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Fourth Nerve Palsy as the Presenting Manifestation of Giant Cell Arteritis. 第四神经麻痹是巨细胞动脉炎的先兆表现。
IF 2.9 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-11-14 DOI: 10.1017/cjn.2024.344
Samira Jafari, Jonathan A Micieli
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引用次数: 0
Principles and Priorities for Responsible Innovation in Neurotechnology for Canada. 加拿大神经技术负责任创新的原则和优先事项。
IF 2.9 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-11-13 DOI: 10.1017/cjn.2024.322
Judy Illes, Susan Boehnke, Jennifer A Chandler, Shirley Fecteau, Pauline Gaprielian, Nir Lipsman, Graeme Moffat, Amy Zarzeczny
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引用次数: 0
Pharmacist-Implemented Self-Management Module in Multiple Sclerosis Patients: A Randomized Controlled Trial. 多发性硬化症患者的药剂师自我管理模块:随机对照试验。
IF 2.9 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-11-13 DOI: 10.1017/cjn.2024.345
Cansu Goncuoglu, Pinar Acar Ozen, Merve Kasikci, Asli Tuncer, Aygin Bayraktar Ekincioglu

Background: Self-management practices can contribute to the lives of patients with multiple sclerosis. The aim of this study is to improve patients' self-management abilities through a multidisciplinary developed module.

Methods: This prospective, randomized controlled trial was conducted between January 2020 and November 2021 at a university hospital in Ankara, Turkiye. The self-management module was implemented by a clinical pharmacist with the aim of enhancing self-management capabilities through an educational approach, with a focus on medication adherence, management of drug-related problems, follow-ups and self-directed activities. The intervention group completed the self-management module, while the control group received usual outpatient care. To evaluate the impact of the module, the Multiple Sclerosis Self-Management Revised scale was administered to the patients. Interviews were conducted at 4-month intervals.

Results: Study (n = 102) and control group (n = 98) patients were followed up for 8 months, and the median duration of intervention was 11 minutes. The mean (± SD) self-management scores of the study group increased from 68.9 (± 9.3) to 79.0 (± 9.4) at the end of the interviews, and this increase was found to be significant compared to the control group (p < 0.001). The self-management module has been shown to improve self-management, medication adherence, perception of care and patient engagement in treatment (p < 0.001).

Conclusions: This single-center randomized controlled trial suggests that a pharmacist-implemented self-management module increased patient engagement and medication adherence. The self-management interventions could be tailored to groups that tend to have lower self-management abilities, such as older individuals, and those who have lower educational attainment, health engagement or medication adherence.

背景:自我管理实践有助于多发性硬化症患者的生活。本研究旨在透过多学科开发的模组,提升病患的自我管理能力。方法:这项前瞻性、随机对照试验于2020年1月至2021年11月在土耳其安卡拉的一家大学医院进行。自我管理模块由一名临床药剂师实施,目的是通过教育方法提高自我管理能力,重点是服药依从性、药物相关问题的管理、随访和自我指导活动。干预组完成自我管理模块,对照组接受常规门诊护理。为了评估该模块的影响,对患者实施多发性硬化症自我管理修订量表。访谈每隔4个月进行一次。结果:研究组(102例)和对照组(98例)患者随访8个月,干预时间中位数为11分钟。访谈结束时,研究组的自我管理平均分(±SD)从68.9(±9.3)上升到79.0(±9.4),与对照组相比,这一增长具有显著性(p < 0.001)。自我管理模块已被证明可以改善自我管理、药物依从性、护理感知和患者参与治疗(p < 0.001)。结论:这项单中心随机对照试验表明,药剂师实施的自我管理模块提高了患者的参与度和药物依从性。自我管理干预措施可以针对自我管理能力较低的群体量身定制,例如老年人,以及教育程度、健康参与度或药物依从性较低的群体。
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引用次数: 0
Non-ischemic Cerebral Enhancing (NICE) Lesions: A Rare Complication of Treating Intracranial Aneurysms with Endovascular Coiling. 非缺血性脑强化(NICE)病变:用血管内旋转术治疗颅内动脉瘤的罕见并发症。
IF 2.9 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-11-13 DOI: 10.1017/cjn.2024.320
Madeline A Komar, Sultan Yahya, Crystal Fong, Zehra Isik Hasiloglu, Nazir Khan
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引用次数: 0
The Challenges of Patients with Spinal CSF Leaks in Canada: A Cross-Sectional Online Survey. 加拿大脊髓脑脊液漏患者面临的挑战:横断面在线调查
IF 2.9 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-11-13 DOI: 10.1017/cjn.2024.315
Yasmine Hoydonckx, Philip Peng, Charlotte Vydt, Farnaz Amoozegar

