Acoustically evoked brain stem potential in Wilson disease

By 48 patients with Wilson's disease the brainstem acoustic evoked potentials were stated. There were 35 pathological findings (73%), 13 were normal (27%). The waves, determined by the middle and upper brainstem (Pons and Mesencephalon) showed most of all pathological changes. Those patients with forms of Wilson's disease called Pseudoskelerose and Pseudoparkinson showed the most pathological findings. But one could watch these findings by about 30% of patients in preclinical stage. This method can possibly be used for early detection of disorders of brainstem functions and in the same way it can be used for control of treatment by patients in preclinical stage.     

Clinical, electroencephalographic and computer tomographic studies of children with West and Lennox-Gastaut syndrome

The results of clinical, electroencephalographic and repeated computed tomographic examination of 14 children suffering from epileptic seizures in connection with the West and Lennox-Gastaud syndrome are analyzed and compared. Computed tomography yields little information regarding the etiology of such forms of epilepsy. Distinct clinical and electroencephalographic findings were accompanied by normal computed tomographic results in a large proportion of the cases. To avoid misinterpretation of computed tomographic findings and consequent prognostic errors in these epileptic cases it is necessary to take possible side effects of ACTH and corticosteroid treatment into account. At the climax of hormone treatment computed tomography revealed imposing changes in the form of generalized atrophy in 10 of the 14 children examined. These receded completely upon termination of hormone treatment.     

Hemispheric alpha asymmetries and behavioral responses of aphasic and normal subjects for the recall and recognition of active, passive, and negative sentences

The hemispheric alpha asymmetries of a group of normal males, a group of normal females, and a group of aphasic patients (fluent and dysfluent) were examined with electroencephalographic (EEG) techniques under memory conditions of recall and recognition of active, passive, and negative sentences. Aphasic patients, regardless of classification, showed right hemispheric alpha suppression across memory conditions and sentence types. Both normal groups were found to have greater left hemispheric alpha suppression for the recall memory condition and greater right hemispheric alpha suppression for the recognition memory condition (males significantly more than females). The aphasic subjects performed better on recognition tasks compared to recalled tasks and active sentences compared to transformed sentences. Results are discussed as providing evidence of the aphasic subjects' greater dependency on information processing resources of the right hemisphere in the recovery of language function. The findings are also discussed as providing support for a multiple-resources model of information processing.     

Pediatric and Adult Recommendations Vary for Sibling Testing in Cystic Fibrosis

Four to 5 % of cystic fibrosis (CF) patients are diagnosed as adults and often have subtler symptoms. Their siblings are at genetic risk to also have a subtler disease state. Diagnostic testing is recommended for siblings of newly diagnosed infants, but recommendations are less clear for later diagnoses. This study explored sibling testing recommendations in pediatric and adult practice using a survey that was emailed to CF clinicians. There were 58 respondents. Results revealed that 82.5% of pediatric and 36.4% of adult care respondents reported always recommending diagnostic testing for siblings of a newly diagnosed patient. In adult care, another 33.3% reported recommending diagnostic testing if the sibling has symptoms. In pediatric care, whether the sibling had newborn screening was most influential. Most pediatric respondents prefer the sweat chloride test, while 40% in adult practice prefer familial mutation analysis. Perceived barriers included cost, insurance coverage and logistical concerns in both settings, parental emotional state in pediatrics, and concern making recommendations for someone who is not the patient in adult care. Genetic counselors may be able to meet familial needs in CF care, including sibling testing. Many newly diagnosed patients/families do not see a genetic counselor, especially in adult care. These data reveal opportunities for practice guidelines and standardization.     

