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Gerstmann-Straussler-Scheinker Disease - A Review Gerstmann-Straussler-Scheinker病综述
Pub Date : 2021-01-01 DOI: 10.36648/2171-6625.21.12.378
Pratham D Shetty
Gerstmann-Straussler-Scheinker Disease (GSS) is an extremely rare, usually familial, fatal prion disease. Such disease affects our one of the most important part of our body that is responsible for our thoughts and coordination of our sensory information, the Nervous System. Some particular variations in the PNRP gene leading to the atypical shape of the prion protein give rise to this disease. PRNP encodes a protein called prion protein (Prp). We are unaware of its functions but we do know that Prp plays a very significant role in the functioning of the human brain and other parts of the human nervous system. The destruction of neural cells due to the clumping of abnormal proteins is one of the characteristic features of this disease. The continuous deterioration of the section of our brain responsible for the motor control in a human body, the cerebellum, and different degrees of dementia are the main characteristics of GSS. Weakness in the legs, diminished reflexes, cognitive decline, ataxia including slurred speech and reduced coordination, and spasticity are some of the main symptoms seen in a person suffering from GSS. The median survival time from onset to death of GSS patients ranges from two to ten years after its diagnosis. The objective of this paper to gather all the data and information available about this rare disease so that future researchers who are interested in this field can refer to this paper without having to curate it all by themselves saving time and increasing their efficiency towards solving this mystery. We are very behind in the understanding of the pathophysiological processes that underlie this disease. Through this paper, we have analyzed and reviewed all the literature on this topic to summarize what is our current understanding of this disease and the possible treatments to cure it or alleviate its symptoms. Through a thorough literature review, we can conclude that research on this topic has potential. With mostly case reports on this disease, the research for its cure and treatments for alleviating its symptoms are not yet advanced.
Gerstmann-Straussler-Scheinker病(GSS)是一种极其罕见的,通常是家族性的,致命的朊病毒疾病。这种疾病影响我们身体最重要的部分之一,即神经系统,它负责我们的思想和感觉信息的协调。PNRP基因的一些特殊变异导致朊蛋白的非典型形状,从而引起这种疾病。PRNP编码一种叫做朊病毒蛋白(Prp)的蛋白质。我们不知道它的功能,但我们知道Prp在人类大脑和人类神经系统其他部分的功能中起着非常重要的作用。异常蛋白的结块导致神经细胞的破坏是本病的特征之一。我们大脑中负责人体运动控制的部分小脑的持续恶化和不同程度的痴呆是GSS的主要特征。腿部无力、反射能力减弱、认知能力下降、共济失调(包括言语不清、协调性下降)和痉挛是GSS患者的一些主要症状。GSS患者从发病到死亡的中位生存时间为诊断后2至10年。本文的目的是收集有关这种罕见疾病的所有可用数据和信息,以便未来对该领域感兴趣的研究人员可以参考这篇论文,而不必自行整理,从而节省时间并提高他们解决这一谜团的效率。我们对这种疾病背后的病理生理过程的理解非常落后。通过本文,我们分析和回顾了所有关于这一主题的文献,总结了我们目前对这种疾病的认识以及可能的治疗方法来治愈或缓解其症状。通过全面的文献回顾,我们可以得出结论,这一主题的研究是有潜力的。本病病例多见,治疗和缓解症状的研究尚不深入。
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引用次数: 1
Assessing Neural Oscillations during Erotic and Negative Emotional Pictures Viewing: An Event-Related Potential Study 情色与负面情绪图片观看时的神经振荡评估:事件相关电位研究
Pub Date : 2021-01-01 DOI: 10.36648/2171-6625.12.1.350
Fetlework Tenssay
The present study was designed to determine whether or not there are components of the ERPs that discriminate the erotic and negative pictures during affective picture processing. Participants viewed erotic, negative and neutral pictures form the International Affective Picture System (IAPS). Eighteen right-handed volunteer students from North-Eastern University participated in the experiment. Electrode from the frontal site (F3 and F4), C3 and C4 (central electrodes), P3 andP4 (parietal electrodes) and O1 and O2 (occipital electrodes) were averaged with the stimuli type and brain hemisphere. The change in spectral power of the brain signal and its main spectral bands of theta (3-7.5 Hz), Alpha (7.5-13 Hz) and Beta (13-25 Hz) were analyzed. Erotic stimuli showed ERP components deflected more negatively at the frontal electrode site than the negative picture stimuli during the time window of 200-300 ms whereas the negative valence stimuli processing reflected a positively twisted amplitude at the time window of 120-300 ms. The erotic and negative valence emotional picture stimuli show a significant difference in the spectral power of theta, alpha and beta frequency bands for the left vs. right hemisphere and frontal vs. occipital sites. Nonetheless, the time-frequency plots show us those emotionally valence visual stimuli triggered an increase of alpha band (7.5-13 Hz).
