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Medulloblastoma 成神经管细胞瘤
Pub Date : 2019-09-01 DOI: 10.1093/med/9780198746706.003.0028
J. Rutka
Under the term embryonal tumours, we examine the malignant neoplasms medulloblastomas, CNS-PNETs, AT/RTs, and ETANTRs. The common histological link between them is the small, round, blue cells which appear on H&E stains. They are considered highly malignant tumours (WHO Grade IV), although significant variation of prognosis exists between the different subtypes. Current classification is based on their histopathological features. Advances on understanding of the molecular biology and behaviour of their cellular lines, has dramatically changed their stratification and scientific approach. Combination of surgical treatment with new schemes of adjuvant chemo- and radiotherapy offers improved survival rates but comes with a high cost on neurocognitive, endocrine, and overall functional status. New therapies with molecular targets will hopefully improve outcomes with minimal side effects and collateral damage.
在胚胎肿瘤这一术语下,我们研究了髓母细胞瘤、CNS-PNETs、AT/RTs和ETANTRs等恶性肿瘤。它们之间的共同组织学联系是H&E染色上出现的小而圆的蓝色细胞。它们被认为是高度恶性肿瘤(世卫组织四级),尽管不同亚型之间的预后存在显著差异。目前的分类是基于他们的组织病理学特征。对其细胞系的分子生物学和行为的理解的进步,极大地改变了它们的分层和科学方法。手术治疗与新的辅助化疗和放疗方案相结合可提高生存率,但在神经认知、内分泌和整体功能状态方面代价高昂。以分子为靶点的新疗法有望以最小的副作用和附带损害改善治疗效果。
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引用次数: 0
Extracranial-intracranial bypass for cerebral ischaemia 脑缺血的颅外-颅内旁路治疗
Pub Date : 2019-09-01 DOI: 10.1093/med/9780198746706.003.0052
M. Guilfoyle, P. Kirkpatrick
Symptomatic stenosis or occlusion of the cervical (C1) internal carotid artery (ICA) is best addressed surgically with direct endarterectomy or thrombectomy procedures. This strategy is not usually possible for more distal lesions, and restoring brain perfusion requires diversion of blood from the extracranial to intracranial circulation with a variety of indirect and direct bypass techniques. However, despite five decades of experience and technical refinement, extracranial-intracranial (EC-IC) bypass for cerebral ischaemia remains contentious. This chapter looks at the conflicting bodies of evidence and opinion regarding how best to select patients for surgery, and asks whether there is meaningful clinical benefit with the surgical options available.
有症状的颈(C1)内颈动脉(ICA)狭窄或闭塞最好通过直接动脉内膜切除术或血栓切除术进行手术治疗。这种策略通常不可能用于更远的病变,恢复脑灌注需要通过各种间接和直接旁路技术将血液从颅外循环转移到颅内循环。然而,尽管有50年的经验和技术的改进,颅外-颅内(EC-IC)旁路治疗脑缺血仍然存在争议。本章着眼于关于如何最好地选择手术患者的证据和意见的冲突体,并询问是否有有意义的临床效益与可用的手术选择。
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引用次数: 0
Neurophakomatoses
Pub Date : 2019-09-01 DOI: 10.1093/med/9780198746706.003.0038
F. Greenway, F. Elmslie, Timothy Jones
The term phakomatosis, from the Greek root phakos, meaning lens or spot, was first used in 1920 to describe a group of multisystem disorders with common neuro-oculocutaneous findings. Each condition is caused by distinct genetic defects, with some overlap in terms of protein function. They share pathogenetic mechanisms in dysfunction of a tumour suppressor gene. This chapter details four of the phakomatoses seen in practice—neurofibromatosis types 1 and 2, tuberous sclerosis complex, and von Hippel-Lindau—paying attention to their neurological sequelae and impact on neurosurgical practice. For each condition, the epidemiology, genetics, diagnostic criteria, screening, clinical features, management, and controversy will be reviewed, with an accompanying illustrative case.
