儿童植物神经性发作与(癎)样放电的关系

目的:探讨儿童植物神经发作性疾病与癫样异常放电的关系。方法:对167例临床诊断为植物神经性发作儿童的24h动态脑电图进行回顾性分析。结果:共监测到69例患儿临床发作,样放电(棘波、尖波、棘慢、尖慢波综合)11例,间期6例,样放电率为10.2%,腹痛性发作样放电率较高(X2=9.35,P<0.01)。结论:24h动态脑电图监测对儿童植物神经性发作诊断和鉴别诊断有着非常重要意义。     

动态脑电图加心脏导联监测对晕厥与癫痫的鉴别诊断价值

目的：探讨24小时动态脑电图（AEEG）加心脏导联监测对晕厥与癫痫的鉴别诊断价值。方法：对92例以昏倒就诊，但常规脑电图（REEG）和心电图（ECG）均阴性的患者做24小时AEEG加心脏导联监测，并结合临床进行观察。结果：46例AEEG检测到多次阵发性棘波、尖波、棘慢波综合、尖慢波综合等痫样放电而诊断为癫痫，24例诊断为晕厥（心源性晕厥占58．3％，脑源性晕厥占25％，反射性晕厥占16．7％），6例不能定性，16例正常，总阳性率为82．6％。结论：24小时AEEG加心脏导联监测能帮助鉴别临床以昏倒发作的晕厥与癫痫，并能区分晕厥的类型。     

18例复杂部分性癫痫发作患者同步录像脑电图监测报告

目的：探讨同步录像脑电图（Video-EEG）在复杂部分性癫痫发作诊断中的应用价值。方法：利用ZN8000型EEG仪对临床确诊的18例复杂部分性发作患者进行脑电和行为监测,监测时间2．5～28h平均用18.8h。结果：共监测到93次临床发作,睡眠中发作54次,清醒时发作39次,发作持续时间7～120s,平均34．8s。发作时的临床表现：双眼向前或向一侧凝视11例,头向一侧转动9例,一侧面部或肢体抽搐9例,行为或口咽自动症6例,精神症状5例,姿势性发作5例,对发作过程不能回忆15例。发作期EEG示癫病样放电（棘波、尖波、棘慢、尖慢综合波）12例,阵发性高幅慢波5例,未见异常1例,EEG异常率95％。发作间期EEG示中高幅阵发性慢波3例,阵发性痫样放电9例,未见异常6例,EEG异常率65％。结论：Video－EEG对复杂部分性癫痫发作有重要诊断价值,痫样放电与临床发作密切相关。     

85例癫痫儿童发作间期动态脑电图分析

目的：探讨动态脑电图（AEEG）对发作间期儿童癫痫的诊疗价值。方法：对85例癫痫儿童于发作间期监测24hAEEG并对其相关因素进行比较分析。结果：AEEG监测异常者74例（87．1％）,其中有痫样放电者49例（57．6％）;发作越频繁,AEEG痫样放电检出率越高（P〈0．01）;睡眠期痫样放电检出率显著高于清醒期（P〈0．01）。治疗后复查组较新诊断组癫痫儿童痫样放电显著减少（P〈0．05）。结论：发作间期AEEG监测有助于儿童癫痫的临床诊断及疗效评估     

脑瘫合并癫痫及发作性疾病患儿动态脑电图监测的临床意义

目的对脑性瘫痪合并癫痫及发作性疾病患儿进行24h动态脑电图（AEEG）监测,了解其对二者的诊断价值。方法50例脑瘫合并癫痫患儿,做常规脑电图及AEEG监测,30例脑瘫伴发作性疾病患儿为对照组,比较两组患儿同步脑电图监测符合率。探讨其临床意义。结果癫痫组,痫样放电45例（90%）,40例为同步放电,符合率80%。临床下电发作发放、非癫痫性事件各5例。发作性疾病组30例,痫样放电16例（53.3%）,4例（13.3%）为同步放电,避免了癫痫的漏诊。12例为发作性疾病与临床下电发作发放并存。14例背景波为落后于生理年龄的慢波,与发作无关。两组比较,癫痫组同步放电率（80%）,明显高于发作性疾病组（13.3%）,χ^2=33.66Р〈0.005。结论AEEG监测,可提高脑瘫合并癫痫及发作性疾病患儿痫样放电检出率。还可监测到发作时同步动态特征性改变,对脑瘫合并癫痫及发作性疾病的诊断、鉴别诊断,有着重要的临床价值。两组脑电图全部异常,可能与患儿多伴有脑皮层广泛病变有关。     

