Neurology
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Clinical and Radiographic Improvement Following Steroid Therapy in Subacute Post-Traumatic Ascending Myelopathy
亚急性创伤后上升性脊髓病类固醇治疗后的临床和影像学改善
A 47-year-old woman struck by a car sustained a T11 chance fracture stabilized with instrumented fusion. 3 weeks later, she developed progressive (over 48 hours) paresthesias and weakness of her legs and urinary retention. Examination revealed paraplegia, asymmetric hyper-reflexia, patchy pinprick sensation, and decreased vibration sense. Myelopathy screening (CSF, serology) was negative including oligoclonal bands, anti-AQP4, and anti-MOG. Clinical and imaging findings were consistent with subacute posttraumatic ascending myelopathy (SPAM, Figure). Treated with a 5-day course of IV methylprednisone 1g, she recovered significant strength and sensation in 1 week, although some urinary retention persisted. At 3-month follow-up, remaining symptoms were confined to distal paresthesias.
一名47岁的妇女被汽车撞倒,并通过器械融合术稳定了T11机会骨折。3周后,她出现进行性 (超过48小时) 感觉异常,腿部无力和尿潴留。检查发现截瘫,不对称反射亢进,片状针刺感和振动感下降。脊髓病筛查 (CSF,血清学) 阴性,包括寡克隆带,anti-AQP4和抗MOG。临床和影像学表现与亚急性创伤后上升性脊髓病一致 (SPAM,图)。用静脉注射甲基强的松1g的5天疗程治疗,她在1周内恢复了明显的力量和感觉,尽管一些尿潴留持续存在。在3个月的随访中,其余症状仅限于远端感觉异常。
REF: Adegunna OE, Rastogi A, Chan Smyth N, Mandell DM, Fasano A. Clinical and Radiographic Improvement Following Steroid Therapy in Subacute Post-Traumatic Ascending Myelopathy. Neurology. 2024;103(11):e210050. doi:10.1212/WNL.0000000000210050 PMID: 39496104
Teaching NeuroImage: Use of Arterial Spin Labeling to Differentiate Ictal Phenomenon From Tumor Progression in Grade 3 Astrocytoma
神经影像教学: 使用动脉自旋标记来区分3级星形细胞瘤的发作现象和肿瘤进展
A 39-year-old woman with grade 3 left parietal isocitrate dehydrogenase–mutant astrocytoma treated with surgery, radiation, and chemotherapy presented with aphasia and impaired consciousness. EEG showed seizures from the left parietal region consistent with nonconvulsive status epilepticus. Brain MRI showed the surgical bed without contrast enhancement (Figure 1A). Arterial spin labeling (ASL) was obtained to determine regional cerebral blood flow, showing markedly increased cerebral blood flow (CBF) in the left temporoparietal lobe (Figure 1, C and D) with diffusion restriction (Figure 1B). Imaging 2 weeks later after seizure control showed complete resolution (Figure 2) indicating a peri-ictal phenomenon.1 ASL-MR perfusion is a highly sensitive marker of increased CBF with its emerging application in distinguishing tumor progression (increased CBF) from post-treatment changes such as radiation necrosis (reduced CBF).1,2 This case highlights the utility of ASL-MR perfusion in differentiating the peri-/postictal phenomenon from tumor progression when used in clinical neuro-oncology practice.