Background: Spinal CSF leak can cause disabling headaches and neurological symptoms. Lack of awareness, diagnostic delay and treatment inconsistencies affect the quality of CSF leak care globally. This is the first study aiming to identify and assess these challenges in Canada.

Methods: A cross-sectional online survey of Canadian patients with spinal CSF leak was designed in collaboration with Spinal CSF Leak Canada, including questions on demographics, headache condition, investigations, treatments, quality of life, financial consequences and out-of-country care.

Results: The survey captured 103 respondents with confirmed spinal CSF leak diagnosis, of whom 56% were still suffering. The majority were female (80%), most being highly educated, with a mean age of 41.8 (SD: 10.37) years at the time of diagnosis. Inconsistencies in care resulted in variable durations for obtaining diagnosis and treatment. The majority of respondents (88%) had seen multiple physicians, and only 50% had seen a CSF leak specialist. Invasive imaging was not performed in 43%. CSF leak relapse after initial successful treatment occurred frequently (43%). The incidence of rebound intracranial hypertension was high (52.5%), and the treatment was difficult to access (77%). Out-of-country care was common (28%), and the impact on financial health was omnipresent (81.5%).

Conclusion: The survey demonstrates significant gaps in spinal CSF leak care in Canada, similar to global observations. Lack of awareness and access, delayed care, and inconsistencies in investigations and management are common. Spinal CSF leak significantly impacts patients' physical, mental and financial well-being. Increased awareness, referral pathways and standardized treatment algorithms are key factors in optimizing patient care in Canada.

背景:脊髓脑脊液漏可导致致残性头痛和神经症状。缺乏认识、诊断延误和治疗不一致影响着全球 CSF 漏护理的质量。这是加拿大第一项旨在确定和评估这些挑战的研究:方法:我们与加拿大脊髓脑脊液漏协会合作设计了一项针对加拿大脊髓脑脊液漏患者的横断面在线调查,调查内容包括人口统计学、头痛状况、检查、治疗、生活质量、经济后果和境外治疗等问题:调查收集了 103 名确诊为脊髓脊髓液漏的受访者,其中 56% 的人仍在遭受痛苦。大多数受访者为女性(80%),大部分受过高等教育,确诊时的平均年龄为 41.8 岁(标准差:10.37 岁)。护理方面的不一致导致获得诊断和治疗的时间长短不一。大多数受访者(88%)看过多位医生,只有 50%的受访者看过脑脊液漏专科医生。43%的受访者未进行侵入性成像检查。最初治疗成功后,脑脊液漏复发的情况经常发生(43%)。颅内高压反弹的发生率很高(52.5%),而且很难获得治疗(77%)。境外治疗很常见(28%),对经济健康的影响无处不在(81.5%):调查显示,加拿大在脊髓CSF渗漏治疗方面存在巨大差距,这与全球观察结果相似。缺乏认识和就医途径、护理延误以及检查和管理不一致是常见问题。脊髓脑脊液漏严重影响患者的身心健康和经济状况。提高认识、转诊途径和标准化治疗算法是优化加拿大患者护理的关键因素。
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引用次数: 0
Equitable Access to Disease-Modifying Therapies for Canadian Children with SMA and Four SMN2 Copies. 加拿大SMA和四种SMN2基因拷贝患儿公平获得疾病调节疗法。
IF 2.9 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-11-13 DOI: 10.1017/cjn.2024.319
Hugh J McMillan, Hernan Gonorazky, Craig Campbell, Nicolas Chrestian, Megan Crone, James J Dowling, Kristina Joyal, Hanna Kolski, Ed Leung, Alex Mackenzie, Jean K Mah, Laura McAdam, Elisa Nigro, Cam-Tu Nguyen, Maryam Oskoui, Chantal Poulin, Jordan Sheriko, Mark Tarnopolsky, Jiri Vajsar, Amanda Yaworski, Kathryn Selby
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引用次数: 0
Exploring Magnetic and Electrical Brain Stimulation in Parkinsonian Dyskinetic Monkeys. 探索对帕金森症运动障碍猴的磁刺激和脑电刺激。
IF 2.9 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-11-12 DOI: 10.1017/cjn.2024.284
Estelle Gouriou, Mélanie Bourque, Cyril Schneider, Thérèse Di Paolo

