468例儿童中枢神经系统肿瘤临床分析

目的 探讨儿童中枢神经系统肿瘤的发病特点、临床表现、病理学特点、治疗效果及其相互关系.方法 1999年1月至2009年1月期间收治的并获得组织病理学诊断的儿童(≤16岁)中枢神经系统肿瘤468例,按照WHO 2007年中枢神经系统肿瘤分类,进行回顾性分析.结果 颅内和椎管内肿瘤分别为431例(92.1％)和37例(7.9％),幕上288例(67.0％),幕下143例(33.0％).男女发病比为1.67∶1,各年龄段均有发病,随年龄的增长肿瘤发病率呈增高趋势.颅内最常见的五种肿瘤类型依次是星形细胞瘤(26.5％)、颅咽管瘤(15.5％)、生殖细胞肿瘤(12.5％)、髓母细胞瘤(10.2％)及室管膜瘤(7.4％).椎管内肿瘤最常见的是神经鞘瘤(48.6％)、星形细胞瘤(21.6％)和间叶肿瘤(13.5％).高级别肿瘤治疗后2年生存率为83.4％,5年生存率可达到65.0％.结论 儿童中枢神经系统肿瘤发病以男性居多,幕上多于幕下,星形细胞瘤最常见,临床表现与肿瘤部位及年龄相关,手术治疗是主要治疗手段,术后辅以放化疗可改善预后.     

小儿颅内肿瘤的临床与流行病学研究:西北地区单中心数据分析

目的回顾性分析西北地区单中心儿童颅内肿瘤的临床与流行病学特征。方法 2008年1月至2017年12月期间,对明确病理学类型的839例儿童(0~15岁)颅内肿瘤,按照2016版WHO中枢神经系统肿瘤分类新标准,分析肿瘤部位、类别和级别,及其与患儿性别、年龄和来源地区的关系。结果 839例颅内肿瘤患儿来源于西北五省,以幕上肿瘤居多(68.4%),低级别肿瘤占多数(56.4%)。总体男∶女为1.53∶1,7~9岁为发病高峰年龄。位于前五位的肿瘤分别是星形细胞肿瘤(16.4%)、颅咽管瘤(11.6%)、髓母细胞瘤(9.3%)、生殖细胞瘤(7.0%)和室管膜瘤(6.3%),男∶女为1.19∶1。低级别∶高级别为2.61∶1。结论本研究基于单中心近10年的儿童颅内肿瘤资料分析,在一定程度上反映了西北地区儿童颅内肿瘤的临床特征和流行病学特点。     

112例儿童幕下脑肿瘤临床资料分析

儿童脑肿瘤的发病率仅次于白血病,是儿童最常见的实质性肿瘤[1].儿童脑肿瘤中幕下肿瘤占大部分,以往报道[2]以小脑星形细胞瘤、髓母细胞瘤、脑干胶质瘤、室管膜瘤为主,其病理特点与成人明显不同.本文总结了112例儿童幕下脑肿瘤的发病部位、临床表现、病理学特点及其相互关系,现报告如下.     

儿童中枢神经系统肿瘤（附766例分析）

目的:探讨儿童中枢神经系统肿瘤的发病特点。方法:对华山医院１９８６~１９９８年儿童中枢神经系统肿瘤７６６例,进行回顾性分析。结果:本组占同期中枢神经系统肿瘤病例数的６．１４%。部位:颅内７２５例,其中幕上４９９例,幕下２７６例,椎管内４１例。组织学分类:前三位分别是:星形细胞性肿瘤(１９例,占２６．０%);颅咽管瘤(１１５例,占１５．０%);髓母细胞瘤(１０１例,占１３．２%)。性别:男孩多见。发病涉及各年龄组,病例数随年龄的增长而增加。结论:儿童中枢神经系统肿瘤位于幕上者多于幕下,颅内肿瘤以星形细胞性肿瘤最常见,男孩比女孩好发,为１．５０:１。     