一名39岁的女性,患有3级左顶叶异柠檬酸脱氢酶突变星形细胞瘤,接受手术,放射和化学疗法治疗,表现为失语症和意识障碍。脑电图显示左顶叶区域的癫痫发作与非惊厥性癫痫持续状态一致。脑MRI显示手术床无对比增强 (图1A)。获得动脉自旋标记 (ASL) 以确定局部脑血流量,显示左颞顶叶中的脑血流量 (CBF) 显着增加 (图1,C和D),扩散受限 (图1B)。癫痫发作控制后2周后的成像显示完全消退 (图2),表明发作周围现象。1 ASL-MR灌注是CBF升高的高度敏感标志物,其新兴应用是区分肿瘤进展 (CBF升高) 和放射坏死等治疗后变化(降低的CBF)。1,2这种情况突出了asl-mr灌注在临床神经肿瘤学实践中用于区分肿瘤周围/肿瘤后现象与肿瘤进展的实用性。
REF: Zahid A, Thatikonda N, Dubey P, Tremont-Lukats IW. Teaching NeuroImage: Use of Arterial Spin Labeling to Differentiate Ictal Phenomenon From Tumor Progression in Grade 3 Astrocytoma. Neurology. 2024;103(11):e210048. doi:10.1212/WNL.0000000000210048 PMID: 39496101
Teaching Video NeuroImage: Noogenic Epilepsy With Orofacial Reflex Myoclonus: A Rare Distinct Electroclinical Syndrome
教学视频神经影像: 伴有口面部反射性肌阵挛的生性癫痫: 一种罕见的独特的电临床综合征
A 20-year-old man presented with a 15-year history of facial jerks, preserved awareness, and generalized tonic-clonic seizures triggered by complex mental tasks, without limb jerks or staring spells (Video 1). His ictal and interictal data showed frontally dominant generalized polyspike-wave discharges without focal slowing (Figure). MRI brain was normal. Video telemetry recorded multiple facial jerks 5 minutes after complex mental tasks including calculation, abstract reasoning, playing chess, sketching, and writing poems, which are inherently subjective (Video 1). He was optimized on sodium valproate and lamotrigine. At 3-month follow-up, he remains seizure-free. The complex stimuli involving higher cognitive ‘thinking’ processes in NE are generated by hyperexcitable neurons involving the frontoparietal network.1Noogenic (thinking) epilepsy (NE) is a rare reflex epilepsy induced by complex thinking.1 Orofacial reflex myoclonia (ORM) is classically described in reading epilepsy triggered by language-related activities.2 Praxis-triggered ORM may occur in JME.2 To our knowledge, ORM in NE has not been described. The complex stimuli involving higher cognitive ‘thinking’ processes in NE are generated by hyperexcitable neurons involving the frontoparietal network.
一名20岁的男子有15年的面部抽搐史,保留意识,以及由复杂的心理任务引发的全身性强直-阵挛发作,没有肢体抽搐或凝视咒语 (视频1)。他的发作和发作间期数据显示,正面占优势的广义多尖峰波放电没有局灶性减慢 (图)。脑部MRI正常。视频遥测记录了复杂的心理任务5分钟后的多次面部抽搐,这些任务包括计算,抽象推理,下棋,素描和写诗,这些本质上是主观的 (视频1)。他对丙戊酸钠和拉莫三嗪进行了优化。在3个月的随访中,他仍然没有癫痫发作。NE中涉及高级认知 “思维” 过程的复杂刺激是由涉及额顶网络的超兴奋神经元产生的。1非致 (思维) 癫痫 (NE) 是由复杂思维引起的罕见反射性癫痫。1口面反射肌阵挛 (ORM)在阅读由语言相关活动触发的癫痫中被经典地描述。2实践触发的ORM可能发生在jme2中。据我们所知,NE中的ORM尚未被描述。NE中涉及高级认知 “思维” 过程的复杂刺激是由涉及额叶网络的超兴奋神经元产生的。
REF: Nandana J, Manisha KY, Surabhi P, et al. Teaching Video NeuroImage: Noogenic Epilepsy With Orofacial Reflex Myoclonus: A Rare Distinct Electroclinical Syndrome. Neurology. 2024;103(11):e210045. doi:10.1212/WNL.0000000000210045 PMID: 39496103
Epidemiologic Study of Myasthenia Gravis in the Elderly US Population: A Longitudinal Analysis of the Medicare Claims Database, 2006–2019
美国老年人群中重症肌无力的流行病学调查: 医疗保险索赔数据库的纵向分析,2006-2019
Epidemiologic studies suggest increasing incidence and prevalence of myasthenia gravis (MG) among the elderly population outside the United States. We aimed to provide an estimation of MG incidence and prevalence and their trend among the Medicare Fee-For-Service (FFS)-covered elderly US population. We performed a retrospective longitudinal study using Medicare claims data (2006-2019). Study-eligible beneficiaries were aged 65 years and older, had at least 1 month of FFS Part A/B coverage, and were without any health maintenance organization insurance coverage. Increasing trends in MG prevalence and incidence in the elderly US population, with variation in rates of certain subgroups, are confirmed in this 14-year period.