本研究旨在确定在情感图像处理过程中,erp中是否存在区分色情图片和消极图片的成分。参与者观看了国际情感图片系统(IAPS)中的情色、消极和中性图片。来自东北大学的18名右撇子志愿者参加了这项实验。额叶(F3和F4)、C3和C4(中央电极)、P3和p4(顶叶电极)、O1和O2(枕叶电极)电极的刺激类型和脑半球平均。分析脑信号的谱功率变化及其主要谱带theta (3-7.5 Hz)、Alpha (7.5-13 Hz)和Beta (13-25 Hz)。在200 ~ 300 ms的时间窗内,情色刺激的ERP分量在额电极部位呈现负向偏转,而负效刺激的ERP分量在120 ~ 300 ms的时间窗内呈现正向偏转。情色和负效性情绪图片刺激在左半球和右半球以及额叶和枕叶部位的θ、α和β频带频谱功率上存在显著差异。尽管如此,时间-频率图显示情绪性效价视觉刺激触发了α波段(7.5-13 Hz)的增加。
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引用次数: 0
How Brain Creates the Mind? Recent Advancements in the Brain Science 大脑如何创造思维?脑科学的最新进展
Pub Date : 2021-01-01 DOI: 10.36648/2171-6625.21.12.382
Pop-Jordanova Nada, Poppenk Jordan
The main question in neuroscience is how the material tissue (brain) is producing the non-material phenomena (mind; thought, memory, emotions). Recent advancements in the brain sciences have enabled researchers to determine locations and patterns of neural activation associated with various psychological functions. Still, the mind-brain puzzle is complex and not yet elucidate. The fundamental relevance of EEG spectra for mental activation (both, general and focused) can be related to the interaction of electric field with neuronal quantum dipoles. The obtained analytical expressions and numerical values based on quantum transition probabilities correspond to classical empirical results for arousal and attention, including the characteristic frequency dependence and intervals. This paper proposes a theoretical approach to explain the characteristic empirical interdependence between the states of arousal (representing the level of consciousness) and EEG activity. Consequently, we introduced mathematical formula to calculate field-dipole interactions, which corresponds to clinical states and can be considered as an integral brain state attribute, correlated to its electric, mental and metabolic activity. The article is a compilation of some our related articles, published before. There is a substantial distinction between the coarsegrained arousal (as background state of consciousness) and the fine-grained content of consciousness, whereby attention appears to be a bridge. However, deeper understanding of substrates and emergence of arousal, attention and consciousness must take into account the subtle interplay of neurophysical, neurochemical and neurobiological correlates.