小儿颅内肿瘤535例临床病理分析

自 1990年 12月至 2 0 0 1年 12月 ,我科共收治小儿颅内肿瘤 5 35例 ,占同时期成年颅内肿瘤 2 6 38例的 2 0 3%。发生儿童颅内肿瘤于中线位置居多。现在分析常见的鞍区颅咽管瘤与第四脑室周围的髓母细胞瘤临床病理与手术因果关系 ,报告如下。临床资料与方法　　 1.一般情况 :男 32 7例 ,女 2 0 8例。肿瘤幕上下以中线为最常见 ,占 2 4 0例。其中星形细胞瘤12 2例 ,室管膜瘤 78例 ,髓母细胞瘤 10 7例 ,生殖细胞瘤 32例 ,颅咽管瘤 82例 ,脑膜瘤 4 1例 ,松果体瘤 2 4例 ,血管网状细胞瘤 2 7例 ,其他如畸胎瘤等 2 2例。2 .症状及体征 :多以头痛…     

小儿幕上胶质瘤形态学和病理原因的研究

儿童脑肿瘤主要影响到旧的大脑结构、脑干、小脑和脊髓。它们常以髓母细胞瘤、室管膜瘤、星形细胞瘤(如少枝胶质母细胞瘤)出现,相反,新脑的神经外胚叶肿瘤不常见,成人肿瘤的特点(如     

高原波和b波与颅内顺应性的关系

高原波和Ｂ波与颅内顺应性的关系陈立华曹美鸿廖达光秦天森马建荣刘运生张明宇周艳红一、临床资料１．一般资料：男２８例，女１６例，年龄２～６９岁，平均年龄２３．４岁。星形细胞瘤１２例，颅咽管瘤４例，髓母细胞瘤６例，室管膜瘤３例，血管母细胞瘤３例，听神经瘤３...     

婴幼儿脑肿瘤临床及病理学特点：单中心100例分析

目的探讨婴幼儿脑肿瘤的特殊临床表现、病理类型以及分布特点。方法选择2011年1月至2018年12月行手术治疗的儿童(18岁)脑肿瘤患者,分为婴幼儿组(0～3岁)与大龄儿童组(4～18岁),归纳分析两组患者肿瘤病理类型、好发部位、临床表现等特点。结果垂体瘤仅发生于4～18岁大龄儿童(P=0.001);而室管膜瘤(P=0.001)、脉络丛乳头状瘤/癌(P=0.000)、髓母细胞瘤以外的其他胚胎性肿瘤(P=0.013)则好发于0～3岁婴幼儿,且大多位于幕上(P=0.023)。715例中共371例详细记录肿瘤大小,组间差异具有统计学意义(P=0.000);肿瘤大小30 mm(χ~2=33.939,P=0.000)、30～60 mm(χ~2=12.483,P=0.000)和61～100 mm(χ~2=23.158,P=0.000)患儿,与 100 mm患儿之间差异有统计学意义,且肿瘤大小 100 mm患儿中以婴幼儿所占比例最高。低级别脑肿瘤(WHOⅠ～Ⅱ级)患儿总生存期高于高级别脑肿瘤(WHOⅢ～Ⅳ级)患儿(P=0.000)。结论婴幼儿脑肿瘤具有不同于大龄儿童群体的特殊病理类型和临床特点。婴幼儿低级别胶质瘤预后良好,呈慢病特点;恶性脑肿瘤包括少见的各种胚胎性肿瘤,恶性程度高,易复发和发生神经系统多处种植转移,患儿无法接受放射治疗,而化疗药物的选择和疗效欠佳,预后极差。提高婴幼儿脑肿瘤患儿的生活质量和预后,需要更广泛的多学科合作。     

儿童第四脑室肿瘤的显微外科治疗(附43例报告)

目的 探讨儿童第四脑室常见肿瘤的诊断和显微手术技巧.方法 回顾性分析2006~2010年手术治疗的43例儿童第四脑室肿瘤患者的临床表现与影像学等临床资料;根据肿瘤与小脑蚓部和第四脑室的关系,设计个体化手术入路切除肿瘤.结果 肿瘤全切除38例,次全切5例(为与第四脑室底粘连紧密者).绝大多数病例无明显并发症或并发症较轻,经治疗后好转.病理结果示髓母细胞瘤27例,室管膜瘤8例,星形细胞瘤7例(其中毛细胞型3例),神经细胞瘤(交界性)1例.髓母细胞及WHOⅢ级的室管膜瘤术后进行了全脑和脊髓放疗,次全切的室管膜瘤和星形细胞瘤进行了局部放疗.结论 儿童第四脑室肿瘤以髓母细胞瘤最多见,其次是室管膜瘤和星形细胞瘤;正确的入路选择和手术技巧是取得良好疗效、减少并发症的关键.     

