特发性正常压力脑积水影像学特征

正常压力脑积水(normal pressure hydrocephalus,NPH)这一概念最早由Hakim于1964年提出,之后Adams和Hakim等又详细阐述了该疾病的特征性临床表现,即可以通过治疗而改善的"步态障碍、认知障碍、尿失禁"三联征[1].特发性正常压力脑积水(idiopathic normal pressure hydrocephalus,iNPH)是病因不明的正常颅压下脑室扩大,临床表现为步态异常和/或痴呆及括约肌功能障碍的疾病,大多发生于老年人.iNPH是极少数可以通过分流来改善痴呆症状的疾病之一,所以对于该疾病的正确诊断和有效治疗无论对于家庭还是社会都有着重要意义.然而目前临床上对于该病的诊断方法相对简单,准确性不高,且难以与其他的老年性疾病,如其他伴随脑室扩大的痴呆性疾病、颈椎或腰椎狭窄、外周神经性疾病、关节炎或前列腺肥大鉴别.幸运的是,伴随着近些年来影像学技术的迅猛发展,各国学者的对iNPH影像学特征研究得以不断深化,针对于该疾病影像学检查手段的阐述也层出不穷,并于2005年由Relkin等[2]通过循证医学方法制定出iNPH诊断标准,其目的就在于提高iNPH的诊断准确性并预测分流手术的效果.     

重视特发性正常压力性脑积水的规范化诊治

随着社会进步和医疗水平的不断提高,人类寿命也在不断地延长。随着老龄化社会的发展,老年人相关疾病如老年痴呆症、帕金森病和特发性正常压力性脑积水(idiopathic normal pressure hydrocephalus,iNPH)日益成为医学界亟待解决的现实问题[1-2]。典型iNPH表现为步态不稳、痴呆和尿失禁     

国际和日本特发性正常压力脑积水指南解读

特发性正常压力脑积水(idiopathic normal pressure hydrocephalus,iNPH)是以步态不稳、痴呆和尿失禁为典型表现的综合征,以老年人多见,伴随脑室扩大.患者的症状可通过分流手术得到改善,但是术前诊断并非容易且分流术后并发症也并非少见.     

国际和日本特发性正常压力脑积水指南解读

特发性正常压力脑积水(idiopathic normal pressure hydrocephalus,iNPH)是以步态不稳、痴呆和尿失禁为典型表现的综合征,以老年人多见,伴随脑室扩大.患者的症状可通过分流手术得到改善,但是术前诊断并非容易且分流术后并发症也并非少见.     

国际和日本特发性正常压力脑积水指南解读

特发性正常压力脑积水(idiopathic normal pressure hydrocephalus,iNPH)是以步态不稳、痴呆和尿失禁为典型表现的综合征,以老年人多见,伴随脑室扩大.患者的症状可通过分流手术得到改善,但是术前诊断并非容易且分流术后并发症也并非少见.     

特发性正常压力脑积水的诊断及治疗进展

正特发性正常压力脑积水(idiopathic normal pressure hydrocephalus,iNPH)是一种发病原因未知,以步态不稳、认知障碍和/或小便失禁为主要表现,脑脊液压力正常,影像学检查见脑室扩大的疾病,在老年人发生率较高,且伴随年龄增大患病率升高,有研究发现发病率在0.2%~5.9%~([1-3])。其病理生理机制不完全明确,给治疗带来了一定困难。本文期望通过对近年iNPH的相关研究进行综述,加深对其认识。     

成都地区老年期痴呆患病率调查

目的 调查成都地区城乡老年期痴呆和阿尔茨海默病(AD)的患病率.方法 采用分层随机整群抽样方法 ,将成都市19个区市县(总人口972万)分为城区、郊区、近郊和远郊,根据各层人口占总人口比例确定各层应查≥55岁的老人数(5 555人),实查5 353人.调查采用筛查和确诊两阶段法,按美国精神障碍诊断与统计手册第3版修订本的标准诊断痴呆.结果 (1)查出痴呆患者143例,患病率为2.67%.其中AD为2.05%,血管性痴呆(VD)为0.37%,其他痴呆为0.24%.(2)女性患病率(3.14%)高于男性(2.16%;P<0.05).(3)痴呆患者的平均年龄[男(80±9)岁,女(82±7)岁]高于正常老年人[男女均为(67±8)岁;P<0.001],且痴呆患病率随年龄增长而上升.(4)农业人口的患病率(2.79%)高于非农业人口(2.40%),但差异无显著性.(5)文盲老人的患病率(4.45%)较小学(1.48%)和初中及其以上(0.17%)文化程度者高(P<0.001).结论 AD是成都地区老人中主要的痴呆类型,VD次之.文盲老人的痴呆患病率高,老年期痴呆患病率随年龄的增长而升高.     

