Primary rhabdoid tumour of the brain

Summary A posterior fossa tumour in a 3 year old child is presented with characteristic histological, ultrastructural and immunohistochemical features of rhabdoid tumour. Many tumour cells contained cytoplasmic eosinophilic hyaline inclusions. Ultrastructurally concentric whorls of 10 nm intermediate filaments were identified. Immunohistochemical staining disclosed vimentin, cytokeratin and epithelial membrane antigen positivity. Renal and extrarenal rhabdoid tumours have been well documented but a primary rhabdoid tumour of the brain is extremely rare. Additional ultrastructural features seen were tubular crystalline inclusions in endoplasmic reticulum and abnormal large mitochondria.     

The effect of erroneous responses on response monitoring in patients with major depressive disorder: a study with event-related potentials

Perceived failure is reported to have detrimental effects on subsequent performance in patients with major depressive disorder. We investigated the error-related negativity (ERN)/error negativity (Ne), an electrophysiological correlate of response monitoring, using a 64-channel EEG. Sixteen patients with DSM-IV major depressive disorder and 16 matched controls participated in an Eriksen flanker task with continuous performance feedback that signaled monetary reward. Compared to controls, patients with major depressive disorder showed a less negative ERN/Ne in error trials following error trials. This result might reflect impaired response monitoring processes in major depressive disorder resulting from an underactivity in a central reward pathway and/or a deficit in strategic reasoning.     

Ara h 8, a Bet v 1-homologous allergen from peanut, is a major allergen in patients with combined birch pollen and peanut allergy

BACKGROUND: We recently described patients with soybean allergy mainly mediated by cross-reactivity to birch pollen allergens. A majority of those patients were reported to have peanut allergy. OBJECTIVE: We sought to study the occurrence of peanut allergy in patients allergic to birch pollen and characterized the Bet v 1-homologous peanut allergen Ara h 8. METHODS: Recombinant Ara h 8 was cloned with degenerated primers and expressed in Escherichia coli. Nine Swiss and 11 Dutch patients with peanut and birch pollen allergy and a positive double-blind, placebo-controlled food challenge result to peanut were investigated for IgE reactivity to birch pollen and purified peanut allergens and cross-reactivity between birch and peanut. Ara h 8 stability against digestion and roasting was assessed by means of RAST inhibition. The IgE cross-linking potency of Ara h 8 was tested on the basis of basophil histamine release. RESULTS: During double-blind, placebo-controlled food challenge, all patients experienced symptoms in the oral cavity, progressing to more severe symptoms in 40% of patients. CAP-FEIA detected recombinant (r) Ara h 8-specific IgE in 85%. IgE binding to Ara h 8 was inhibited by Bet v 1 in peanut extract immunoblotting and in RAST inhibition. In EAST inhibition recombinant rAra h 8 inhibited IgE binding to peanut in 4 of 7 tested patient sera. Antipeanut response was dominated by Ara h 8 in 12 of 17 tested patients. Furthermore, our results demonstrate a low stability of Ara h 8 to roasting and no stability to gastric digestion. Basophil histamine release with rAra h 8 was more than 20% in 5 of 7 tested sera. CONCLUSIONS: Peanut allergy might be mediated in a subgroup of our patients by means of cross-reaction of Bet v 1 with the homologous peanut allergen Ara h 8.     

Malignant epithelioid haemangioendothelioma of the liver: a clinicopathological and histochemical study of 12 cases

We describe the clinicopathological findings in 12 cases of hepatic epithelioid haemangioendothelioma in order to identify diagnostic and prognostic features of this unusual vascular neoplasm. Three main tumour patterns were observed histologically: (1) a peripheral pattern with neoplastic cells scattered between fairly normal liver cell plates; (2) a cellular pattern showing a confusing admixture of pleomorphic tumour cells and atrophied hepatocytes set in a small amount of fibrous stroma; and (3) a scarred pattern with sparse tumour cells in a dense fibrous matrix. There were two types of vascular invasion: tuft-like intravascular proliferations of epithelioid cells and fibro-thrombotic venous occlusions. Awareness of these different aspects is important, as they are variably sampled by biopsy needles. The clinical course in this series was less favourable than that previously reported. Eight patients have died, in six instances of liver failure within 4-41 months of diagnosis. Extensive involvement of both lobes of the liver heralds imminent hepatic failure. The slow growth of metastases may justify liver transplantation in order to prolong life.     

A mathematical simulation of the AIDS patient and extracorporeal detoxification

A simple numerical simulation of AIDS patient detoxification by a hypothetical extracorporeal device for the removal of viruses, infected white cells, and syncytia has been designed. The mathematical model accounts for healthy blood white cells attacking and destroying the viruses, while at the same time the viruses attack and infect certain white cells. The infected white cells serve as a site for viral growth; eventually the cells lyse, releasing a large number of viruses into the blood stream. The healthy white cells and infected white cells combine to form syncytia, where the virus multiplies, and finally the syncytium ruptures releasing all the virus. This model can be used to predict concentrations over a specified period for the patient. This is a mathematical model to be used as a research and design tool only.     