儿童髓母细胞瘤的综合治疗

儿童中枢神经系统原发肿瘤占所有儿童期肿瘤的 2 0 % ,发生率仅次于白血病居于第二位。髓母细胞瘤(ｍｅｄｕｌｌｏｂｌａｓｔｏｍａＭＢ)占儿童原发脑肿瘤的 2 0 % ,其发生率仅次于星形细胞瘤[1] 。发病高峰年龄在 7岁左右。髓母细胞瘤具有高度的软脑膜转移倾向 ,尤其在低龄儿童中常见。髓母细胞瘤 (ＭＢ)起源于小脑蚓部向四脑室生长 ,是最常见的儿童原发恶性脑肿瘤 ,是中枢神经系统恶性程度最高的神经上皮性肿瘤之一 ,属于原始神经外胚层肿瘤 (ｐｒｉｍｉｔｖｅｎｅｕｒｃｅｐｉｔｈｅｌｉａｌｔｕｍｏｒｓＰＮＥＴＳ)的一种 ,在ＷＨＯ的神…     

Treatment Considerations in Anticonvulsant Monotherapy

Summary: The long-standing practice of polypharmacy in treating epilepsy is giving way to use of monotherapy. Monotherapy can improve seizure control as well as reduce the risk of serious idiosyncratic reactions, dose-related side effects, and complex drug interactions. Monotherapy also offers improved compliance and cost-effectiveness. The basis of monotherapy is accurate diagnosis and assessment of the patient's seizure type(s), followed by selection of a single appropriate anticonvulsant drug. Many patients currently treated with multiple anticonvulsants can be successfully converted to monotherapy with a carefully monitored program in which troublesome and redundant drugs are gradually withdrawn from the therapeutic regimen.     

Dextromethorphan: Cellular Effects Reducing Neuronal Hyperactivity

Summary: Dextromethorphan is a dextrorotary morphinan without affinity for opioid receptors, commonly used as an antitussive medication. During the past 5 years, interest in the compound and its demethylated derivative, dextrorphan, has been revived because additional neuroprotective and an-tiepileptic properties were found in in vitro studies, animal experiments, and a few clinical cases. Both morphinans are able to inhibit N -methyl-D-aspartate (NMDA) receptor channels and voltage-operated calcium and sodium channels with different potencies. The inhibition of the NMDA receptor is believed to be the predominant mechanism of action responsible for the anticonvulsant and neuroprotective properties of the compounds.     

Pediatric Epilepsy Surgery

Summary: The use of implantable arrays of epidural electrodes has made it possible to carry out extraoperative electrocorticography (ECoG) and functional localization in the awake child. This has permitted cortical excisions that are determined by criteria similar to those obtained during surgical procedures performed under local anesthesia in adults. In addition, the method also permits simultaneous ECoG and video monitoring during the child's symptomatic seizures, providing additional important localizing information that is impractical to obtain in operations under local anesthesia. We report our experience with 75 children, ages 5 months to 15 years, whom we have managed with epidural electrode arrays. The method of extraoperative ECoG is described and illustrative cases are presented to demonstrate its feasibility and utility in children. In addition, we call attention to gliomas as a common cause of chronic focal seizures in children. Of 49 children undergoing resection and followed for from 1 to 14 years (mean of 5.8 years), 32 (65%) are either seizure free or have had a significant reduction in seizure frequency that has unambiguously improved their quality of life. The results are analyzed further by relating the surgical outcome to each of the pathologic entities that caused the seizures. This analysis reveals the variety of neurological conditions that commonly cause intractable focal seizure disorder in children and distinguishes those pathologic entities in which the seizure disorder is apt to respond to surgical intervention from those that will not.     

