MRS在神经外科脑干疾病中的应用

磁共振波谱（MRS）是利用MRI的原理与方法研究组织代谢,在分子水平反映组织代谢情况的成像方法,目前在神经外科得到广泛运用。脑干疾病是神经外科中诊断及治疗比较困难的一类疾病。本文对MRS在脑干疾病诊断中的研究及其进展进行综述。     

Creutzfeldt-Jakob病非侵入性诊断方法研究新进展

朊病毒是一种非常规意义上的传染性病原体,常导致致命性神经系统疾病,其中以Creutzfeldt-Jakob病(CJD)最为常见。CJD属于一种罕见的神经系统退行性疾病,临床进展迅速,致死性达100%。此疾病有多种类型,其中以散发型最为常见。目前此病的最大挑战即临床诊断。到目前为止临床确诊的金标准仍为脑组织活检,但将其作为诊断金标准仍存在一定局限性。随着研究的逐步进展,亟需探讨新的非侵入性的检测(手段),以利于疾病诊断。本文就有关CJD生物标记物检测、基因筛查、影像学检查及电生理检查等非侵入性手段的研究进展进行综述,旨在协助CJD的诊断,为CJD的确诊提供新的思路。     

磁共振动脉自旋标记灌注成像技术在脑部疾病中的应用进展

<正>灌注成像有助于了解组织局部血流动力学及功能方面的变化,能够从病理和生理学角度研究疾病的发病机制及用于疗效评估。动脉自旋标记(arterial spin labeling,ASL)技术作为一种新兴的磁共振(MRI)灌注成像方法,凭借其非侵袭性、无电离辐射及可重复性好等诸多优势,近年来得到了较快速发展,尤其是在脑部疾病诊断中的应用价值已经逐渐得到临床认可。现就ASL技术在脑部疾病诊断中的应用进展进行综述。     

4D-CTA在脑血管疾病诊断中的应用进展

正4D-CTA (4-dimensions-computed tomography angiography)是近年来出现的一种脑血管疾病影像学检查技术,由于其在脑血管疾病诊断领域的突出优势,逐渐成为数字减影血管造影(digital subtraction angiography, DSA)的非侵入性替代方案,越来越多地用于临床实践。现将其在脑血管疾病诊断中的应用进展综述如下。1概述     

抑郁症生物标志物研究进展

抑郁症作为情感性障碍一种临床类型,是以显著而持久的情绪低落为主要特征的精神障碍。其主要表现是以情感低落、思维迟缓和精神运动性抑制三大症状为基本特征。部分抑郁症患者伴有严重的焦虑症状,出现自杀观念及行为,目前抑郁症已经成为精神科自杀率最高的疾病。抑郁症的病因十分复杂,迄今仍未完全阐明。生物标志物是指可以标记系统、器官、组织、细胞及亚细胞结构或功能的改变或可能发生改变的生化指标。生物标志物可用于疾病诊断、判断疾病分期或者用来评价新药物或新疗法在目标人群中的安全性及有效性。本文主要针对抑郁症与几种生物标志物之间的相关性研究作一综述。     

外泌体微小RNA在中枢神经系统疾病诊断中作用的研究进展

正中枢神经系统(central nervous system,CNS)疾病是指发生于脑和脊髓的疾病,以感觉、运动、意识、植物神经功能障碍为主要表现。本文就外泌体微小RNA(microRNA,miRNA)在CNS疾病诊断中的作用进行综述,为CNS疾病诊断提供新的思路和方法。1外泌体简介1.1外泌体的一般生物特性及发现过程外泌体是一种由细胞分泌的、大小30～150 nm膜状囊泡,由多泡体与细胞膜融合后释放。在电子显微镜下,外泌体通常表现出杯状或碟状的形态[1]。其外膜与细胞膜相似,含磷脂双分子层,其内含有蛋白质、脂类、     