癫痫性痉挛发作的头皮及颅内脑电图特点

目的：研究难治性癫痫性痉挛发作患者的头皮及颅内脑电图（EEG）特点,探讨与痉挛发作相关的EEG变化及其与发作间期放电、神经影像学之间的关系。方法：回顾性分析经外科手术治疗的11例患者的临床资料,分析头皮同步视频脑电图（V-EEG）。此11例患者均行术中皮层EEG监测30～60min,其中4例术前行颅内电极长程EEG监测。结果：8例患者表现为双侧基本对称的痉挛发作,发作期头皮EEG为全导高波幅慢波、尖波伴低波幅快波活动或广泛低波幅快波活动发放;另3例患者表现为一侧肢体的痉挛发作,EEG为局灶性棘慢波发放。术中皮层监测5例患者为反复的、暴发出现的多棘波活动,2例患者见持续性的棘波、尖波活动,4例未见明显的癫痫样电活动。4例行颅内电极监测者发作期EEG表现,2例为“前导性”的高波幅棘波伴随20Hz左右的低波幅快波发放;另2例为局灶性低波幅快波活动并迅速扩散,无“前导性棘波”。手术切除“前导性棘波”或反复性、节律性痫样放电的皮层可消除发作。结论：在一部分癫痫性痉挛发作患者,其痉挛发作可能因新皮层局灶的电发放点燃,颅内EEG如果存在前导性的棘波,这个棘波部位可能是促发痉挛发作的点燃灶。完整切除术中监测呈现反复性、节律性痫样放电的皮层可取得较好的手术效果。     

151例儿童发作性疾病的24h动态脑电图监测

目的对儿童发作性疾病的脑电活动进行分析。方法对１５１例发作性疾病儿童应用２４ｈ便携式磁带记录脑电图（ＡＥＥＧ）进行监测。结果总异常率８０８％，痫样放电出现率７９５％。癫痫组痫样放电出现率７８８％，可疑癫痫组痫样放电出现率４５５％，两组之间有非常显著差异（Ｐ＜０００５）。ＡＥＥＧ痫样放电出现率明显高于常规脑电图（ＥＥＧ）；癫痫组与可疑癫痫组之间临床发作中所描记到的痫样放电出现率也有非常显著差异（Ｐ＜００１）。本组病人的痫样放电部位以全脑或一侧，或限局性一侧偏胜者多见，痫样放电时间以睡眠时期为主占７４２％，其中６７７％见于浅睡期。结论２４ｈ动态脑电图监测对于儿童发作性疾病的诊断有着非常重要的意义。     

儿童非癫癎性发作事件的动态脑电图监测

目的：观察儿童非癫痫性发作事件24h动态脑电图（AEEG）的特点及其诊断意义。方法：对AEEG监测到的121例儿童非癫痫性发作事件临床发作期EEG进行分析，并以常规脑电图（REEG）进行对照。结果：REEG非特异性异常13例10.7%)，AEEG非特异性异常30例，（24.8%），痫样放电3例（2.5%），总异常率27.2%。非特异性异常检出率以偏头痛最高（34.8%），其次为晕厥（23.5%）及睡眠障碍（22.2%）。结论：儿童非癫痫性发作事件AEEG多为正常或非特异性异常，这有助于儿童非癫痫性发作事件的诊断和鉴别诊断。     

癫痫病人24小时动态脑电图分析

目的：分析动态脑电图对癫的应用价值。方法：对121例常规脑电图未见痫样放电的癫痫病人，在一周内行24小时动态脑电图检查，观察痫样放电的检出率，结果：24小时动态脑电图痫样放电检出率72．73％（88／121）明显高于常规脑电图，涉及额颞区占73．86％（65／88），自然睡眠中痫样放电81例占92．05％（81／88），NREMⅠ，Ⅱ期出现痫样放电59例，占72．84％（59／81），结论：24小时动态脑电图对癫痫的诊断有重要意义。     

长程视频脑电图监测对非癫痫性发作诊断价值探讨

目的探讨长程视频脑电图监测在非癫痫性发作诊断中的应用价值。方法对48例酷似非癫痫性发作患者行24 h长程视频脑电图监测,将发作表现及同期的脑电图进行分析。结果 48例患者在监测中均出现临床发作,长程视频脑电图监测所有发作期脑电图均未见痫样放电。其中1例患者表现单个θ波发放,27例患者为肌电伪差或动作伪迹。13例患者在发作间期脑电图见单个尖波或慢波发放,35例患者为正常范围。结论长程视频脑电图监测可将非癫痫性发作的临床表现和同步脑电图结合起来,为非癫痫性发作的诊断提供重要的临床资料。     