“白斑病”一词源于希腊语词根phakos,意为晶状体或斑点,于1920年首次用于描述一组具有常见神经-眼部病变的多系统疾病。每种情况都是由不同的遗传缺陷引起的,在蛋白质功能方面有一些重叠。它们在肿瘤抑制基因功能障碍中具有共同的发病机制。本章详细介绍了实践中常见的四种吞噬病- 1型和2型神经纤维瘤病,结节性硬化症和von hippel - lindau -并关注其神经系统后遗症及其对神经外科实践的影响。对于每种情况,流行病学,遗传学,诊断标准,筛选,临床特征,管理和争议将进行审查,并附有一个说明性病例。
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引用次数: 0
Haemangioblastoma 血管母细胞瘤
Pub Date : 2019-09-01 DOI: 10.1093/med/9780198746706.003.0030
Donald McArthur, A. Natalwala
Haemangioblastomas are rare and benign tumours which favour the infratentorial structures in the central nervous system. Around 30% of these tumours are associated with Von Hippel-Lindau syndrome, which if the diagnosis is missed can lead to rapid disease progression and significant morbidity and mortality. Complete surgical resection is the main treatment but other techniques including stereotactic radiosurgery are described in this chapter. Screening for Von Hippel-Lindau (VHL), the optimal timing, and mode of treatment remain controversial due to the diversity of clinical presentation in these patients. This chapter also outlines the general principles of managing patients with haemangioblastomas and the factors which impact on prognosis.
血管母细胞瘤是一种少见的良性肿瘤,主要发生在中枢神经系统的幕下结构。这些肿瘤中约有30%与Von Hippel-Lindau综合征相关,如果误诊,可导致疾病快速进展和显著的发病率和死亡率。完全手术切除是主要的治疗方法,但其他技术包括立体定向放射手术在本章中进行了描述。由于这些患者临床表现的多样性,Von Hippel-Lindau (VHL)的筛查、最佳时机和治疗方式仍然存在争议。本章还概述了管理血管母细胞瘤患者的一般原则和影响预后的因素。
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引用次数: 0
Medical pathologies of the spinal cord 脊髓的医学病理学
Pub Date : 2019-09-01 DOI: 10.1093/med/9780198746706.003.0059
C. McGuigan, K. O'Connell, E. McGovern, I. McGurgan
Spinal cord presentations commonly seen by neurosurgeons suggestive of surgical pathology can be mimicked by many medical conditions. The most common presentations of medical, neurological conditions seen by neurosurgeons include conditions causing acute spastic paraparesis, such as transverse myelitis, and acute flaccid paralysis, such as Guillain–Barré syndrome. Other medical conditions such as motor neuron disease, neuropathy pain syndromes can also mimic radiculopathies and may be referred for a neurosurgical outpatient review. Therefore, a knowledge of these conditions and recognition of some of the key questions to ask during a history is beneficial. Key findings to look for on examination will also help with a timely diagnosis and involvement of the relevant specialist without unnecessary investigations or interventions.
神经外科医生常见的提示外科病理的脊髓表现可以被许多医学条件所模仿。神经外科医生看到的最常见的内科和神经系统疾病包括引起急性痉挛性截瘫的疾病,如横贯脊髓炎,和急性弛缓性麻痹,如格林-巴罗综合征。其他医疗条件,如运动神经元疾病,神经病变疼痛综合征也可以模仿神经根病,并可能被转介到神经外科门诊复查。因此,了解这些条件并认识到在历史中要问的一些关键问题是有益的。在检查中寻找的关键发现也将有助于及时诊断和相关专家的参与,而无需进行不必要的调查或干预。
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引用次数: 0
The pathophysiology of subarachnoid haemorrhage 蛛网膜下腔出血的病理生理学
Pub Date : 2019-09-01 DOI: 10.1093/med/9780198746706.003.0048
J. McMillen
Aneurysm rupture with subarachnoid haemorrhage is the most devastating complication of intracerebral aneurysms. Grading the severity of the haemorrhage assists with prognostication and assists with decision-making for patient management. Both radiological and clinical grading systems can be predictive of outcomes following subarachnoid haemorrhage. The pathophysiology of, and risk factors for, intracerebral aneurysm formation, growth, and rupture are complicated and poorly understood. In order to make an informed judgement about which patients to treat, it is important to balance the natural history of the condition against each patient’s risks of treatment. Numerous risk factors for aneurysm rupture have been identified, and this quantification of risk continues to be refined. Cerebral vasospasm is a common complication after subarachnoid haemorrhage. The pathological mechanisms of vasospasm involve numerous changes acting in concert to result in ischaemic neurological deficits. Clinical and radiological monitoring allows early detection and treatment to prevent ischaemic neurological deficits.