神经丝蛋白轻链和磷酸化的神经丝蛋白重链作为肌萎缩侧索硬化生物标志物的研究进展

肌萎缩侧索硬化(ALS)是一种累及上、下运动神经元的致死性神经变性疾病。从发病至诊断普遍延迟1年左右,这与其发病机制不明、异质性高、缺乏特异性生物标志物相关。神经丝蛋白轻链(NFL)和磷酸化的神经丝蛋白重链(p-NFH)是神经元特异性细胞骨架蛋白,其在CSF和血液中的水平会随着神经轴突的损伤而升高,是一些神经系统疾病潜在的生物标志物。众多研究表明,ALS患者的CSF和血液NFL和p-NFH水平较健康者及其他神经系统疾病显著升高,具有高度的诊断敏感性和特异性。同时,NFs与其他生物标志物的联合检测有助于提高诊断的准确性。不仅如此,它们还与ALS的疾病进展和预后密切相关。NFL和p-NFH是目前ALS最有前景的生物标志物。本文主要从CSF和血液NFL和p-NFH在ALS诊断、监测疾病进展和判断预后等方面的应用进行综述。     

脑脊液生物学标志物在朊蛋白疾病中的应用研究进展

朊蛋白疾病是一组致死性的神经变性病,但目前在早期诊断、监测患者的病情变化等方面尚缺乏明确特异性的生物学标志物。发现可靠的生物学标志物对朊蛋白疾病的早期诊断和预防具有重要意义,与之相关的研究在国内外已取得了长足进步。本文综述了近年来1 4-3-3蛋白、β淀粉样蛋白、Tau蛋白、S1 0 0蛋白以及神经元特异性烯醇化酶(NSE)、脑型肌酸激酶和脂肪酸结合蛋白等生物学标志物在疾病诊断、临床表现及病情进展等相关方面的研究进展。     

中华医学会放射学分会2013年头颈部影像学术年会暨头颈部影像解剖提高班征文通知

由中华医学会放射学分会头颈学组和《中华放射学杂志》编辑委员会主办的中华医学会放射学分会2013年头颈部影像学术年会暨头颈部影像解剖提高班拟定于2013年3月8-10日在北京召开。届时将邀请国内著名专家进行专题讲座,内容涉及头颈部影像诊断进展、新技术的临床应用等,重点讲解和讨论头颈部疾病临床诊断对影像学的需求、临床和影像学进展、存在的问题和未来发展方向;同时举行的头颈部影像解剖提高班详细讲解头颈部影像解剖与解剖变异以及在疾病诊断和治疗中的     

端粒-端粒酶在神经退行性疾病中的研究进展

神经退行性疾病多以β-淀粉样蛋白（β-amyloid,Aβ）、α-突触核蛋白（α-synuclein,α-syn）作为生物标记物来辅助临床进行诊断。近年来,人们发现染色体末端的端粒能够作为衡量生物衰老程度的指标,并发现端粒长度、端粒酶活性可能作为评估老年神经退行性疾病发病风险、疾病进展、不良预后的血液标记物,但目前相关国内外研究结果并不具有一致性。了解端粒相关生物标记物在年龄相关疾病中的作用,是可以帮助临床医生更好地了解疾病的发生发展机制。现就端粒-端粒酶系统在神经退行性疾病中的最新研究进展综述如下,以介绍端粒长度、端粒酶活性对神经退行性疾病的影响以及潜在作用机制。     