Background: Parkinson's disease (PD) chronic L-Dopa treatment often triggers motor complications, such as L-Dopa-induced dyskinesias (LID). LID are reported to be associated with abnormal glutamatergic activity between the striatum and primary motor cortex (M1), resulting in M1 hyperactivation. Beneficial noninvasive brain stimulation (NIBS) paradigms were reported to normalize glutamatergic activity. The objective of the present study was thus to set up a NIBS paradigm in parkinsonian monkeys to investigate motor behavior under basal conditions and with L-Dopa treatment-inducing dyskinesias.

Methods: Motor behavior was investigated in five 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) dyskinetic female Macaca fascicularis monkey models of PD, allowing us to monitor the administration of NIBS and drugs. NIBS used were inhibitory protocols, that is, cathodal transcranial direct current stimulation (c-tDCS) and continuous theta-burst stimulation (cTBS). A procedure of three weeks was developed to progressively acclimate animals to the experimental conditions, equipment and noise of c-tDCS and cTBS before stimulating them with either vehicle or L-Dopa.

Results: One session of c-tDCS with L-Dopa yielded no effect, whereas five sessions briefly reduced LID but decreased the duration of L-Dopa anti-PD effects. cTBS alone improved (decreased) parkinsonian scores as compared to sham stimulation or vehicle alone. Two sessions of cTBS with L-Dopa decreased LID without affecting L-Dopa anti-PD effects.

Conclusion: This is the first study testing c-tDCS and cTBS on the motor behavior of MPTP dyskinetic monkeys. As compared to medicated patients, MPTP monkeys offer the opportunity to evaluate NIBS after-effects in drug-free and LID conditions, which are critical in the search for new PD treatment.

背景:帕金森病(PD)的长期左旋多巴治疗通常会引发运动并发症,如左旋多巴诱发的运动障碍(LID)。据报道,LID 与纹状体和初级运动皮层(M1)之间的谷氨酸能活动异常有关,导致 M1 过度激活。据报道,有益的非侵入性脑刺激(NIBS)范式可使谷氨酸能活动恢复正常。因此,本研究的目的是在帕金森病猴中建立一个 NIBS 范例,以研究在基础条件下和左旋多巴治疗诱发运动障碍时的运动行为:在五只1-甲基-4-苯基-1,2,3,6-四氢吡啶(MPTP)运动障碍的雌性帕金森病猕猴模型中对运动行为进行了研究,使我们能够监测NIBS和药物的给药情况。NIBS 采用抑制性方案,即阴极经颅直流电刺激(c-tDCS)和连续θ-脉冲刺激(cTBS)。在对动物进行载体或左旋多巴刺激之前,我们制定了一个为期三周的程序,让动物逐渐适应 c-tDCS 和 cTBS 的实验条件、设备和噪音:与假刺激或单独使用药物相比,单独使用 cTBS 可改善(降低)帕金森评分。两个疗程的 cTBS 加左旋多巴可降低 LID,但不影响左旋多巴的抗帕金森效果:这是第一项测试 c-tDCS 和 cTBS 对 MPTP 运动障碍猴运动行为影响的研究。与接受药物治疗的患者相比,MPTP 猴提供了在无药和 LID 条件下评估 NIBS 后效应的机会,这对于寻找新的 PD 治疗方法至关重要。
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引用次数: 0
Hereditary Transthyretin Amyloidosis Neuropathy with Intracellular Amyloidosis and Inclusions. 遗传性转甲状腺素淀粉样变性神经病伴细胞内淀粉样变性和包涵体。
IF 2.9 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-11-11 DOI: 10.1017/cjn.2024.327
Jian-Qiang Lu, Gloria Mak, Sandra Grant, Steven K Baker
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引用次数: 0
Wall-Eyed Internuclear Ophthalmoplegia: History and Hypothesis. 壁眼核内眼肌症:历史与假说。
IF 2.9 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-11-11 DOI: 10.1017/cjn.2024.317
Janine L Johnston, James A Sharpe