动脉瘤破裂并蛛网膜下腔出血是脑内动脉瘤最具破坏性的并发症。对出血的严重程度进行分级有助于患者的预后和管理决策。放射学和临床分级系统都可以预测蛛网膜下腔出血后的预后。脑内动脉瘤形成、生长和破裂的病理生理学和危险因素是复杂的,而且人们对其知之甚少。为了对治疗哪些患者做出明智的判断,重要的是要平衡病情的自然史和每个患者的治疗风险。许多动脉瘤破裂的危险因素已经被确定,并且这种风险的量化还在继续完善。脑血管痉挛是蛛网膜下腔出血后常见的并发症。血管痉挛的病理机制包括许多变化,这些变化共同导致缺血性神经功能障碍。临床和放射学监测允许早期发现和治疗,以防止缺血性神经功能缺损。
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引用次数: 0
Classification of seizures and epilepsy 癫痫发作和癫痫的分类
Pub Date : 2019-09-01 DOI: 10.1093/med/9780198746706.003.0081
A. McEvoy, Tim Wehner, V. Wykes
Epileptic seizures are transient neurologic alterations due to abnormal excessive or synchronous neuronal cerebral activity. They may cause subjective symptoms (aura), and objective autonomic, behavioural, or cognitive alterations in any combination. Focal seizures are initially generated in one circumscribed area in the brain, whereas generalized seizures involve bihemispheric neuronal networks from the seizure onset. Epilepsy is a brain disease defined by the occurrence of two unprovoked seizures more than 24 h apart or one unprovoked seizure with underlying pathological or genetic factors resulting in a similar recurrence risk. Focal epilepsy syndromes are best classified by aetiology or anatomical area of origin. A seizure that does not self-terminate results in status epilepticus, and constitutes a medical emergency that requires immediate treatment. Focal cortical dysplasia and hippocampal sclerosis are the commonest aetiologies of epilepsy amenable to surgical treatment and are reviewed here. The limbic pathway may be involved in seizure propagation, and the anatomy is described.
癫痫发作是由于异常过度或同步的神经活动引起的一过性神经系统改变。它们可引起主观症状(先兆)和客观的自主神经、行为或认知改变。局灶性癫痫发作最初发生在大脑的一个限定区域,而全面性癫痫发作从发作开始就涉及双半球神经元网络。癫痫是一种脑部疾病,定义为两次间隔超过24小时的非诱发性癫痫发作或一次非诱发性癫痫发作伴有潜在的病理或遗传因素,导致类似的复发风险。局灶性癫痫综合征最好根据病因或起源解剖区域分类。没有自我终止的癫痫发作会导致癫痫持续状态,并构成需要立即治疗的医疗紧急情况。局灶性皮质发育不良和海马硬化是最常见的癫痫病因,可以手术治疗,在此进行综述。边缘通路可能参与癫痫的传播,解剖描述。
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引用次数: 1
Surgical management of head injury 颅脑损伤的外科治疗
Pub Date : 2019-09-01 DOI: 10.1093/med/9780198746706.003.0043
Hadie Adams, A. Kolias, A. Helmy, P. Hutchinson, R. Chesnut
Traumatic brain injury (TBI) is a significant cause of preventable morbidity and mortality in the United Kingdom and across the world, leading to disability and loss of productivity. The estimated incidence of TBI in Europe is on average 235 per 100 000, ranging of 150–300/100 000 per year. The Glasgow Coma Scale (GCS) is often used to classify the severity of TBI, with patients scoring 8 or less being classified as severe, 9–12 as moderate, and scores of 13–15 as mild. Mild TBI is the most common form of head injury, and the incidence of TBI is the highest in young adult males. In Western countries the major causes of TBI-related hospitalizations are falls, assaults, and motor vehicle traffic collisions.