神经科学的主要问题是物质组织(大脑)如何产生非物质现象(精神;思想、记忆、情感)。脑科学的最新进展使研究人员能够确定与各种心理功能相关的神经激活的位置和模式。尽管如此,心智和大脑的谜题仍然很复杂,尚未得到阐明。脑电图谱与精神活动(一般和集中)的基本相关性可以与电场与神经元量子偶极子的相互作用有关。得到的基于量子跃迁概率的解析表达式和数值符合经典的唤醒和注意的经验结果,包括特征频率依赖关系和间隔。本文提出了一种理论方法来解释觉醒状态(代表意识水平)与脑电图活动之间的典型经验相互依存关系。因此,我们引入数学公式来计算场偶极相互作用,它与临床状态相对应,可以被认为是一个完整的大脑状态属性,与它的电、精神和代谢活动相关。这篇文章是我们以前发表的一些相关文章的汇编。粗粒度的觉醒(作为意识的背景状态)和细粒度的意识内容之间存在着本质的区别,在细粒度的意识内容中,注意力似乎是一座桥梁。然而,更深层次的理解基底和觉醒,注意和意识的出现必须考虑到神经物理,神经化学和神经生物学相关的微妙相互作用。
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引用次数: 0
The Benefits of the Rehabilitation Program in Multiple Sclerosis 多发性硬化症康复计划的益处
Pub Date : 2021-01-01 DOI: 10.36648/2171-6625.21.12.380
F. Marcu
Background: Multiple sclerosis, characterized by demyelination or loss of myelin sheaths affects the discharge of nerve impulses and the ability of the central nervous system CNS, to transmit commands, resulting in disorders of functions that are directed by the CNS such as: sight, speech, walking, writing and memory. Depending on the location of the demyelination, patients have a wide range of symptoms that differ from person to person and may change as the condition progresses. The most common symptoms are: fatigue, balance and coordination disorders, vision and sensitivity disorders, pain and depression. Objective: In this study we aimed to demonstrate that the treatment of sclerosis by combining a drug treatment with a rehabilitation treatment, represented by physical therapy and occupational therapy, has superior therapeutic effects compared to simple drug treatment. Methods: In order to highlight the superior therapeutic efficacy of the mentioned combination, we studied, during one year, two groups of patients: the group that respects the home rehabilitation program and the group that does not respect the home rehabilitation program. In our study, we evaluated the following parameters: number of patients with falls, timed 25-Foot Walk and Multiple Sclerosis Quality of Life-54. Results: The results of our study highlight the superior therapeutic benefits of drug treatment associated with rehabilitation treatment. Conclusion: Patients who follow the rehabilitation program associated with drug treatment we found the following benefits: improving body stability; increase in muscle strength in the lower limbs; improving coordination and balance while walking; improving the QoL.
背景:多发性硬化症,以脱髓鞘或髓鞘丧失为特征,影响神经冲动的释放和中枢神经系统CNS传递命令的能力,导致由CNS指导的功能紊乱,如:视觉,语言,行走,写作和记忆。取决于脱髓鞘的位置,患者有各种各样的症状,这些症状因人而异,并可能随着病情的进展而改变。最常见的症状是:疲劳、平衡和协调障碍、视力和敏感性障碍、疼痛和抑郁。目的:在本研究中,我们旨在证明以物理治疗和职业治疗为代表的药物治疗与康复治疗相结合的方法治疗硬化症的效果优于单纯的药物治疗。方法:为了突出上述组合的优越疗效,我们对两组患者进行了为期一年的研究:尊重家庭康复方案组和不尊重家庭康复方案组。在我们的研究中,我们评估了以下参数:跌倒患者数量,25英尺步行时间和多发性硬化症生活质量54。结果:我们的研究结果突出了药物治疗与康复治疗相结合的优越性。结论:患者按照康复方案联合药物治疗,可获得以下益处:提高身体稳定性;下肢肌肉力量增加;提高行走时的协调性和平衡性;改善生活质量。
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引用次数: 0
Ruptured Infective (Mycotic) Intracranial Aneurysm Secondary to Bacterial Meningitis: A Case Report and Review of Literature 细菌性脑膜炎继发的感染性(真菌性)颅内动脉瘤破裂1例报告及文献复习
Pub Date : 2021-01-01 DOI: 10.36648/2171-6625.21.12.384
Supreeth N Gowda
Mycotic Aneurysm (MA), a rare complication of systemic infections, is a result of degradation and dilation of the arterial wall. Etiology can be due to endovascular seeding of infective emboli (e.g. infective endocarditis, bacteremia) or extravascular spread of infection (e.g. meningitis, osteomyelitis, orbital cellulitis). Infective Intracranial Aneurysms (IIA) is extremely rare and account for a small fraction of all intracranial aneurysms. Most IIA are caused by bacterial or fungal infections. IIA has a higher risk of rupture as compared to other aneurysms due to their rapid progression and increased vessel fragility. Diagnosis is mostly based on clinical history and neurovascular imaging. Medical management and endovascular intervention can be considered the first-line treatment for unruptured IIA. Open surgery is done in cases of complications or failed endovascular intervention. A case of suspected ruptured IIA secondary to bacterial meningitis is discussed in detail along with a review of the literature. Certain proposals on diagnosis, management, and complication have been put forth.
