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Vitamin D in amyotrophic lateral sclerosis. 肌萎缩性侧索硬化症中的维生素D。
Q2 Medicine Pub Date : 2017-01-01 DOI: 10.11138/fneur/2017.32.1.035
L Libonati, E Onesti, M C Gori, Mauro Ceccanti, C Cambieri, A Fabbri, V Frasca, M Inghilleri

Vitamin D supplementation has been proposed as a potential treatment to delay amyotrophic lateral sclerosis (ALS) progression. The aims of this study were to compare retrospectively vitamin D blood levels in ALS patients with those in healthy subjects; to correlate vitamin D blood levels with clinical functions in patients; and to evaluate whether administration of vitamin D could modify the clinical progression of the disease. Vitamin D blood levels were evaluated in 57ALS patients and in 57 healthy subjects. In the ALS patients the following clinical variables were evaluated every 3 months: Medical Research Council scale (MRC) score; revised ALS functional rating scale (ALSFRS-R) score; forced vital capacity (FVC). Twentyfour patients were treated with high doses of cholecalciferol. No significant differences were found between the vitamin D blood levels in the ALS patients (18.8 ± 12.2) and the healthy subjects (20.7 ± 10.1). The vitamin D levels in the ALS patientsdid not correlate with recorded clinical parameters. No clinical differences in terms of ALSFRS-R, MRC or FVC were found between the treated and the untreated patients over time. In ALS, as in other chronic neurological diseases, levels of vitamin D in blood appeared reduced, but no difference was found between the levels in ALS patients and in healthy subjects. Oral vitamin D supplementation in ALS patients was not associated with better prognosis in comparison with untreated ALS patients. Further prospective controlled studies are needed to clarify the effect of vitamin D on the progression of ALS disease.

维生素D补充剂被认为是延缓肌萎缩侧索硬化症(ALS)进展的一种潜在治疗方法。本研究的目的是回顾性比较ALS患者与健康人血液中的维生素D水平;将患者血液中的维生素D水平与临床功能联系起来;并评估服用维生素D是否可以改变疾病的临床进展。对57名als患者和57名健康受试者的血液中维生素D水平进行了评估。在ALS患者中,每3个月评估以下临床变量:医学研究委员会量表(MRC)评分;修订ALS功能评定量表(ALSFRS-R)评分;强制肺活量24例患者接受高剂量胆钙化醇治疗。ALS患者血维生素D水平(18.8±12.2)与健康者(20.7±10.1)无显著差异。ALS患者体内的维生素D水平与记录的临床参数无关。随着时间的推移,治疗组和未治疗组在ALSFRS-R、MRC或FVC方面没有临床差异。与其他慢性神经系统疾病一样,ALS患者血液中的维生素D水平似乎有所降低,但在ALS患者和健康受试者之间的水平没有发现差异。与未经治疗的ALS患者相比,口服维生素D补充剂与更好的预后无关。需要进一步的前瞻性对照研究来阐明维生素D对ALS疾病进展的影响。
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引用次数: 26
Back to the future: 30th anniversary of deep brain stimulation for Parkinson's disease. 回到未来:帕金森病深部脑刺激30周年纪念。
Q2 Medicine Pub Date : 2017-01-01 DOI: 10.11138/fneur/2017.32.1.005
N G Pozzi, Claudio Pacchetti
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引用次数: 2
Reappraisal of the F/M amplitude ratio in carpal tunnel syndrome. 腕管综合征F/M振幅比的重新评估。
Q2 Medicine Pub Date : 2017-01-01 DOI: 10.11138/fneur/2017.32.1.023
F Ginanneschi, M Mondelli, A Aretini, Alessandro Rossi

The F-wave/M-wave amplitude (F/M-amp) ratio has been shown to be increased in peripheral neuropathies, provided the maximum M-wave is relatively preserved. Reduced M-wave amplitudes and central facilitation of antidromically-induced reactivation of the anterior horn cells' axon hillocks (F-wave) are believed to contribute to higher F/M-amp ratios. The present study was undertaken to re-evaluate mechanisms responsible for higher F/M-amp ratios in carpal tunnel syndrome (CTS). We enrolled 232 cases affected by CTS and 108 controls. Fand M-wave amplitudes and F-wave chronodispersion were analyzed for the median and ulnar nerves. The F/M-amp ratio of the median nerve in CTS subjects with moderate-severe nerve damage was significantly higher than that of mild CTS subjects and controls. Chronodispersion of the median nerve F-wave increased with increasing CTS severity. We conclude that the relative preservation of the median nerve F-wave is due to damage to the large diameter muscle afferent fibers responsible for the monosynaptic response. Absence of the monosynaptic response makes the small motoneurons, usually inaccessible to the antidromic volley because of its collision with the orthodromic reflex volley, able to fire in the F-wave.