流行病学研究表明,在美国以外的老年人群中,重症肌无力 (MG) 的发病率和患病率不断增加。我们的目的是提供一个估计MG发病率和患病率及其趋势在医疗保险按服务付费 (FFS) 覆盖的美国老年人口。我们使用医疗保险索赔数据 (2006-2019) 进行了一项回顾性纵向研究。符合研究条件的受益人年龄在65岁以上,至少有1个月的FFS A/B部分保险,并且没有任何健康维护组织保险。在这14年期间,证实了美国老年人群中MG患病率和发病率的上升趋势,以及某些亚组的发病率变化。
REF: Bruckman D, Lee I, Schold JD, et al. Epidemiologic Study of Myasthenia Gravis in the Elderly US Population: A Longitudinal Analysis of the Medicare Claims Database, 2006-2019. Neurology. 2024;103(10):e210005. doi:10.1212/WNL.0000000000210005 PMID: 39496108
Disentangling Neurodegeneration From Aging in Multiple Sclerosis Using Deep Learning: The Brain-Predicted Disease Duration Gap
使用深度学习从多发性硬化症的衰老中解开神经变性: 大脑预测的疾病持续时间差距
Disentangling brain aging from disease-related neurodegeneration in patients with multiple sclerosis (PwMS) is increasingly topical. The brain-age paradigm offers a window into this problem but may miss disease-specific effects. In this study, we investigated whether a disease-specific model might complement the brain-age gap (BAG) by capturing aspects unique to MS. In this retrospective study, we collected 3D T1-weighted brain MRI scans of PwMS to build (1) a cross-sectional multicentric cohort for age and disease duration (DD) modeling and (2) a longitudinal single-center cohort of patients with early MS as a clinical use case. The brain-predicted DD gap is sensitive to MS-related lesions and brain atrophy, adds to the brain-age paradigm in explaining physical disability both cross-sectionally and longitudinally, and may be used as an MS-specific biomarker of disease severity and progression.
在多发性硬化症 (PwMS) 患者中,将大脑衰老与疾病相关的神经退行性变相结合越来越成为热门话题。大脑年龄范式为这个问题提供了一个窗口,但可能会错过特定疾病的影响。在这项研究中,我们调查了疾病特异性模型是否可以通过捕获MS特有的方面来补充大脑年龄差距 (BAG)。在这项回顾性研究中,我们收集了pwm的3D T1-weighted脑MRI扫描,以建立 (1) 年龄和疾病持续时间 (DD) 建模的横断面多中心队列和 (2)早期MS患者的纵向单中心队列作为临床使用案例。脑预测的DD间隙对MS相关病变和脑萎缩敏感,在解释横向和纵向身体残疾方面增加了脑年龄范式,并可用作疾病严重程度和进展的MS特异性生物标志物。
REF: Pontillo G, Prados F, Colman J, et al. Disentangling Neurodegeneration From Aging in Multiple Sclerosis Using Deep Learning: The Brain-Predicted Disease Duration Gap. Neurology. 2024;103(10):e209976. doi:10.1212/WNL.0000000000209976 PMID: 39496109
Lumipulse-Measured Cerebrospinal Fluid Biomarkers for the Early Detection of Alzheimer Disease
Lumipulse测量的脑脊液生物标志物用于阿尔茨海默病的早期检测
CSF biomarkers of Aβ42 and phosphorylated tau (p-tau181) are used clinically for the detection of Alzheimer disease (AD) pathology during life. CSF biomarker validation studies have largely used clinical diagnoses and/or amyloid PET imaging as the reference standard. The few existing CSF-to-autopsy studies have been restricted to late-stage AD. This CSF-to-autopsy study investigated associations between CSF biomarkers of AD and AD neuropathologic changes among brain donors who had normal cognition at the time of lumbar puncture (LP). This study supports Lumipulse-measured CSF Aβ42 and p-tau181 and, particularly, the ratio of p-tau181 to Aβ42, for the early detection of AD pathophysiologic processes. This study provides Class II evidence that Lumipulse measures of p-tau181/Aβ42 in the CSF accurately discriminated cognitively normal participants with and without Alzheimer disease neuropathologic change.