霉菌性动脉瘤(MA)是一种罕见的全身感染并发症,是动脉壁退化和扩张的结果。病因可能是由于血管内感染性栓塞的播散(如感染性心内膜炎、菌血症)或血管外感染的扩散(如脑膜炎、骨髓炎、眼眶蜂窝织炎)。感染性颅内动脉瘤(IIA)是非常罕见的,占所有颅内动脉瘤的一小部分。大多数IIA是由细菌或真菌感染引起的。与其他动脉瘤相比,IIA由于其快速发展和血管脆弱性增加而具有更高的破裂风险。诊断主要基于临床病史和神经血管影像学。医疗管理和血管内介入治疗可被认为是未破裂IIA的一线治疗。在并发症或血管内介入失败的情况下进行开放手术。一例疑似IIA破裂继发于细菌性脑膜炎的详细讨论与文献回顾。在诊断、处理和并发症方面提出了一些建议。
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引用次数: 0
Elastoplasty: A New Technique for Treatment of steoporotic Vertebral Compression Fractures 弹性成形术:治疗骨质疏松性椎体压缩性骨折的新技术
Pub Date : 2021-01-01 DOI: 10.36648/2171-6625.21.10.376
F. Villarejo, B. Mansilla, M. Budke
Objective: Vertebral compression fractures (VCF) cause significant disability and pain and are usually treated by vertebroplasty using polymethylmethacrylate (PMMA), which has several drawbacks, including fractures and exothermic neural damage associated to lea-kage. VK100 silicone is a new polymer for vertebroplasty to resolve these disadvantages. Material and Methods: This study follows the evolution of seventy patients with VCF who received a VK100 elastoplasty. The period of follow up was a year average. Results: Four patients had asymptomatic silicone leakage, none had neural damage and, at the end of follow up, there had been no adjacent fractures. Patients reported less pain. Conclusion: We conclude VK100 is a good option for vertebroplasty to treat osteoporotic spinal compression fractures.