IQ correlations in transracial adoptive families

IQ tests were administered to all available members over 4 years old in 101 transracial adoptive families when the adopted children were an average of 7 years old and again when they averaged 17 years old. At both times, 426 members of 93 families were studied; 398 were seen in person and administered the WAIS-R or WISC-R. IQ correlations were calculated for adopted and biological parent-child pairs, and for genetically related and unrelated siblings. Educational levels of birth parents were correlated with the IQ scores of their adopted-away children. Results show that biologically related family members tended to resemble each other intellectually more than did adoptive family members at both time points. IQ correlations for biological parent-child pairs exceeded those for adoptive parent-child pairs, and correlations were greater for genetically related than unrelated siblings. In late adolescence, the IQ scores of unrelated siblings in the transracial adoptive families were more similar than those of unrelated adolescent siblings pairs reported in other studies. The pattern of IQ correlations for unrelated siblings suggested that familial environmental influences on IQ decline from childhood to late adolescence, but this conclusion was not supported by parent-child IQ correlations. The effects of selective placement on familial IQ correlations were small. Estimates of genetic and familial environmental influences on IQ were very similar to those of other studies. This suggests that the influences on intellectual development in this sample of black/interracial adoptees reared in white families are similar to those for children in the majority populations of the United States and Western Europe.     

Explaining Rigidity and Pragmatism in Political Leaders: A General Theory and a Plausibility Test from the Reagan Presidency

Classic studies of a few extreme, almost pathological cases of rigidity in political leaders (e.g., Woodrow Wilson's League of Nations debacle) have tended to obscure the fact that virtually all leaders alternate between periods of pragmatic flexibility and rigid intransigence in the face of political opposition or unpleasant facts. This study develops a general theoretical framework for explaining rigidity and flexibility in political leaders by extending insights from classic studies of extreme cases to everyday policymaking and by integrating these findings with more recent theoretical and empirical work in political psychology. The plausibility of hypotheses derived from this framework is investigated by examining the conditions associated with pragmatism and rigidity in 15 cases from the presidency of Ronald Reagan. The results are generally consistent with the proposed model, which stresses person-situation interaction and traces rigidity to the arousal of leaders' sources of self-validation in a particular policy context. I conclude by evaluating the implications of this new framework for other areas of theory and research.     

Does depression interfere with effort mobilization? Effects of dysphoria and task difficulty on cardiovascular response

On the basis of predictions of the mood-behavior model (G. H. E. Gendolla, 2000) and motivational intensity theory (J. W. Brehm & E. A. Self, 1989), the authors conducted 2 studies that critically tested the common assumption that dysphoria is associated with a motivational deficit. Dysphoric and nondysphoric undergraduates performed a cognitive task that was either easy or difficult. Effort intensity (i.e., resource mobilization) was assessed as performance-related cardiovascular reactivity. In support of the authors' predictions and in contrast to the popular view of a general motivational deficit, both studies found a crossover interaction between dysphoria and task difficulty: In the difficult condition, nondysphoric participants indeed showed stronger systolic blood pressure reactivity than dysphoric participants. But in the easy condition, dysphoric participants showed stronger systolic reactivity than nondysphoric participants. The findings are discussed with respect to motivational deficits in depression and possible underlying mechanisms.     

A study of alpha hemispheric asymmetries for verbal and nonverbal stimuli in males and females

The alpha hemispheric asymmetries of males and females were examined with electroencephalographic (EEG) techniques during exposure to connected speech and nonlinguistic stimuli. The subject selection controlled for familial handedness and each subject was administered the Block Design and Vocabulary Subtests of the WAIS as a general measure of “visual-spatial” and “verbal” ability. The results showed that there was less alpha in the right hemisphere for nonverbal tasks and less alpha in the left hemisphere for verbal tasks. The females showed significantly more “lateralization” during the tasks as compared to the males. The results are discussed in terms of processing differences and the possible effects of subject variables, stimulus mode, stimulus presentation, and task requirements on neurolinguistic research results.     