Background: Most patients with internuclear ophthalmoplegia (INO) are orthotropic, although a subset is exotropic. When INO is bilateral, this is termed wall-eyed bilateral internuclear ophthalmoplegia (WEBINO). In 1979, Sharpe described his "first case" of wall-eyed monocular internuclear ophthalmoplegia (WEMINO) as "a unique clinical syndrome" characterized by unilateral INO and ipsilateral exotropia.

Methods: WEMINO was clinically identified in seven patients, with oculographic correlation in six and neuropathological confirmation in one. Oculographic features of exotropic INO patients were compared with those of six orthotropic INO patients using magnetic search coil and infrared oculography.

Results: All clinically defined WEMINO patients showed slowed, hypometric ipsilateral saccades by oculography. Six patients had ipsilateral exotropia, and three had ipsilateral hypertropia. Ipsilateral abducting saccades had faster peak velocities for smaller saccades, more so for orthotropic patients. Exotropic patients had normal sinusoidal mean vestibulo-ocular reflex (VOR) gains and phases; orthotropic patients had subnormal mean VOR gains and phase leads.

Conclusion: WEMINO is a clinical ocular motor syndrome characterized by unilateral slow, hypometric adducting saccades with exotropia and hypertropia of the ipsilateral eye. We propose that it results from discrete unilateral damage to burst-tonic fibers in the medial longitudinal fasciculus (MLF) with sparing of the adjacent extrafascicular pathways. Paradoxically, orthotropic INO results from more extensive damage to ascending pathways lateral, ventral and caudal to the MLF. Direct injury to the medial rectus subnucleus is not required. This manuscript was in preparation at the time of Dr Sharpe's death in 2013 and is an acknowledgement of his forward-thinking, as his hypotheses have stood the test of time.

背景:大多数核间眼肌麻痹(INO)患者都是直向性的,但也有一部分是外向性的。如果 INO 是双侧的,则称为壁眼双侧核间眼肌麻痹(WEBINO)。1979 年,夏普(Sharpe)描述了他的 "第一例 "壁眼单眼核间眼肌麻痹(WEMINO),认为这是一种 "独特的临床综合征",其特征是单侧 INO 和同侧外斜视:方法: 七名患者经临床确诊为 WEMINO,六名患者经眼球影像学检查确诊,一名患者经神经病理学检查确诊。使用磁搜索线圈和红外线眼底照相术比较了外向性 INO 患者和六名正向性 INO 患者的眼底特征:结果:所有临床定义的 WEMINO 患者的眼图都显示出同侧眼球移动缓慢、屈光度过低。六名患者有同侧外斜视,三名患者有同侧内斜视。同侧外展眼球时,较小的眼球移动的峰值速度较快,正视眼患者的情况更为明显。外向型患者的前庭眼反射(VOR)平均增益和相位均呈正常正弦曲线;而正交型患者的前庭眼反射平均增益和相位均不正常:WEMINO是一种临床眼球运动综合征,其特征是单侧缓慢、低度数内收眼球,同时伴有同侧眼球外斜和内斜。我们认为,这是由于内侧纵筋膜(MLF)的突触音调纤维单侧离散受损,而邻近的筋膜外通路受损。矛盾的是,正交性 INO 是由 MLF 外侧、腹侧和尾侧的上升通路受到更广泛的损伤造成的。无需直接损伤内侧直肌亚核。夏普博士于2013年逝世时,本手稿正在准备之中,他的假设经受住了时间的考验,这是对他前瞻性思维的认可。
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引用次数: 0
Lead-In Parenteral Anticoagulation Prior to Direct Oral Anticoagulation for Cerebral Venous Thrombosis. 在直接口服抗凝剂治疗脑静脉血栓之前,先行输入肠外抗凝剂。
IF 2.9 4区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2024-11-11 DOI: 10.1017/cjn.2024.325
Thalia S Field, Liqi Shu, Diana J Kim, Piers Klein, James E Siegler, Mary Cushman, Lily W Zhou, Thanh N Nguyen, Shadi Yaghi