目的:椎体压缩性骨折(VCF)会导致严重的残疾和疼痛,通常采用聚甲基丙烯酸甲酯(PMMA)椎体成形术治疗,该方法有几个缺点,包括骨折和放热神经损伤相关的渗漏。VK100硅胶是一种用于椎体成形术的新型聚合物,解决了这些缺点。材料和方法:本研究跟踪了70例接受VK100弹性成形术的VCF患者的进展。随访时间平均为一年。结果:4例患者无症状硅胶渗漏,无神经损伤,随访结束时无相邻骨折。患者报告疼痛减轻。结论:VK100椎体成形术是治疗骨质疏松性脊柱压缩性骨折的良好选择。
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引用次数: 0
Factors Contributing to the Length of Stay in Patients Admitted to the Epilepsy Monitoring Unit 影响癫痫病患者住院时间的因素
Pub Date : 2021-01-01 DOI: 10.36648/2171-6625.12.1.348
Ashwaq Alsulami, A. Khoja, Mohammad Alsumaili, Mohamed Alkhaja, M. Al-Khateeb
Electroencephalography with video monitoring (VEEG) is considered the gold standard for differentiating epileptic from non-epileptic events. The standardization of length of hospital stay (LOS) in the Epilepsy Monitoring Unit (EMU) setting is challenging due to the paroxysmal nature of seizures. This study evaluates the factors contributing to the LOS and days of VEEG recording with the EMU final diagnosis and plan. A retrospective study was conducted on inpatients admitted to the EMU, at a tertiary center from January to December 2016. All consecutive patients admitted to EMU during the study period were included. A total of 160 patients were enrolled. The mean age was 21.10 years (SD 13.33). Upon discharge, most of the patients were diagnosed with epileptic seizures, of whom 91 (56.9%) had focal seizures, and 30 (18.8%) had generalized epilepsy. Patient who stayed in the EMU>12 days required 6-10 days of VEEG recording to reach the diagnosis. Patients who had the surgical plan as a discharge diagnosis were significant to have a prolonged length of stay>12 days. Six to ten days of EEG recording were significant to diagnose focal seizures in 44 (60.3%). Patients were concluded to have a surgical plan (resective surgery), focal seizures and Attention Deficit Hyperactivity Disorder (ADHD) as a comorbidity are all significantly associated with a prolonged length of stay. Our findings suggest that factors affecting the length of hospital stay or period of EEG recording during the admission should be considered when planning EMU admissions.
带有视频监控的脑电图(VEEG)被认为是区分癫痫与非癫痫事件的金标准。由于癫痫发作的阵发性,在癫痫监测单位(EMU)设置的住院时间(LOS)的标准化具有挑战性。本研究以EMU的最终诊断和计划来评估影响视神经损伤的LOS和VEEG记录天数的因素。对2016年1月至12月在EMU三级中心住院的患者进行了回顾性研究。所有在研究期间连续入住EMU的患者均被纳入。共有160名患者入组。平均年龄21.10岁(SD 13.33)。出院时,大多数患者诊断为癫痫发作,其中局灶性癫痫91例(56.9%),全局性癫痫30例(18.8%)。在EMU住院12天的患者需要6-10天的VEEG记录才能确诊。将手术方案作为出院诊断的患者显著延长住院时间至12天。6 ~ 10天的脑电图记录对局灶性癫痫的诊断有重要意义,44例(60.3%)。结论患者有手术计划(切除手术),局灶性癫痫发作和注意缺陷多动障碍(ADHD)作为合并症都与住院时间延长显著相关。我们的研究结果表明,在计划急诊住院时应考虑影响住院时间或住院期间脑电图记录时间的因素。
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引用次数: 0
Physical Activity Assessment in Patients with Multiple Sclerosis 多发性硬化症患者的身体活动评估
Pub Date : 2021-01-01 DOI: 10.36648/2171-6625.21.12.358
Dorokhov Ad, Shkilnyuk Gg, Stolyarov Id, K. K. Mineev, Ivashkova Ev, Malikh Aa, TartakovskiyVN, Kryukova Ev, Tcvetkova Tl
Multiple sclerosis (MS) is on the second place after epilepsy in the young people fourth place among the neurological diseases of central nervous system. The main feature of the up-to-date MS therapy is the individual approach to the patient. Interest to the monitoring of the individual load in the daily activity is tremendously increased the last years. The purpose of this research was to demonstrate the possibility of the loading monitoring in the MS patients with a help of special measurement insoles and mobile application (both iOS and Android), allowing calculating not only the number of steps, but also the total loading, contact time, foot contact areas, imbalance, and cadence. Comparison of 2 patients with the same MS type and the same therapy was performed. Both patients are males, differ by age and body mass index, with no disability (minimal disability in 2 functional systems). Application of up-to-date measurement devices and mobile application allows to estimate the daily patient activity as well as the other parameters characterizing the gait pattern and its impairments. Functional diagnostics during barefoot platform measurements and influence of the shoes in in-shoe measurements give the opportunity to assume the limitation of physical activity in persons with MS.