周围神经病变的F波/ m波振幅(F/M-amp)比增加,但最大m波相对保留。m波振幅的降低和抗药诱导的前角细胞轴突丘(F波)再激活的中枢促进作用被认为有助于提高F/M-amp比率。本研究旨在重新评估腕管综合征(CTS)中F/M-amp比值较高的机制。我们纳入了232例CTS患者和108例对照组。分析正中神经和尺神经的f波、m波振幅和f波时频散。中重度神经损伤CTS组正中神经F/M-amp比值显著高于轻度CTS组和对照组。随着CTS严重程度的增加,正中神经f波的时间离散度增加。我们得出结论,中央神经f波的相对保存是由于负责单突触反应的大直径肌肉传入纤维的损伤。单突触反应的缺失使得小的运动神经元能够发射f波,而这些小的运动神经元通常因为与正形反射阵的碰撞而无法进入反形反射阵。
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引用次数: 2
A retrospective analysis of 254 acute stroke cases admitted to two university hospitals in Beirut: classification and associated factors. 贝鲁特两所大学医院收治的254例急性脑卒中病例的回顾性分析:分类及相关因素
Q2 Medicine Pub Date : 2017-01-01 DOI: 10.11138/fneur/2017.32.1.041
N Lahoud, Marie-Helene Abbas, Pascale Salameh, N Saleh, Samer Abes, Hassan Hosseini, Souheil Gebeily

Lebanon is a developing country where the prevalence of stroke subtypes and their correlation with risk factors have not been studied, even though stroke management is an ongoing major national healthcare challenge. In a retrospective study conducted in two university hospitals, data were collected on all stroke cases admitted in 2012 and 2013. Ischemic strokes were then classified according to a modified TOAST classification. A total of 254 inpatients (mean age 68.41 years ±13.34, 55.1% males) was included in the study; of these, 15% had had a hemorrhagic stroke and was therefore excluded. Conversely to findings from studies in other Arab and Asian countries, where small vessel disease is the most frequent subtype, our study showed a predominance of large artery atherosclerosis (53.5%) which, in comparison with other subtypes, was found to be associated with dyslipidemia (OR= 3.82, 95% CI= [1.76-8.28]; p=0.001). Cardioembolic stroke and small vessel disease were found to be positively associated with aging and living in Beirut, respectively. Larger studies are needed to explain these findings.

黎巴嫩是一个发展中国家,尽管中风管理是一个持续的主要国家卫生保健挑战,但尚未研究中风亚型的患病率及其与危险因素的相关性。在两所大学医院进行的回顾性研究中,收集了2012年和2013年入院的所有中风病例的数据。然后根据改进的TOAST分类对缺血性中风进行分类。共纳入254例住院患者(平均年龄68.41岁±13.34岁,男性55.1%);在这些人中,有15%患有出血性中风,因此被排除在外。与其他阿拉伯和亚洲国家的研究结果相反,在这些国家,小血管疾病是最常见的亚型,我们的研究显示大动脉粥样硬化占主导地位(53.5%),与其他亚型相比,发现与血脂异常相关(OR= 3.82, 95% CI= [1.76-8.28];p = 0.001)。心脏栓塞性中风和小血管疾病分别与年龄和居住在贝鲁特呈正相关。需要更大规模的研究来解释这些发现。
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引用次数: 9
In the face of pain there are no heroes. An open letter to the members of the European Parliament about ensuring in the whole Europe high quality care, research, equitable employment opportunities, and socio-economic support for Cluster Headache (CH) pati. 面对疼痛,没有英雄。致欧洲议会成员的一封公开信,内容涉及确保在整个欧洲为丛集性头痛(CH)患者提供高质量的护理、研究、公平的就业机会和社会经济支持。
Q2 Medicine Pub Date : 2017-01-01
Paolo Rossi, A Craven, E R De La Torra
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引用次数: 0
Network functional connectivity and whole-brain functional connectomics to investigate cognitive decline in neurodegenerative conditions. 网络功能连接和全脑功能连接组学研究神经退行性疾病的认知能力下降。
Q2 Medicine Pub Date : 2016-12-01 DOI: 10.11138/FNEUR/2016.31.4.191
O. Dipasquale, M. Cercignani
Non-invasive mapping of brain functional connectivity (FC) has played a fundamental role in neuroscience, and numerous scientists have been fascinated by its ability to reveal the brain's intricate morphology and functional properties. In recent years, two different techniques have been developed that are able to explore FC in pathophysiological conditions and to provide simple and non-invasive biomarkers for the detection of disease onset, severity and progression. These techniques are independent component analysis, which allows a network-based functional exploration of the brain, and graph theory, which provides a quantitative characterization of the whole-brain FC. In this paper we provide an overview of these two techniques and some examples of their clinical applications in the most common neurodegenerative disorders associated with cognitive decline, including mild cognitive impairment, Alzheimer's disease, Parkinson's disease, dementia with Lewy Bodies and behavioral variant frontotemporal dementia.