Background: Growing evidence suggests that direct oral anticoagulants (DOACs) may be suitable for cerebral venous thrombosis (CVT). The optimal strategy regarding lead-in parenteral anticoagulation (PA) prior to DOAC is unknown.

Methods: In this post hoc analysis of the retrospective ACTION-CVT study, we compared patients treated with DOACs as part of routine care: those given "very early" DOAC (no PA), "early" (<5 days PA) and "delayed" (5-21 days PA). We compared baseline characteristics and outcomes between the very early/early and delayed groups. The primary outcome was a composite of day-30 CVT recurrence/extension, new peripheral venous thromboembolism, cerebral edema and intracranial hemorrhage.

Results: Of 231 patients, 11.7% had very early DOAC, 64.5% early (median [IQR] 2 [1-2] days) and 23.8% delayed (5 [5-6] days). More patients had severe clinical/radiological presentations in the delayed group; more patients had isolated headaches in the very early/early group. Outcomes were better in the very early/early groups (90-day modified Rankin Scale of 0-2; 94.3% vs. 83.9%). Primary outcome events were rare and did not differ significantly between groups (2.4% vs. 2.1% delayed; adjusted HR 1.49 [95%CI 0.17-13.11]).

Conclusions: In this cohort of patients receiving DOAC for CVT as part of routine care, >75% had <5 days of PA. Those with very early/early initiation of DOAC had less severe clinical presentations. Low event rates and baseline differences between groups preclude conclusions about safety or effectiveness. Further prospective data will inform care.

背景:越来越多的证据表明,直接口服抗凝剂(DOAC)可能适用于脑静脉血栓形成(CVT)。在使用 DOAC 之前先行肠外抗凝(PA)的最佳策略尚不清楚:在这项回顾性 ACTION-CVT 研究的事后分析中,我们比较了作为常规治疗一部分接受 DOACs 治疗的患者:"非常早期 "DOAC(无 PA)患者、"早期 "DOAC(无 PA)患者、"非常早期 "DOAC(无 PA)患者和 "非常早期 "DOAC(无 PA)患者:在 231 名患者中,11.7% 的患者接受了 "极早期 "DOAC,64.5% 的患者接受了 "早期 "DOAC(中位数[IQR] 2 [1-2] 天),23.8% 的患者接受了 "延迟 "DOAC(5 [5-6] 天)。延迟组中有更多患者出现严重的临床/放射学表现;极早期/早期组中有更多患者出现孤立性头痛。极早期/早期组的疗效更好(90 天改良兰金量表 0-2 分;94.3% 对 83.9%)。主要结局事件很少发生,组间差异不大(2.4% 对 2.1%;调整后 HR 1.49 [95%CI 0.17-13.11]):结论:在这批接受 DOAC 作为常规治疗的 CVT 患者中,超过 75% 的患者有以下症状
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引用次数: 0
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Canadian Journal of Neurological Sciences
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