在英国和世界各地,创伤性脑损伤(TBI)是可预防的发病率和死亡率的重要原因,导致残疾和生产力丧失。据估计,欧洲脑外伤的发病率平均为每10万人235例,范围为每年每10万人150 - 300例。格拉斯哥昏迷评分(GCS)常用于对TBI的严重程度进行分类,8分及以下为重度,9-12分为中度,13-15分为轻度。轻度脑损伤是最常见的头部损伤形式,在年轻成年男性中发病率最高。在西方国家,与脑外伤有关的住院治疗的主要原因是跌倒、袭击和机动车交通碰撞。
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引用次数: 0
Concussion and sports-related head injury 脑震荡和运动相关的头部损伤
Pub Date : 2019-09-01 DOI: 10.1093/med/9780198746706.003.0045
Mark Wilson
Interest in concussion and sports-related injury has intensified in recent years for three main reasons: (1) it is a preventable form of brain injury; (2) there is increasing evidence that repeated injury can result in long-term neurocognitive loss; and (3) as a result there are potential medicolegal costs to organizations that, possibly inadvertently, allow this form of brain injury to occur within their sport. The long-term effects of boxing resulting in dementia pugilistica have been appreciated for some time, however the results of repeated mild head injury in other sports is now under focus. Concussion, increasingly termed mild traumatic brain injury, should be graded. Imaging, removal from, and return to sport are all discussed in this chapter.
近年来,人们对脑震荡和运动相关损伤的兴趣日益浓厚,主要有三个原因:(1)它是一种可预防的脑损伤;(2)越来越多的证据表明,重复损伤可导致长期的神经认知丧失;(3)因此,组织可能会在无意中允许这种形式的脑损伤发生在他们的运动中,从而产生潜在的医疗法律成本。一段时间以来,拳击运动对拳击运动员痴呆的长期影响一直受到重视,然而,其他运动中反复出现的轻度头部损伤的结果现在受到关注。脑震荡,越来越多地被称为轻度创伤性脑损伤,应该分级。本章将讨论成像、移除和回归运动。
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引用次数: 0
Managing spinal cord trauma 处理脊髓损伤
Pub Date : 2019-09-01 DOI: 10.1093/med/9780198746706.003.0067
S. Smithason, Bryan S. Lee, E. Benzel
Spinal cord injury (SCI), either traumatic or non-traumatic in aetiology, leads to temporary or permanent damage to the spinal cord function. Significant efforts have been directed towards the elucidation of the underlying pathophysiology of SCI. Both primary and secondary mechanisms of SCI exist, leading to immediate and often irreversible cell damage. Immediate treatment and adequate management in the setting of acute SCI are essential, preservation of even a small amount of functional neuronal tissue can permit ambulation. SCI is associated with a prolonged hospital stay, rehabilitation stay, and often associated with enormous monetary costs. Neurological recovery after SCI is largely dependent on the extent of injury. The management of SCI and the determination of the role and timing of surgical decompression remain crucial and yet controversial. Further epidemiological research and studies are warranted in order to enhance healthcare planning and cost-effectiveness.
脊髓损伤(SCI)的病因可分为创伤性损伤和非创伤性损伤,可导致脊髓功能的暂时性或永久性损伤。在阐明脊髓损伤的潜在病理生理学方面,人们已经付出了巨大的努力。脊髓损伤存在原发性和继发性机制,可导致立即且往往不可逆的细胞损伤。在急性脊髓损伤的情况下,及时治疗和适当的管理是必不可少的,即使保存少量的功能神经元组织也可以允许行走。脊髓损伤与延长住院时间、康复时间有关,并且通常与巨大的金钱费用有关。脊髓损伤后神经系统的恢复在很大程度上取决于损伤的程度。脊髓损伤的治疗以及手术减压的作用和时机的确定仍然是至关重要的,但也存在争议。有必要进行进一步的流行病学研究,以加强保健规划和成本效益。
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引用次数: 0
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Oxford Textbook of Neurological Surgery
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