Discrimination by olfactory cues in albino rats reflecting familiarity and relatedness among conspecifics

The present investigation sought to determine whether albino rat pups could discriminate among familiar siblings, nonfamiliar siblings, familiar nonsiblings (foster littermates), and nonfamiliar nonsiblings (unrelated agemates) using only olfactory cues. Pairing all possible combinations of familiarity and kinship variables (six), the odors were presented in paired combinations to each of twelve experimental subjects. Results from dependent t tests indicated that the albino rat pups could discriminate between the following pairs of odors using only the olfactory sensory modality: (a) familial siblings and nonfamiliar siblings, (t = 3.41, p = .006); (b) familiar siblings and nonfamiliar nonsiblings, (t = 9.62, p = .001); (c) nonfamiliar siblings and nonfamiliar nonsiblings, (t = 3.15, p = .009); and (d) familiar nonsiblings and nonfamiliar nonsiblings (t = 2.58, p = .026).     

Imaging Brain Effects of APOE4 in Cognitively Normal Individuals Across the Lifespan

The ε4 allele of the apolipoprotein E (APOE4) is associated with an increased risk of developing Alzheimer’s disease (AD). Hence, several studies have compared the brain characteristics of APOE4 carriers versus non-carriers in presymptomatic stages to determine early AD biomarkers. The present review provides an overview on APOE4-related brain changes in cognitively normal individuals, focusing on the main neuroimaging biomarkers for AD, i.e. cortical beta-amyloid (Aβ) deposition, hypometabolism and atrophy. The most consistent findings are observed with Aβ deposition as most studies report significantly higher cortical Aβ load in APOE4 carriers compared with non-carriers. Fluorodeoxyglucose-positron emission tomography studies are rare and tend to show hypometabolism in brain regions typically impaired in AD. Structural magnetic resonance imaging findings are the most numerous and also the most discrepant, showing atrophy in AD-sensitive regions in some studies but contradicting results as well. Altogether, this suggests a graded effect of APOE4, with a predominant effect on Aβ over brain structure and metabolism. Multimodal studies confirm this view and also suggest that APOE4 effects on brain structure and function are mediated by both Aβ-dependent and Aβ-independent pathological processes. Neuroimaging studies on asymptomatic APOE4 carriers offer relevant information to the understanding of early pathological mechanisms of the disease, although caution is needed as to whether APOE4 effects reflect AD pathological processes, and are representative of these effects in non-carriers.     

Genetic architecture of verbal abilities in children and adolescents

The etiology of individual differences in general verbal ability, verbal learning and letter and category fluency were examined in two independent samples of 9‐ and 18‐year‐old twin pairs and their siblings. In both age groups, we observed strong familial resemblance for general verbal ability and moderate familial resemblance for verbal learning, letter and category fluency. All familial resemblance was explained by genetic factors. There was significant covariance among the tests, which was stronger in magnitude in the adolescent cohort. The covariance was mainly explained by genetic effects shared by subtests, both in middle childhood and in late adolescence. In addition to a shared set of genes that influenced all phenotypes, there were also genetic influences specific to the different verbal phenotypes.     

Adoption Is a Successful Natural Intervention Enhancing Adopted Children's IQ and School Performance

ABSTRACT— Is the cognitive development of adopted children different from that of (a) children who have remained in institutional care or in their birth families or (b) their current (environmental) nonadopted siblings or peers? We attempt to answer these questions on the basis of a meta-analysis of 62 studies including 17,767 adopted children. Compared to their nonadopted siblings or peers who stayed behind, adopted children scored substantially higher on IQ tests and they performed much better at school. Compared to their current nonadopted environmental peers or siblings, adopted children showed similar IQ scores but their school performance and language abilities lagged somewhat behind. Most importantly, we found a twofold increase in special-education referrals in adopted children compared to their nonadopted peers. Taken together, the findings document the positive impact of adoption on children's cognitive development and adopted children's remarkably normal cognitive competence but somewhat delayed school performance.     