多发性硬化症(MS)在青少年中枢神经系统疾病中排名第二,仅次于癫痫,排名第四。最新的多发性硬化症治疗的主要特点是个体化治疗。近年来,对日常活动中单个负载监测的兴趣急剧增加。本研究的目的是证明在特殊测量鞋垫和移动应用程序(包括iOS和Android)的帮助下,MS患者负荷监测的可能性,不仅可以计算步数,还可以计算总负荷、接触时间、足部接触面积、不平衡和节奏。对2例相同MS类型、相同治疗方法的患者进行比较。两例患者均为男性,年龄和体重指数不同,无残疾(2个功能系统有轻微残疾)。应用最新的测量设备和移动应用程序可以估计患者的日常活动以及表征步态模式及其损伤的其他参数。赤脚平台测量时的功能诊断和鞋内测量时鞋子的影响提供了假设多发性硬化症患者身体活动受限的机会。
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引用次数: 0
Cheiro-Oral syndrome due to Acute Lacunar Thalamic Infarct 急性腔隙性丘脑梗死引起的颅口综合征
Pub Date : 2021-01-01 DOI: 10.36648/2171-6625.21.12.386
Raghav Kapoor, Sumeet Roy, G. Nikita, R. Saini
A brain infarct is a tissue death due to inadequate blood supply to the affected area. While large or massive infarcts present with a very vague motor symptom, small thalamic infarcts can present with a variety of sensory deficits that can be difficult to diagnose clinically because of their seemingly disconnected manifestations. Cheiro-oral Syndrome is a pure sensory deficit which is confined to the perioral region and ipsilateral distal fingers. This very subtle clinical presentation might be missed in acute settings. We are reporting a case of a 46-year-old who was admitted with the complaints of peri-oral numbness on right side associated with weakness in right upper limb and lower limb.
脑梗死是由于受影响区域血液供应不足而导致的组织死亡。大面积或大面积的脑梗死表现为非常模糊的运动症状,而小的丘脑梗死可表现为各种感觉缺陷,这些感觉缺陷由于其看似不相关的表现而难以临床诊断。口口综合征是一种纯粹的感觉缺陷,局限于口周区域和同侧远端手指。这种非常微妙的临床表现可能在急性环境中被遗漏。我们报告一个46岁的病例谁是入院的投诉,口腔周围麻木的右侧,并在右上肢和下肢无力。
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引用次数: 0
A Study of How Hydrotherapy Affects the Brain and Its Effectiveness on Children with Varying Levels of Autism Spectrum Disorder 水疗法如何影响大脑及其对不同程度自闭症谱系障碍儿童有效性的研究
Pub Date : 2021-01-01 DOI: 10.36648/2171-6625.21.12.383
A. Manga
The goal of this research is to test which parts of the brain hydrotherapy triggers and the behaviours linked to each part of the brain. There has been extensive research on potential drugs that can benefit Autism, including Risperidone and Balovaptan, as well as music therapy. Hydrotherapy is a fairly novel therapy idea, and there has been some research conducted on the effectiveness of this therapy without accounting for the functionality of the child. Therefore, this research aims to determine which parts of the brain will receive blood flow, measured by fMRI, following hydrotherapy and which level of Autism Spectrum Disorder (Autistic Disorder, Asperger’s Syndrome and Pervasive Developmental Disorder) will experience the greatest benefits.
这项研究的目的是测试水疗法触发大脑的哪些部分以及与大脑各部分相关的行为。对可能对自闭症有益的药物进行了广泛的研究,包括利培酮和巴洛瓦坦,以及音乐疗法。水疗法是一种相当新颖的治疗理念,在没有考虑儿童功能的情况下,已经对这种疗法的有效性进行了一些研究。因此,这项研究的目的是确定大脑的哪些部分将接受血液流动,通过功能磁共振成像测量,在水疗之后,以及自闭症谱系障碍(自闭症,阿斯伯格综合症和广泛性发育障碍)的哪个级别将体验到最大的好处。
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引用次数: 1
期刊
Journal of neurology and neuroscience
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