Renal dysplasia and asplenia in two sibs

A family is reported in which two sibs, one male and the other female, both died within 24 hours of birth with enlarged polycystic kidneys. Postmortem histology in the second child showed gross renal dysplasia. In both children the pancreas was enlarged, nodular and cystic but the liver appeared macroscopically normal. In the second child, histological examination confirmed pancreatic fibrosis with cystic dilation of ducts, but showed portal fibrosis with bile duct proliferation in the liver.
This combination of findings is very reminiscent of those in a girl and her brother reported by Ivemark et al. (1959). The children reported here also showed absence or hypoplasia of the spleen, cardiac anomalies and other features of the Ivemark syndrome (Ivemark 1955), a quite different, usually sporadic, congenital disorder. It is suggested that the children described here have a distinct lethal congenital disorder, probably inherited in an autosomal recessive manner.     

Schizophrenia in a North Swedish geographical isolate, 1900–1977. Epidemiology, genetics and biochemistry

Over 200 schizophrenic patients belonging to three major and interrelated pedigree complexes have been investigated over the past 30 years in a North Swedish geographically isolated population, presently numbering about 6,000. An intensive investigation of a number of biochemical correlates and genetic markers in a few selected families belonging to one of the major pedigrees has indicated new strategies for the current research program.
Schizophrenia, as defined operationally, is significantly associated with decreased activities of two enzymes (1) blood platelet monoamine oxidase, (2) plasma dopamine-β-hydroxylase, and (3) with the genetic marker Gc² (group specific antigen). Both enzymes are subject to genetic variation. A positive score for linkage between schizophrenia and low plasma DBH activity has been calculated, but, so far, available data are insufficient for discrimination between linkage and partial contribution of genetically controlled low plasma DBH to the pathogenesis of the disease. Alternatively, both mechanisms could be involved.
As a model for continued research, schizophrenia is explained as based on a double dominant-recessive genotype (Aabb), representing a vulnerability which in about 50 % of cases develops into clinical schizophrenia. It is suggested that the dominant mutation (A) operates on or affects MAO activity, and that the recessive genotype (bb) is instrumental in low variates of DBH activity and very likely such variates within the normal range of physiological variation. Moreover, it is suggested that the combined effects of MAO- and DBH-reduced efficiency on the metabolism of e.g. dopamine could be an essential pathogenic mechanism for the schizophrenic illness which is segregating in this population.     

The dominant diseases of modernized societies as omega-3 essential fatty acid deficiency syndrome: Substrate beriberi

About 1900, modern food selection and processing caused widespread $\text{epidemics}$ of the B vitamin deficiency diseases of beriberi and pellagra which, for genetic reasons, often expressed as different diseases ranging from bowel and heart disease to dermatoses and psychoses. But the B vitamins merely help convert essential fatty acids (EFA) into the prostaglandin (PG) tissue regulators and it now turns out that, through hydrogenation, milling and selection of w3-poor southern foods, we have also been systematically depleting, by as much as 90%, a newly discovered trace Nordic EFA (w3) of special importance to primates and sole precursor of the PG3(4) series, even as a concurrent fiber deficiency increases body demand for EFA. Since substrate EFA is processed by many B vitamin catalysts, an EFA deficiency will mimic a $\text{panh}$ypovitaminosis B, i.e., a mixture of $\text{substrate}$ beriberi and $\text{substrate}$ pellagra resembling vitamin beriberi and pellagra but exhibiting as even more diverse $\text{endemic}$ disease. This would consitute a second stage of the Modern Malnutrition and explain why some workers now hold the dominant diseases of modermized societies to be new, nutritionally based, pellagraform yet lipid-related and to range, once again, from heart disease to psychosis. It is an assumption that our dominant diseases are unrelated to each other or are merely revealed by our diagnostic acumen and therapeutic success; and that hydrogenating millions of tons of food oils annually, to destroy the rancidity producing w3-EFA, is safe for $\text{primates}$. Extensive beriberiform disease is reported here in 32 typical cases taken from medical practice which responds strikingly to linseed oil supplements (60% w3-EFA) in confirmation of identical results in Capuchins.     

What will studies of Fulani individuals naturally exposed to malaria teach us about protective immunity to malaria?

There are an estimated over 200 million yearly cases of malaria worldwide. Despite concerted international effort to combat the disease, it still causes approximately half a million deaths every year, the majority of which are young children with Plasmodium falciparum infection in sub-Saharan Africa. Successes are largely attributed to malaria prevention strategies, such as insecticide-treated mosquito nets and indoor spraying, as well as improved access to existing treatments. One important hurdle to new approaches for the treatment and prevention of malaria is our limited understanding of the biology of Plasmodium infection and its complex interaction with the immune system of its human host. Therefore, the elimination of malaria in Africa not only relies on existing tools to reduce malaria burden, but also requires fundamental research to develop innovative approaches. Here, we summarize our discoveries from investigations of ethnic groups of West Africa who have different susceptibility to malaria.     