Wilson's disease in East Germany: in retrospect and perspectives -- an evaluation

Wilson's disease is an autosomal recessive inherited metabolic disorder due to a disturbance of copper metabolism. Although the primary genetic defect is not known a longlife treatment is necessary for establishing a negative copper balance by removing the metal of the abnormal body stores. Experiences in handling with this disease in our country over a period of 20 years are reported. Especially epidemiologic findings, the diagnostic procedures and the strategies in therapeutic regimes are discussed. Future advances in genomic diagnostics are mentioned.     

Electroencephalographic examinations following strangulation]

An electroencephalogram (E.E.G.) was recorded from 12 patients within 4 hours after strangulation. The tracings were mainly flat and showed severe general alterations. Repeated recordings were performed in 16 patients during the further course at regular intervals until the findings had normalized. Despite the marked initial findings, in 13 patients the basic activity had normalized within 48 hours after strangulation, but often it was asymmetric. Marked asymmetry and focal findings subsided within a year. Only in juvenile patients with initially marked neurological symptoms and subsequent demential course focal findings were found over several years. Contrary to the results of other investigations, spike potentials occurred in 2 patients with tonic-clonic seizures as focal and generalized, resp. steep. waves.     

Useful visual field in patients with schizophrenia: a choice reaction time study

This study examined the size of the useful visual field in patients (9 men, 6 women) with schizophrenia. A choice reaction task was conducted, and performances at 2.5, 5, 7, 10, and 25 degrees in both visual fields were measured. Three key findings were shown. First, patients had slower choice reaction times (choice RTs) than normal controls. Second, patients had slower choice RTs in the right visual field than in the left visual field. Third, patients and normal controls showed the same U-shaped choice RT pattern. The first and second findings were consistent with those of other studies. The third finding was a clear indication of the patients' performance in peripheral vision, and a comparison with normal controls suggested that there was no difference in the size of the useful visual field, at least within     

NR3C1基因Bcl1多态性与青少年抑郁：压力性生活事件的调节作用

迄今,有关抑郁的基因×环境交互研究多数基于“单胺缺陷假说”,相对较少有研究以“HPA轴假说”为框架考察抑郁的遗传机制,且忽视了基因-环境相关的影响。本研究对1081名青少年进行追踪研究,考察NR3C1基因Bcl1多态性与压力性生活事件对抑郁的影响。结果发现,经历较多压力性事件时,C等位基因携带者的抑郁水平显著高于GG纯合子携带者;经历较少压力性事件时,不同基因型携带者的抑郁水平无差异。此外,通过区分独立性压力性事件和预测早期抑郁进一步排除了基因-环境相关的影响,在一定程度上验证了结果的可靠性。     

COCAINE ADDICTS AND THEIR FAMILIES: AN EMPIRICAL STUDY OF THE PROCESSES OF IDENTIFICATION

The processes of identification between adolescent cocaine addicts and their parents were studied in 402 subjects, in total 134 familial triads (father"mother"son), subdivided into two groups of 67 triads, one of these groups having as the child an adolescent of masculine sex dependent on cocaine and the other, equal in number, being a control group, duly matched for age and socio-economic status. The instrument employed was the Rorschach test (1922), limited to the application of the Lerner Defense Scale (LDS; Lerner & Lerner, 1980). The findings in the affected triads showed up as consistent statistically for the presence of intense processes of pathological identification, especially between father and son, a sign of the importance of the presence of disturbances of paternal function in the development of this addiction. The utilisation of very regressive defence mechanisms, above all of projective identification, was the predominant mode of procedure in triads with a dependent child. In comparisons between the fathers the odds ratio (OR) for projective identification was 8.66 to 1, which points to the association between cocaine addiction and the primitive mental functioning of the fathers. With empirical methodology these findings serve to corroborate the psychoanalytical conclusions based on studies of single case studies, testifying that the dysfunctions of identificatory phenomena in familial functioning are predominant in the mental organisation of cocaine addicts.     