脑功能连接(FC)的非侵入性映射在神经科学中发挥了重要作用,许多科学家被其揭示大脑复杂形态和功能特性的能力所吸引。近年来,已经开发了两种不同的技术,能够在病理生理条件下探索FC,并为检测疾病的发生、严重程度和进展提供简单且无创的生物标志物。这些技术是独立成分分析和图论,前者允许对大脑进行基于网络的功能探索,后者提供全脑FC的定量表征。在本文中,我们概述了这两种技术及其在最常见的与认知能力下降相关的神经退行性疾病中的临床应用,包括轻度认知障碍、阿尔茨海默病、帕金森病、路易体痴呆和行为变异性额颞叶痴呆。
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引用次数: 27
Modeling white matter microstructure. 模拟白质微观结构。
Q2 Medicine Pub Date : 2016-10-01 DOI: 10.11138/FNEUR/2016.31.4.217
T. Duval, N. Stikov, J. Cohen-Adad
Quantitative magnetic resonance imaging can be combined with advanced biophysical models to measure microstructural features of white matter. Non-invasive microstructural imaging has the potential to revolutionize neuroscience, and acquiring these measures in clinically feasible times would greatly improve patient monitoring and clinical studies of drug efficacy. However, a good understanding of microstructural imaging techniques is essential to set realistic expectations and to prevent over-interpretation of results. This review explains the methodology behind microstructural modeling and imaging, and gives an overview of the breakthroughs and challenges associated with it.
定量磁共振成像可以与先进的生物物理模型相结合,测量白质的微观结构特征。非侵入性显微结构成像有可能彻底改变神经科学,在临床可行的时间内获得这些措施将大大改善患者监测和药物疗效的临床研究。然而,对显微结构成像技术的良好理解对于设定切合实际的期望和防止过度解释结果至关重要。这篇综述解释了微观结构建模和成像背后的方法,并给出了与之相关的突破和挑战的概述。
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引用次数: 26
Impact of cerebellar atrophy on cortical gray matter and cerebellar peduncles as assessed by voxel-based morphometry and high angular resolution diffusion imaging. 基于体素的形态测量和高角分辨率扩散成像评估小脑萎缩对皮质灰质和小脑蒂的影响。
Q2 Medicine Pub Date : 2016-10-01 DOI: 10.11138/FNEUR/2016.31.4.239
M. Dayan, G. Olivito, M. Molinari, M. Cercignani, M. Bozzali, M. Leggio
In recent years the cerebellum has been attributed amore important role in higher-level functions than previously believed. We examined a cohort of patients suffering from cerebellar atrophy resulting in ataxia, with two main objectives: first to investigate which regions of the cerebrum were affected by the cerebellar degeneration, and second to assess whether diffusion magnetic resonance imaging (dMRI) metrics within the medial (MCP) and superior cerebellar peduncle (SCP) - namely fractional anisotropy (FA) and radial diffusivity (RD) - could be used as a biomarker in patients with this condition. Structural and dMRI data of seven patients with cerebellar atrophy (2 with spinocerebellar atrophy type 2, 1 with Friedreich's ataxia, 4 with idiopathic cerebellar ataxia) and no visible cortical lesions or cortical atrophy were investigated with Freesurfer and voxel-based morphometry (VBM) of gray matter (GM) as well as MCP and SCP FA maps. Correlations of MCP and SCP mean FA with ataxia scores and subscores were also evaluated. Freesurfer showed that patients had significantly reduced volume of the thalamus, ventral diencephalon and pallidum. VBM also demonstrated significantly lower local GM volumes in patients, notably in the head of the caudate nucleus, posterior cingulate gyrus and orbitofrontal cortex bilaterally, as well as in Broca's area in the left hemisphere, and a