'Very sore nights and days': the child's experience of illness in early modern England, c.1580-1720

Sick children were ubiquitous in early modern England, and yet they have received very little attention from historians. Taking the elusive perspective of the child, this article explores the physical, emotional, and spiritual experience of illness in England between approximately 1580 and 1720. What was it like being ill and suffering pain? How did the young respond emotionally to the anticipation of death? It is argued that children’s experiences were characterised by profound ambivalence: illness could be terrifying and distressing, but also a source of emotional and spiritual fulfilment and joy. This interpretation challenges the common assumption amongst medical historians that the experiences of early modern patients were utterly miserable. It also sheds light on children’s emotional feelings for their parents, a subject often overlooked in the historiography of childhood. The primary sources used in this article include diaries, autobiographies, letters, the biographies of pious children, printed possession cases, doctors’ casebooks, and theological treatises concerning the afterlife.     

Guidelines for the Validation and Use of Immunoassays for Determination of Introduced Proteins in Biotechnology Enhanced Crops and Derived Food Ingredients

Recent advancements in agricultural biotechnology have created a need for analytical techniques to determine introduced proteins in crops enhanced through modern biotechnology techniques. These proteins are expressed in plant tissues and may be present in food ingredients. Immunoassays are ideally suited for protein detection and may be used as both quantitative and threshold methods. Microplate ELISA and lateral flow devices are two of the most commonly used immunoassay formats for agricultural biotechnology applications. This paper provides general background information and a discussion of criteria for the validation and application of immunochemical methods to the analysis of proteins introduced into plants and food ingredients using biotechnology methods. It is the result of a collaborative effort of members of the Analytical Environmental Immunochemical Consortium. This collaborative effort represents the combined expertise of several organizations to reach consensus on establishing guidelines for the validation and use of immunoassays. Further, the paper offers developers and users a consistent approach to adopting the technology as well as aid in producing accurate and meaningful results.     

Ultracryotomy: development and application (with examples from the yeast cytology) (author's transl)

The preparation steps usually necessary for obtaining ultrathin frozen sections of biological material (chemical prefixation, enclosing, cryoprotective treatment, freezing, sectioning, and post-staining the sections for transmission electron microscopy) are submitted to a critical analysis. The application of cryo-ultramicrotomy, in particularly for cytochemical purposes, is reviewed. Fundamental considerations of chemical prefixation and poststaining are supported by examples from yeast cytology. Furthermore, the efficiency of the cryo-ultramicrotomy (electron optical resolution of ultrastructural details) is demonstrated on yeast cells and protoplasts.     

HLA in North Colombia Chimila Amerindians

HLA-A,-B,-C,-DRB1 and -DQB1 alleles have been studied in Chimila Amerindians from Sabana de San Angel (North Colombian Coast) by using high resolution molecular typing. A frequent extended haplotype was found:HLA-A*24:02-B*51:10-C*15:02-BRB1*04:07-DQB1*03:02 (28.7%) which has also been described in Amerinndian Mayos Mexican population (Mexico, California Gulf, Pacific Ocean). Other haplotypes had already been found in Amerindians from Mexico (Pacific and Atlantic Coast), Peru (highlands and Amazon Basin), Bolivia and North USA. A geographic pattern according to HLA allele or haplotype frequencies is lacking in Amerindians, as already known. Also, five new extended haplotypes were found in Chimila Amerindians. Their HLA-A*24:02 high frequencies characteristic is shared with aboriginal populations of Taiwan; also, HLA-C*01:02 high frequencies are found in New Zealand Maoris, New Caledonians and Kimberly Aborigines from Australia. Finally, this study may show a model of evolutionary factors acting and rising one HLA allele frequency (-A*24:02), but not in others that belong to the same or different HLA loci.     

The case for allele‐specific recognition by the TCR

There is a sharp difference in how one views TCR structure–function–behaviour dependent on whether its recognition of major histocompatibility complex‐encoded restriction elements (R) is germline selected or somatically generated. The generally accepted or Standard model is built on the assumption that recognition of R is by the V regions of the αβ TCR, which is not driven by allele specificity, whereas the competing model posits that recognition of R is allele‐specific. The establishing of allele‐specific recognition of R by the TCR would rule out the Standard model and clear the road to a consideration of a competing construct, the Tritope model. Here, the case for allele‐specific recognition (germline selected) is detailed making it obvious that the Standard model is untenable.