Aβ42和磷酸化tau (p-tau181) 的CSF生物标志物在临床上用于检测生命中的阿尔茨海默病 (AD) 病理。CSF生物标志物验证研究已大量使用临床诊断和/或淀粉样蛋白PET成像作为参考标准。现有的少数CSF到尸检研究仅限于晚期AD。这项CSF到尸检研究调查了在腰椎穿刺 (LP) 时具有正常认知的脑供体中AD的CSF生物标志物与AD神经病理变化之间的关联。这项研究支持Lumipulse测量的CSF Aβ42和p-tau181,尤其是p-tau181与Aβ42的比率,以早期发现AD病理生理过程。这项研究提供了II类证据,表明CSF中p-tau181/Aβ42的Lumipulse测量可以准确区分有或没有阿尔茨海默病神经病理变化的认知正常参与者。
REF: Safransky M, Groh JR, Blennow K, et al. Lumipulse-Measured Cerebrospinal Fluid Biomarkers for the Early Detection of Alzheimer Disease. Neurology. 2024;103(11):e209866. doi:10.1212/WNL.0000000000209866 PMID: 39496102
Association of Cerebrovascular Reactivity With 1-Year Imaging and Clinical Outcomes in Small Vessel Disease: An Observational Cohort Study
脑血管反应性与小血管病1年影像学和临床结局的关系: 一项观察性队列研究
In patients with cerebral small vessel disease (SVD), impaired cerebrovascular reactivity (CVR) is related to worse concurrent SVD burden, but less is known about cerebrovascular reactivity and long-term SVD lesion progression and clinical outcomes. We investigated associations between cerebrovascular reactivity and 1-year progression of SVD features and clinical outcomes. Between 2018 and 2021, we recruited patients from the Edinburgh/Lothian stroke services presenting with minor ischemic stroke and SVD features as part of the Mild Stroke Study 3, a prospective observational cohort study (ISRCTN 12113543). Lower baseline cerebrovascular reactivity predicted an increase in WMH and perivascular space volumes after 1 year. CVR should be considered in SVD future research and intervention studies.
在患有脑小血管病 (SVD) 的患者中,脑血管反应性 (CVR) 受损与并发SVD负担加重有关,但对脑血管反应性和长期SVD病变进展和临床结局的了解较少。我们调查了脑血管反应性与SVD特征和临床结局的1年进展之间的关系。在2018和2021之间,我们从爱丁堡/洛希安卒中服务中心招募了患有轻度缺血性卒中和SVD特征的患者,作为轻度卒中研究3的一部分,这是一项前瞻性观察性队列研究 (ISRCTN 12113543)。较低的基线脑血管反应性预测1年后WMH和血管周围空间体积的增加。在SVD未来的研究和干预研究中应考虑CVR。
REF: Sleight E, Stringer MS, Clancy U, et al. Association of Cerebrovascular Reactivity With 1-Year Imaging and Clinical Outcomes in Small Vessel Disease: An Observational Cohort Study. Neurology. 2024;103(11):e210008. doi:10.1212/WNL.0000000000210008 PMID: 39499872
Optimizing Anti–Myelin-Associated Glycoprotein and IgM-Gammopathy Testing for Neuropathy Treatment Evaluation
优化抗髓鞘相关糖蛋白和igm-γ 病测试用于神经病治疗评估
Patients with typical anti-myelin-associated glycoprotein (anti-MAG) neuropathy have IgM-gammopathy, mimic distal chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), and are treatment resistant. Anti-MAG patients go unrecognized when IgM-gammopathy is undetected or with atypical phenotypes. We investigated an optimal anti-MAG titration cutoff for excluding CIDP and the impact of IgM-gammopathy detection on neuropathy treatment evaluation without anti-MAG antibodies. A cutoff of 10,000 BTU seems optimal for typical anti-MAG neuropathy while ≥1,500 BTU reduces the likelihood of immune-treatable CIDP. Mass-Fix improves IgM-gammopathy detection in anti-MAG and other IgM-gammopathy neuropathies. Patients with IgM-gammopathy lacking MAG antibodies show reduced treatment response.