significant increase in RD in the MCP and SCP of both hemispheres. A significant correlation was found between MCP mean FA and total ataxia score (R=-0.7, p=0.03), and subscores for kinetic functions (R=-0.74, p=0.03) and oculomotor disorders (R=-0.70, p=0.04). The regions of the cerebrum found to have significantly lower local GM volumes have been described to be involved in higher-level cerebellar functions such as initiation of voluntary movements, emotional control, memory retrieval and general cognition. Our findings corroborate recent research pointing to a more extensive corticocerebellar system than previously thought. The significant difference in the MCP and SCP dMRI metrics between patients and controls as well as the significant correlation with ataxia total score and subscores support the use of dMRI metrics as an imaging biomarker for cerebellar degeneration and ataxia.
近年来,人们认为小脑在高级功能中的作用比以前认为的更重要。我们检查了一组患有小脑萎缩导致共济失调的患者,主要有两个目的:第一,研究大脑的哪些区域受到小脑变性的影响,第二,评估内侧(MCP)和上小脑脚(SCP)内的弥散性磁共振成像(dMRI)指标-即分数各向异性(FA)和径向弥散性(RD) -是否可以用作患有这种疾病的患者的生物标志物。采用Freesurfer和基于体素的灰质形态测量(VBM)以及MCP和SCP FA图对7例无明显皮质病变或皮质萎缩的小脑萎缩患者(2例为脊髓小脑萎缩2型,1例为friedrreich 's共济失调,4例为特发性小脑共济失调)的结构和dMRI数据进行了研究。评估MCP和SCP平均FA与共济失调评分和亚评分的相关性。Freesurfer显示,患者的丘脑、腹侧间脑和苍白球的体积明显减少。VBM还显示患者的局部GM体积显著降低,特别是在双侧尾状核头部、扣带回后部和眶额皮质以及左半球Broca区,两半球MCP和SCP的RD显著增加。MCP平均FA与共济失调总分(R=-0.7, p=0.03)、运动功能亚评分(R=-0.74, p=0.03)和动眼肌障碍亚评分(R=-0.70, p=0.04)之间存在显著相关性。被发现具有显著较低局部GM体积的大脑区域被描述为涉及高级小脑功能,如自发运动的启动、情绪控制、记忆检索和一般认知。我们的发现证实了最近的研究指出一个比以前认为的更广泛的皮质小脑系统。MCP和SCP dMRI指标在患者和对照组之间的显著差异以及与共济失调总分和亚分的显著相关性支持dMRI指标作为小脑变性和共济失调的成像生物标志物的使用。
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引用次数: 19
Magnetic resonance imaging and positron emission tomography in the diagnosis of neurodegenerative dementias. 磁共振成像和正电子发射断层扫描在神经退行性痴呆诊断中的应用。
Q2 Medicine Pub Date : 2016-10-01 DOI: 10.11138/FNEUR/2016.31.4.205
A. Del Sole, S. Malaspina, A. Magenta Biasina
Neuroimaging, both with magnetic resonance imaging (MRI) and positron emission tomography (PET), has gained a pivotal role in the diagnosis of primary neurodegenerative diseases. These two techniques are used as biomarkers of both pathology and progression of Alzheimer's disease (AD) and to differentiate AD from other neurodegenerative diseases. MRI is able to identify structural changes including patterns of atrophy characterizing neurodegenerative diseases, and to distinguish these from other causes of cognitive impairment, e.g. infarcts, space-occupying lesions and hydrocephalus. PET is widely used to identify regional patterns of glucose utilization, since distinct patterns of distribution of cerebral glucose metabolism are related to different subtypes of neurodegenerative dementia. The use of PET in mild cognitive impairment, though controversial, is deemed helpful for predicting conversion to dementia and the dementia clinical subtype. Recently, new radiopharmaceuticals for the in vivo imaging of amyloid burden have been licensed and more tracers are being developed for the assessment of tauopathies and inflammatory processes, which may underlie the onset of the amyloid cascade. At present, the cerebral amyloid burden, imaged with PET, may help to exclude the presence of AD as well as forecast its possible onset. Finally PET imaging may be particularly useful in ongoing clinical trials for the development of dementia treatments. In the near future, the use of the above methods, in accordance with specific guidelines, along with the use of effective treatments will likely lead to more timely and successful treatment of neurodegenerative dementias.