年老化对风险决策和模糊决策的影响：来自生理性和病理性老化的证据

随着“全球老龄化”时代的到来, 老年人认知功能的下降引起了研究人员的广泛关注, 其中年老化对决策的影响成为了近年来的一个新关注点。风险决策和模糊决策受生理性老化和病理性老化影响的研究显示, 正常老化个体模糊决策能力受损, 但其风险决策能力受老化影响较小; 而病理性老化个体, 以阿尔兹海默症患者为例, 在两类决策行为上均表现出损伤; 此外, 脑神经机制的研究发现在完成决策任务时正常年老化个体纹状体激活模式与年轻人存在显著差异, 病理性老化个体杏仁核与腹内侧前额叶的功能连接亦存在异常。未来的研究应同时考察并比较两类决策类型, 结合外周和中枢神经证据, 深入探讨老化对决策行为及神经环路造成影响的机制。     

Is the association between childhood maltreatment and aggressive behavior mediated by hostile attribution bias in women? A discordant twin and sibling study

Understanding the mechanisms behind aggressive behavior (AGG) is vital so that effective prevention and intervention strategies can be developed. Maltreated children are hypothesized to be prone to social information processing biases, such as hostile attribution bias (HAB), which, in turn, may increase the likelihood of behaving aggressively. The first aim of the present study was to replicate findings regarding associations between childhood maltreatment (CM), HAB, and aggression in a population‐based sample of Finnish female twins and their sisters (N = 2,167). However, these associations might not be causal but instead confounded by familial factors, shared between the variables. The second aim was, thus, to test the associations when potential confounding by familial (genetic or common environmental) effects were controlled for using a multilevel discordant twin and sibling design within (a) 379 pairs of twins (n_pairs = 239) or siblings (n_pairs = 140), and (b) within the 131 monozygotic (MZ) twin pairs. Consistent with previous studies, HAB mediated the association between CM and AGG when familial confounding was uncontrolled. No support was found for the mediation when controlling for familial confounding. Between‐pair associations were found between CM and AGG, and between CM and HAB. In addition, within‐pair associations were found between HAB and AGG, and between CM and AGG, however, these were nonsignificant in the discordant MZ analysis, offering the most stringent control of familial confounding. The results indicate the necessity of taking familial confounding into account when investigating the development of AGG.     

Worry and Pathological Worry in Patients with Psoriasis: Cross Sectional and Longitudinal Analyses of the Penn State Worry Questionnaire (PSWQ) in Four Samples of Patients

The purpose of the present study was to examine the psychometric properties of the Penn state Worry Questionnaire (PSWQ) in patients with psoriasis. A series of cross-sectional and longitudinal studies were undertaken to assess the reliability, stability and validity of the measure. Patients with psoriasis from four samples (consecutive attendees, anxious patients, depressed patients, and 6-month follow-up of consecutive attendees) completed the PSWQ and measures assessing related constructs of anxiety and depression (HADS) and coping (COPE). The clinical severity of patients psoriasis was also assessed by dermatologists using the Psoriasis Area and Severity Index (PASI). Exploratory factor analysis was undertaken and receiver operator characteristic (ROC) analyses were used to examine the clinical utility of the PSWQ cut-off score for normal and pathological worry. Exploratory factor analysis suggested that the PSWQ is essentially unidimensional in patients with psoriasis. Intraclass correlation demonstrated that, over a 6-month period, the reproducibility of the PSWQ total scale was good in patients with anxiety and moderate in patients with depression. ROC analysis suggested that the optimum cutoff differentiating pathological worry was > 60, which is commensurate with findings in adult mental health more generally. The PSWQ cutoff for pathological worry showed a lower specificity for depression than anxiety. The PSWQ is an appropriate measure of pathological worry in patients with psoriasis. The cutoff on the scale for pathological worry demonstrates appropriate conceptual overlap with subordinate distress categories of anxiety and depression.