患有典型的抗髓鞘相关糖蛋白 (抗MAG) 神经病的患者患有igm-γ 病,模仿远端慢性炎症性脱髓鞘性多发性神经根神经病 (CIDP),并且对治疗有抵抗力。当未检测到igm-γ 病或具有非典型表型时,抗MAG患者无法识别。我们研究了排除CIDP的最佳抗MAG滴定截止值,以及IgM-丙种球蛋白病检测对无抗MAG抗体的神经病治疗评估的影响。对于典型的抗MAG神经病,10,000 BTU的临界值似乎是最佳的,而 ≥ 1,500 BTU则降低了可免疫治疗的CIDP的可能性。Mass-Fix可改善抗MAG和其他IgM-丙种球蛋白病神经病的IgM-丙种球蛋白病检测。缺乏MAG抗体的IgM-丙种球蛋白病患者的治疗反应降低。
REF: Klein CJ, Triplett JD, Murray DL, et al. Optimizing Anti-Myelin-Associated Glycoprotein and IgM-Gammopathy Testing for Neuropathy Treatment Evaluation. Neurology. 2024;103(11):e210000. doi:10.1212/WNL.0000000000210000 PMID: 39499873
Multimodal Assessment of the Origin of Myoclonus in Lance-Adams Syndrome
兰斯-亚当斯综合征肌阵挛起源的多模态评估
Lance-Adams syndrome (LAS), or chronic posthypoxic myoclonus, is a long-term disabling neurologic disorder occurring in survivors of anoxia. The cortical or subcortical origin of this myoclonus is unclear. We aimed to identify the neuroanatomical origin of myoclonus in LAS. We conducted a cross-sectional study and investigated patients diagnosed with LAS from the Department of Neurology of Pitié-Salpêtrière Hospital, using multimodal neurologic explorations: EEG with quantitative analyses, polygraphic EMG recording of myoclonus, coupled EEG-EMG analyses with jerk-locked back averaging, and 18fluorodeoxyglucose PET/CT imaging.Our findings, based on a large cohort of patients with LAS, strongly suggest a cortical myoclonus, originating within the motor cortex and related to epileptiform mechanisms.
Lance-adams综合征 (LAS) 或慢性缺氧后肌阵挛是一种长期致残的神经系统疾病,发生在缺氧幸存者中。这种肌阵挛的皮质或皮质下起源尚不清楚。我们旨在确定LAS中肌阵挛的神经解剖学起源。我们进行了一项横断面研究,并调查了pitié-salpêtrière医院神经内科诊断为LAS的患者,使用多模式神经系统探索: 定量分析的EEG,肌阵挛的多图EMG记录,结合jerk锁定的EEG-EMG分析平均和18氟脱氧葡萄糖PET/CT成像。我们的研究结果基于大量的LAS患者,强烈提示皮质肌阵挛,起源于运动皮层,与癫痫样机制有关。
REF: Vellieux G, Apartis E, Baudin P, et al. Multimodal Assessment of the Origin of Myoclonus in Lance-Adams Syndrome. Neurology. 2024;103(11):e209994. doi:10.1212/WNL.0000000000209994 PMID: 39499871
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