Epilepsy and anomalies of neuronal migration: MRI and clinical aspects

Neuronal migration disorders are the result of disturbed brain development. In such disorders, neurons are abnormally located. In diagnosing these conditions, magnetic resonance imaging is superior to any other imaging technique. This enables us to improve our knowledge of the clinical correlates of neuronal migration. With reference to migrational disorder, a retrospective study of all 303 patients with epileptic seizures referred for magnetic resonance imaging during a 3-year period was performed, 13 patients (aged 12-41, mean age 27) were identified. They represent 4.3% of the entire study group. Of the patients with known epilepsy, 6.7% and of the mentally retarded, 13.7% had migrational disorders. Four patients had schizencephaly as the dominant finding, one was classified as hemimegalencephaly, 2 had isolated heterotopias, and 6 had localized pachy- and/or poly-microgyria. The clinical pictures are complex. Ectopias of grey matter are recognised foci of epilepsy, but from an epileptological and a clinical viewpoint little attention has been given to these disorders. The present study shows that malmigration is not rare in epilepsy patients, especially not in the mentally retarded.     

Hepatic Considerations in the Use of Antiepileptic Drugs

Summary: Virtually all of the major antiepileptic drugs (AEDs) can cause hepatotoxicity, although fatal hepatic reactions are rare. The mechanisms, incidences, and risk profiles for such reactions differ from drug to drug. With carbamazepine and phenytoin, hepatotoxicity may be due to drug hypersensitivity. Although the profiles of patients at risk have not been well-defined for these two antiepileptic drugs, it would appear from reports in the literature that older adolescents and adults are at higher risk than children of developing serious or fatal hepatotoxicity. Once hepatotoxicity develops, mortality rates are 10–38% with phenytoin and 25% for carbamazepine. The risk profile for valproate fatal hepatotoxicity has been more clearly defined. Those at primary risk of fatal hepatic dysfunction are children under the age of 2 years who are receiving multiple anticonvulsants and also have significant medical problems in addition to severe epilepsy. The risk is considerably lower for patients over the age of 2 years on valproate monotherapy. In contrast to the risk profile with other AEDs, adults receiving valproate as monotherapy have the lowest risk of hepatotoxicity. Fatal hepatic dysfunction coincident with valproate may be the result of aberrant drug metabolism. Concomitant use of AEDs that induce microsomal P450 enzymes (e.g., phenytoin and phenobarbital) may enhance the production of a toxic metabolite, and hence the greater risk of hepatotoxicity with polypharmacy.     

Vascular Malformations and Epilepsy: Clinical Considerations and Basic Mechanisms

Summary: Vascular malformations (VMs) are associated with epilepsy. The natural history of the various VMs, clinical presentation, and tendency to provoke epilepsy determine treatment strategies. Investigations have probed the mechanisms of epileptogenesis associated with these lesions. Electrophysiologic changes are associated with epileptogenic cortex adjacent to VMs. Putative pathophysiologic mechanisms of epileptogenesis include neuronal cell loss, glial proliferation and abnormal glial physiology, altered neurotransmitter levels, free radical formation, and aberrant second messenger physiology.     

Classification of methods in transcranial Electrical Stimulation (tES) and evolving strategy from historical approaches to contemporary innovations

Transcranial Electrical Stimulation (tES) encompasses all methods of non-invasive current application to the brain used in research and clinical practice. We present the first comprehensive and technical review, explaining the evolution of tES in both terminology and dosage over the past 100 years of research to present day. Current transcranial Pulsed Current Stimulation (tPCS) approaches such as Cranial Electrotherapy Stimulation (CES) descended from Electrosleep (ES) through Cranial Electro-stimulation Therapy (CET), Transcerebral Electrotherapy (TCET), and NeuroElectric Therapy (NET) while others like Transcutaneous Cranial Electrical Stimulation (TCES) descended from Electroanesthesia (EA) through Limoge, and Interferential Stimulation. Prior to a contemporary resurgence in interest, variations of transcranial Direct Current Stimulation were explored intermittently, including Polarizing current, Galvanic Vestibular Stimulation (GVS), and Transcranial Micropolarization. The development of these approaches alongside Electroconvulsive Therapy (ECT) and pharmacological developments are considered. Both the roots and unique features of contemporary approaches such as transcranial Alternating Current Stimulation (tACS) and transcranial Random Noise Stimulation (tRNS) are discussed. Trends and incremental developments in electrode montage and waveform spanning decades are presented leading to the present day. Commercial devices, seminal conferences, and regulatory decisions are noted. We conclude with six rules on how increasing medical and technological sophistication may now be leveraged for broader success and adoption of tES.     

