变功能网络需要花费更多的能量,这可能解释了为什么这些转换发生的频率较低。尤其是从默认模式网络状态到视觉网络状态的转换与这些患者的认知相关。为了进一步研究导致这些网络紊乱的事件发生顺序,未来的工作应包括不同疾病阶段的纵向数据。
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引用次数: 0
Teaching NeuroImage: Basilar Artery Septation With Nodularity. 神经影像教学:基底动脉闭塞伴结节。
IF 7.7 1区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2024-11-12 Epub Date: 2024-09-30 DOI: 10.1212/WNL.0000000000209967
Rogan Magee, Yombe Fonkeu, Lamya Ibrahim, Jae W Song, Steven R Messe
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引用次数: 0
Seizure Assessment and Forecasting With Efficient Rapid-EEG: A Retrospective Multicenter Comparative Effectiveness Study. 利用高效快速脑电图评估和预测癫痫发作:一项回顾性多中心比较效应研究。
IF 7.7 1区 医学 Q1 CLINICAL NEUROLOGY Pub Date : 2024-11-12 Epub Date: 2024-10-14 DOI: 10.1212/WNL.0000000000209840
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引用次数: 0
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Neurology
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