Structural and functional analysis of a new desmin variant causing desmin‐related myopathy

Desmin‐related myopathy is a familial or sporadic disease characterized by skeletal muscle weakness and cardiomyopathy as well as the presence of intracytoplasmic aggregates of desmin‐reactive material in the muscle cells. Previously, two kinds of deletions and eight missense mutations have been identified in the desmin gene and proven to be responsible for the disorder. The present study was conducted to determine structural and functional defects in a pathogenic desmin variant that caused a disabling disorder in an isolated case presenting with distal and proximal limb muscle weakness and cardiomyopathy. We identified a novel heterozygous Q389P desmin mutation located at the C‐terminal part of the rod domain as the causative mutation in this case. Transfection of desmin cDNA containing the patient’s mutation into C2.7, MCF7, and SW13 cells demonstrated that the Q389P mutant is incapable of constructing a functional intermediate filament network and has a dominant negative effect on filament formation. We conclude that Q389P mutation is the molecular event leading to the development of desmin‐related myopathy. Hum Mutat 18:388–396, 2001. © 2001 Wiley‐Liss, Inc.     

音乐治疗对眼底手术病人焦虑的影响

目的：在于观察经听觉通道输入音乐对眼底手术病人焦虑状态的影响。方法：实验对象为39名择期进行眼底手术的男性病人，他们被随机地分为实验和对照两组，其中实验组19人，对照组20人，在手术过程中通过耳机给患者输入他们自己选定的音乐、观察焦虑（STAI），抑郁（SDS），视觉模拟焦虑（VAS）和脑电（BI）的变化。结果：实验组STAI，SDS，VAS值与对照组比明显降低，病人的焦虑、抑郁情绪有了明显改善，而脑电的变化没有显著性的差异。结论：音乐治疗可降低择期眼底手术男性病人的焦虑、抑郁与视觉模拟焦虑水平。     

P300等诱发电位对亚临床肝性脑病早期诊断的意义

目的:观察事件相关电位P_(300)、体感诱发电位、视觉诱发电位和脑干听觉诱发电位在亚临床肝性脑病诊断中的意义。方法:50例亚临床肝性脑病患者进行了心理测验,并做了头颅CT和视觉诱发电位、体感诱发电位、脑干听觉诱发电位和事件相关电位检查,30例正常人做为对照组。结果:亚临床肝性脑病的心理测验,韦氏法智力低下率为76％,视觉诱发电位异常率为13％,脑干听觉诱发电位异常率为20％,体感诱发电位异常率为40％,事件相关电位异常率为71％,正常对照组四种诱发电位的正常率是100％。头颅CT结果与亚临床肝性脑病无关。结论:视觉诱发电位、体感诱发电位、脑干听觉诱发电位和事件相关电位在亚临床肝性脑病中是异常的。事件相关电位更为敏感,对亚临床肝性脑病的早期诊断有重要意义。     

The “Mozart Effect”: An Electroencephalographic Analysis Employing the Methods of Induced Event-Related Desynchronization/Synchronization and Event-Related Coherence

The event-related responses of 18 individuals were recorded while they were listening to 3 music clips of 6 s duration which were repeated 30 times each. The music clips differed in the level of their complex structure, induced mood, musical tempo and prominent frequency. They were taken from Mozart's sonata (K. 448), and Brahms' Hungarian dance (no. 5). The third clip was a simplified version of the theme taken from Haydn's symphony (no. 94) played by a computer synthesizer. Significant differences in induced event-related desynchronization between the 3 music clips were only observed in the lower-1 alpha band which is related to attentional processes. A similar pattern was observed for the coherence measures. While respondents listened to the Mozart clip, coherence in the lower alpha bands increased more, whereas in the gamma band a less pronounced increase was observed as compared with the Brahms and Haydn clips. The clustering of the three clips based on EEG measures distinguished between the Mozart clip on the one hand, and the Haydn and Brahms clips on the other, even though the Haydn and Brahms clips were at the opposite extremes with regard to the mood they induced in listeners, musical tempo, and complexity of structure. This would suggest that Mozart's music--with no regard to the level of induced mood, musical tempo and complexity--influences the level of arousal. It seems that modulations in the frequency domain of Mozart's sonata have the greatest influence on the reported neurophysiological activity.     

糖尿病大鼠视网膜基因表达谱差异的初步分析

目的 建立大鼠正常视网膜和糖尿病8周视网膜基因表达谱，比较两者差异，初步分析糖尿病视网膜病变的相关基因。方法 通过限制片段差异显示 PCR（ restriction fragments differential display-PCR，RFDD-PCR）获得正常大鼠视网膜及8周糖尿病大鼠视网膜组织转录组片段。应用Fraent Analysis等软件，对差异片段进行生物信息学分析，初步确定糖尿病视网膜病变相关基因／表达序列标签（ expression sequence tag, Ksr）。结果 获得有意义的片段共3639个，有差异的片段840个，占表达数的23．08％。其中包括5个视觉传导相关基因，13个兴奋性神经递质受体基因和3个抑制性神经递质受体基因。糖尿病8周大鼠视网膜Rhodopsin kinase,β-arrestin,Phosducin, rod photoreceptor cGMP-gated channel 和 Rpe65的表达下调，离子型谷氨酸受体iGluR1-4下调，代谢性谷氨酸受体及γ-氨基丁酸受体各亚型则普遍上调，而甘氨酸受体表达无变化。结论 糖尿病8周大鼠神经视网膜已受到累及，其基因表达模式的改变，可能与糖尿病早期视功能损害有关。