Carbamazepine Efficacy and Utilization in Children

Summary: Carbamazepine is effective for preventing partial and generalized tonic-clonic seizures in children. Although absence epilepsies are more common in children than adults, an estimated 80% of children with epilepsy have seizure types or epilepsies that are potentially responsive to carbamazepine. The differential diagnosis of ictal staring is an especially important issue in children because absence and atypical absence seizures are more prevalent in children than adults. Age-related pharmacokinetic differences and drug interactions are major considerations in children. On average, children have higher clearance rates of carbamazepine, shorter half-lives, and higher ratios of carbamazepine-10, 11-epoxide to carbamazepine than adults. In addition, children with severe epilepsy are more likely to require multiple-drug therapy, which can lead to complex drug interactions. When carbamazepine is administered along with valproate, drug protein binding interactions can cause intermittent side effects.     

Neonatal Seizures: Problems in Diagnosis and Classification

Summary: The clinical identification of neonatal seizures is critical for the recognition of brain dysfunction; however, diagnosis is often difficult because of the poorly organized and varied nature of these behaviors. Current classification systems are limited in their ability to communicate motor, autonomic, and electroencephalo-graphic features of seizures precisely and to provide a basis for uniform effective diagnosis, therapy, and determination of prognosis. Recent investigations of neonates, utilizing bedside electroencephalographic/polygraphic/ video monitoring techniques, have provided the basis for improved diagnosis and classification of seizures in the newborn. These studies have demonstrated that not all clinical phenomena currently considered to be seizures require electrocortical epileptiform activity for their initiation or elaboration. In addition, the specific clinical character of the phenomena considered to be seizures, the clinical state of the infant, and the character of the EEG indicate the probable pathophysiological mechanisms involved and suggest probable etiologies, prognosis, and therapy. Similarities between animal models that demonstrate reflex physiology and neonates with motor automatisms and tonic posturing suggest that these clinical behaviors may not be epileptic in origin but, rather, primitive movements of progression and posture mediated by brainstem mechanisms. Although not all clinical behaviors currently considered to be neonatal seizures may have similar pathophysiological mechanisms, they are clinically significant because they all indicate brain dysfunction.     

Valproate Monotherapy in the Management of Generalized and Partial Seizures

Summary: For decades, therapeutic tradition has promoted the concept of polypharmacy in the management of epilepsy. In recent years, however, studies have shown that, for most patients, monotherapy can provide comparable or better seizure control than administration of multiple anticonvulsants, while diminishing the potential for adverse reactions, drug interactions, and poor compliance. Valproate is an important monotherapeutic agent that is highly effective in the control of idiopathic primary and secondarily generalized epilepsies, and partial seizures that do not generalize. Comparative studies have found that valproate is at least as effective as phenytoin and carbamazepine in the treatment of generalized and partial seizures. Given the similar efficacy, other factors such as pharmacokinetics and side effects may therefore determine anticonvulsant selection for monotherapy.     

Un nouveau cadre conceptuel de travail pour la psychiatrie

In an attempt to place psychiatric thinking and the training of future psychiatrists more centrally into the context of modern biology, the author outlines the beginnings of a new intellectual framework for psychiatry that derives from current biological thinking about the relationship of mind to brain. The purpose of this framework is twofold. First, it is designed to emphasize that the professional requirements for future psychiatrists will demand a greater knowledge of the structure and functioning of the brain than is currently available in most training programs. Second, it is designed to illustrate that the unique domain which psychiatry occupies within academic medicine, the analysis of the interaction between social and biological determinants of behavior, can best be studied by also having a full understanding of the biological components of behavior.