Un nouveau cadre conceptuel de travail pour une psychiatrie revisitée ? Introduction à deux articles de E. Kandel

In two articles which appeared in the American Journal of Psychiatry and that were subsequently translated for Évolution Psychiatrique, E. Kandel examines the bases for a reinterpreted psychiatry that is prepared to confront the major challenge of the 3rd millenium: that of insight into the mind and brain. This requires a major reorganization of the discipline, which involves a reinvestment of the scientific approach and a critical  assessment of the data provided by psychoanalytical psychiatry and cognitive neurosciences. Seven concepts have therefore been proposed for interactive re-examination: consciousness, the unconscious, memory, emotion, development, desire, impulse. The dynamic relations existing between genetics and the environment allow one to see how evolutions are possible from actions at different levels, both psychotherapeutic and pharmacological. Imaging and other techniques provide additional objective information to the process of human interaction which remains the basis of psychiatry. A common framework for psychiatry and the neurosciences, a reconsideration and renewal of the psychoanalytical approach are both possible and necessary.     

Opioids and the developing organism: A comprehensive bibliography, 1984–1988

A comprehensive bibliography of the literature concerned with opioids and the developing organism for 1984-1988 is presented. Utilized with companion papers (Neurosci. Biobehav. Rev. 6:439-479; 1982; 8:387-403; 1984), these articles cover the clinical and laboratory references beginning in 1875. For the years 1984, 1985, 1986, 1987, and 1988, a total of 877 citations were recorded. A series of indexes accompanies the citations in order to make the literature more accessible. These indexes are divided into clinical and laboratory topics, and subdivided into such topics as the type of opioid explored and the general area of biological interest (e.g., physiology).     

La biologie et le futur de la psychanalyse : un nouveau cadre conceptuel de travail pour une psychiatrie revisitée

The American Journal of Psychiatry has received a number of letters in response to my earlier “Framework” article (1). Some of these are reprinted elsewhere in this issue, and I have answered them briefly there. However, one issue raised by some letters deserves a more detailed answer, and that relates to whether biology is at all relevant to psychoanalysis. To my mind, this issue is so central to the future of psychoanalysis that it cannot be addressed with a brief comment. I therefore have written this article in an attempt to outline the importance of biology for the future of psychoanalysis.     

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.     

Facteurs de risque environnementaux à la schizophrénie

Schizophrenia is currently a major concern, its prevalence being estimated at around 1% and its social consequences being severe. The elucidation of the pathophysiology of the disease is difficult due to the great variability of clinical expressions, the instability of the clinical symptoms during the evolution and the absence of reliable biological markers. The existence of a familial aggregation in schizophrenia is well known, the risk of presenting the disease for first-degree relatives of patients being 5 to 10 times higher than the risk observed in the general population. The genetic component was further confirmed by twin and adoption studies. Although the concordance for the disease is higher (40 to 70%) among monozygotic twins as compared with dizygotic twins (15%) it does not reach 100%, which implies that environmental factors modulate the effects of the genotype. However, the role of these factors and especially their interaction with genetic factors remain unclear but the implications of some specific environmental factors are well documented by recent research data. The current literature on sex differences in schizophrenia is consistent. Several studies have suggested that male and female patients may differ in age at the onset and expression of clinical symptoms. Complications during pregnancy or birth-giving may increase the risk of developing schizophrenia later in life. The major complications are oxygen deprivation during pregnancy, bleeding, maternal malnutrition or infection (exposure to influenza, for example). A low birth weight is associated with an increased risk of schizophrenia. Psychoses are more common among people living in an urban environment and among those born during winter months. Schizophrenia is probably more prevalent in people who are living promiscuously, are subject to toxic abuse, poor nutrition and stress but here more precise data are needed. Moreover, immigrants have a higher risk of developing psychotic disorders. In addition, head traumas are associated with an increased risk of schizophrenia. Though they are contentious, some studies suggest that substance abuse (cannabis use in European countries) is related to the development of schizophrenia, especially in people with genetic vulnerability. Moreover, substance misuse may worsen the symptoms. If the environment is sufficiently stressful, people with a high genetic vulnerability will develop some degree of mental illness, including schizophrenia. Conversely, a less stressful or a protective environment may decrease the risk of its onset in persons with a predisposition to schizophrenia.