早期帕金森病诊断的研究进展

帕金森病(PD)是常见的年龄相关性的神经退行性疾病,60岁以上人群的患病率为1％ ～2％,随着人口老龄化,其发病率逐渐升高[1].预计2030年PD的患病数会增加两倍多,全世界将达到900万[2].PD是一种致残性疾病,给患者和家庭带来极大的痛苦.尽管目前尚不能治愈PD,但左旋多巴治疗可以改善症状,减轻残疾.研究[3]证实,PD早期神经保护治疗可以延缓病情发展,延后左旋多巴的应用,对整个病程是有益的.对于早期轻症患者,推荐单胺氧化酶( MAO-B)抑制剂和多巴胺受体激动剂治疗,以延缓左旋多巴的应用[1,3].第三方付费的研究[4]发现,早期神经保护治疗可以达到一个较好的成本-效益比.然而,神经保护治疗的效果取决于其应用的时间;另外,PD早期的运动症状或非运动症状与其他运动异常性疾病,如多系统萎缩(MSA)、进行性核上性麻痹(PSP)等不易鉴别.因此,PD的早期治疗依赖于及时、准确的诊断.     

特发性正常压力脑积水患者的认知功能损害

目的 通过系列神经心理学测试分析特发性正常压力脑积水(iNPH)患者认知功能损害的特点及各种测试方法对诊断的作用.方法 运用神经心理学测试方法对18例iNPH患者、20例轻度阿尔茨海默病(AD)患者和20名健康对照者进行记忆力、注意力、执行功能、视空间能力评估,同时用受试者工作特征曲线分析这些测试方法对iNPH诊断的敏感度和特异度.结果 与健康对照组比较,iNPH组和AD组的总体认知状态和各认知领域均有受损,差异均有统计学意义.iNPH组与AD组相比较[数值以中位数(四分位数间距)表示],在额叶功能评定量表评分[9.0(2.8)分与13.0(3.5)分]、言语流畅性测验评分[10.0(8.5)分与12.0(8.0)分]、斯特鲁普色词测验卡片A耗时数[65.0(20.8)s与52.0(24.5)s]、斯特鲁普色词测验卡片B耗时数[157.5(44.3)s与108.0(37.3)s]、斯特鲁普干扰量耗时数[68.5(67.0)s与50.0(25.8)s]、积木测试评分[5.0(3.3)分与6.5(3.5)分]方面差异均有统计学意义(Z =4.047、2.025、2.857、3.864、3.218、2.221,均P＜0.05),而AD评估量表中单词回忆和单词辨认、数字广度测验、画钟测验评分差异均无统计学意义.其中,斯特鲁普色词测验卡片A耗时数、斯特鲁普色词测验卡片B耗时数、斯特鲁普干扰量耗时数对iNPH诊断的受试者工作特征曲线下面积分别为0.874、0.929、0.869,敏感度和特异度分别为88.9％和75.0％,88.9％和85.0％,94.4％和72.5％.结论 iNPH患者亦有记忆力、执行功能、注意力、视空间能力损害,其中以执行功能障碍最为突出.另外,斯特鲁普色词测验对iNPH诊断具有很好的识别能力.     

帕金森病与microRNA研究

帕金森病(Parkinson's disease,PD)又称为震颤麻痹,是一种与年龄相关的常见神经系统退行性疾病[1],由环境和遗传因素引起,65岁以上人群患病率为1％～2％[2-3].PD 临床表现主要为局限于身体一侧的肢体静止性震颤,伴随症状包括运动迟缓、肌强直及姿势步态不稳[4].大部分PD患者为散发性,仅不足10％的患者呈家族性.PD的诊断特征性标志物是存活的多巴胺神经元中出现嗜酸性蛋白质包涵体,又称路易小体[4].目前已发现18个基因位点和11个致病基因与PD有关,但这些基因如何参与PD发病尚不完全清楚[5-6].阐明这些基因突变所导致的神经细胞退行性变的机制,将对延缓PD发病和治疗起着重要的作用[2].PD症状的出现是由于大脑黑质纹状体致密部多巴胺能神经元(dopaminergic neuron,DN)不断丢失,导致多巴胺含量的显著下降.当病变累及脑内其他区域时,很大一部分患者出现痴呆和幻觉,有研究表明microRNA(miRNA)与DN分化及生存可能相关[2,4].新近研究显示,miRNA广泛参与疾病的发生过程,对基因表达转录后调节和神经细胞表型调控起重要作用,目前已发现数个miRNA的分布和功能与PD存在相关性,且部分已在PD动物模型中得到证实[7].我们就miRNA与PD研究新进展作一概述.     

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.     

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.     

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.     

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.     

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.     

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.