神经影像学,无论是磁共振成像(MRI)还是正电子发射断层扫描(PET),都在原发性神经退行性疾病的诊断中发挥了关键作用。这两种技术被用作阿尔茨海默病(AD)病理和进展的生物标志物,并用于区分AD与其他神经退行性疾病。MRI能够识别结构变化,包括神经退行性疾病特征的萎缩模式,并将其与其他认知障碍原因(如梗死、占位性病变和脑积水)区分开来。PET被广泛用于识别葡萄糖利用的区域模式,因为不同的脑糖代谢分布模式与不同亚型的神经退行性痴呆有关。PET在轻度认知障碍中的应用,尽管存在争议,但被认为有助于预测痴呆和痴呆临床亚型的转化。最近,用于淀粉样蛋白负荷体内成像的新放射性药物已经获得许可,更多的示踪剂正在开发中,用于评估tau病变和炎症过程,这可能是淀粉样蛋白级联发病的基础。目前,PET成像的大脑淀粉样蛋白负荷可能有助于排除AD的存在并预测其可能的发病。最后,PET成像可能在正在进行的痴呆症治疗发展的临床试验中特别有用。在不久的将来,按照具体的指导方针使用上述方法,加上使用有效的治疗方法,可能会更及时、更成功地治疗神经退行性痴呆。
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引用次数: 25
Reconstructing contralateral fiber tracts: methodological aspects of cerebello-thalamocortical pathway reconstruction. 重建对侧纤维束:小脑-丘脑皮质通路重建的方法学方面。
Q2 Medicine Pub Date : 2016-10-01 DOI: 10.11138/FNEUR/2016.31.4.229
Fulvia Palesi, J. Tournier, F. Calamante, N. Muhlert, G. Castellazzi, D. Chard, E. D’Angelo, C. Wheeler-Kingshott
The identification of pathways connecting the cerebral cortex with subcortical structures is critical to understanding how large-scale brain networks operate. The cerebellum, for example, is known to project numerous axonal bundles to thecerebral cortex passing through the thalamus. This paper focuses on the technical details of cerebello-thalamo-cortical pathway reconstruction using advanced diffusion MRI techniques in humans in vivo. Pathways reconstructed using seed/target placement on super-resolution maps, created with track density imaging (TDI), were compared with those reconstructed by defining regions of interest (ROIs) on non-diffusion weighted images (b0). We observed that the reconstruction of the pathways was more anatomically accurate when using ROIs placed on TDI rather than on b0 maps, while inter-subject variability and reproducibility were similar between the two methods. Diffusion indices along pathways showed a position-dependent specificity that will need to be taken into consideration in future clinical investigations.
识别连接大脑皮层和皮层下结构的通路对于理解大规模大脑网络是如何运作的至关重要。例如,小脑通过丘脑向大脑皮层投射大量的轴突束。本文重点介绍了利用先进的扩散MRI技术在人体内重建小脑-丘脑-皮层通路的技术细节。在用轨迹密度成像(TDI)创建的超分辨率地图上使用种子/目标放置重建的路径,与在非扩散加权图像(b0)上定义感兴趣区域(roi)重建的路径进行比较。我们观察到,当将roi放置在TDI而不是b0地图上时,通道的重建在解剖学上更加准确,而两种方法之间的受试者间变异性和可重复性相似。扩散指数沿通路显示位置依赖性特异性,将需要考虑在未来的临床研究。
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引用次数: 11
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Functional neurology
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