Cowden Syndrome

Cowden syndrome is an autosomal dominant inherited cancer syndrome characterized by multiple hamartomas which may develop in any organ. Mucocutaneous papillomas and trichilemmomas are hallmarks of the syndrome. Affected individuals are at high risk of developing both benign and malignant disease of the thyroid and breast. Epithelial thyroid carcinoma occurs in 3–10% of affected individuals while women have a 25–50% lifetime risk of developing adenocarcinoma of the breast. Because of the cancer risk, it is imperative that health care providers recognize Cowden syndrome. The susceptibility gene has been localized to 10q22-23 and preliminary studies demonstrate no genetic heterogeneity.     

The Place and Role of Psychology in Cognitive Science: An International Survey

This paper reports the results of an international survey on the place and role of psychology in cognitive science (CS). The respondents were psychological societies from 31 countries illustrating a variety of situations, from countries where psychology has been established as a science for a long time to countries where it has been established more recently and is still developing. The paper reviews topics covered by the CS approach, as well as research operations and initiatives taken in this domain. The analysis of responses reflects the belief that psychology, in general, has a central role in CS. This is based on epistemological considerations, as well as on the recognition of the methodological expertise of psychology for collecting and analyzing empirical data. Psychology is reported to have a unique integrative capacity and to take care to maintain the place of the human being at the centre of CS. Limitations and obstacles to the development of pluridisciplinary research on cognition are also reported, and information is provided on the training programmes in CS and the institutions that support research in this domain.     

Genes and family environment in familial clustering of cancer

Familial clustering of a disease is defined as the occurrence of the disease within some families in excess of what would be expected from the occurrence in the population. It has been demonstrated for several cancer types, ranging from rare cancers as the adenomatosis-coli-associated colon cancer or the Li-Fraumeni syndrome to more common cancers as breast cancer and colon cancer. Familial clustering, however, is merely an epidemiological pattern, and it does not tell whether genetic or environmental causes or both in combination are responsible for the familial clustering. Familial clustering may be due to genetic predisposition to the disease, but exposure to environmental factors — shared by members of some families, but not by members of other families — may also cause familial clustering and hence mimic genetic inheritance in the study of nuclear families. Based on assumptions regarding the individual steps in the biological process starting with exposure to carcinogens and ending with death from disseminated cancer we suggest that genetic and environmental factors may both be involved in most of these steps. The present paper focuses on research methodologies necessary to discriminate between the effect of genes and family environment in the development of cancer.     

原发性肾病综合征合并甲状腺功能异常患者临床资料分析

分析原发性肾病综合征合并甲状腺功能异常患者的临床特征,探讨其,I盏床意义。对37例原发性肾病综合征合并甲状腺功能异常患者的,陆床资料进行回顾性分析,治疗前检测甲状腺过氧化物酶抗体（Anti～TPO）、甲状腺球蛋白抗体（Anti—TG）,并分别于治疗前后检测血清游离T3（FT3）、血清游离甲状腺激素（FT4）、促甲状腺激...     

Stability of the internal structure of the millon clinical multiaxial inventory

The Millon Clinical Multiaxial Inventory (MCMI) has become increasingly popular in clinical use. Along with this, there has been more interest in the internal structure of the 20 scales of the original 175-item MCMI-I. The literature reports some agreement on four components, although both three- and five-component solutions have been reported. The degree of similarity of these components across populations remains arguable, as none of the previous studies have used quantitative measures of component similarity. The present study reports on two new samples of psychiatric patients, one of 82 cases from a general hospital and the other of 145 inpatients from a psychiatric hospital. It also reanalyzes the data from nine samples from the literature, using Tucker's coefficient of congruence and ten Berge's analysis of principal component weights (PCW). The congruence analyses showed good agreement of the first three components across samples and notably lower agreement for the fourth. The PCW analyses showed two major types of structure matrices. In the first, there was a large and dominant first component, with three smaller ones. In the second, the variance was distributed more evenly across the four components. The results are discussed in terms of the overlapping scales of the MCMI-I.This work was supported by funds from the Departments of Psychiatry and Psychology, The University of Western Ontario.     

Developmental regression in autism spectrum disorders

The occurrence of developmental regression in autism is one of the more puzzling features of this disorder. Although several studies have documented the validity of parental reports of regression using home videos, accumulating data suggest that most children who demonstrate regression also demonstrated previous, subtle, developmental differences. Counter to clinical intuition, the earlier development of social, language, and attachment behaviors followed by regression does not seem to support later recovery of skills or better developmental outcomes compared to children who never had speech or typical social responsivity. In fact, this regressive group may have somewhat greater developmental impairment than the nonregressive group, though the two groups do not appear to present different behavioral phenotypes. Although autism is not the only condition in which regression occurs, it appears to be the most frequent condition. Other disorders that demonstrate an early regression with no known etiology include total blindness from birth and childhood disintegrative disorder, both of which demonstrate behavioral relations to autism. In addition, two biological conditions with known etiologies also involve regression with some behaviors resembling autism behavioral phenotype: Rett syndrome (a genetic disorder; see Glaze, this issue) and Landau-Kleffner syndrome (see McVicar and Shinnar, this issue), which involves a seizure disorder.     

Pavlovian Disgust Conditioning and Generalization: Specificity and Associations With Individual Differences

Although studies have identified differences between fear and disgust conditioning, much less is known about the generalization of conditioned disgust. This is an important gap in the literature given that overgeneralization of conditioned disgust to neutral stimuli may have clinical implications. To address this knowledge gap, female participants (n = 80) completed a Pavlovian conditioning procedure in which one neutral food item (conditioned stimulus; CS+) was followed by disgusting videos of individuals vomiting (unconditioned stimulus; US) and another neutral food item (CS–) was not reinforced with the disgusting video. Following this acquisition phase, there was an extinction phase in which both CSs were presented unreinforced. Importantly, participants also evaluated generalization stimuli (GS+, GS?) that resembled, but were distinct from, the CS after each conditioning phase. As predicted, the CS+ was rated as significantly more disgusting and fear inducing than the CS? after acquisition and this pattern persisted after extinction. However, disgust ratings of the CS+ after acquisition were significantly larger than fear ratings. Participants also rated the GS+ as significantly more disgusting, but not fear inducing, than the GS? after acquisition. However, this effect was not observed after extinction. Disgust proneness did predict a greater increase in disgust and fear ratings of the CS+ relative to the CS? after acquisition and extinction. In contrast, trait anxiety predicted only higher fear ratings to the CS+ relative to the CS? after acquisition and extinction. Disgust proneness nor trait anxiety predicted the greater increase in disgust to the GS+ relative to the GS? after acquisition. These findings suggest that while conditioned disgust can generalize, individual difference variables that predict generalization remain unclear. The implications of these findings for disorders of disgust are discussed.     

The effects of motor neurone disease on language: further evidence

It might sound surprising that Motor Neurone Disease (MND), regarded still by many as the very example of a neurodegenerative disease affecting selectively the motor system and sparing the sensory functions as well as cognition, can have a significant influence on language. In this article we hope to demonstrate that language dysfunction is not only a pronounced and well documented symptom in some MND patients but also that the study of language in MND can address interesting theoretical questions about the representation of language and conceptual knowledge in the brain. After a brief introduction delineating clinical and pathological features of the disease we discuss the evidence available in the literature for language dysfunction in MND. We then present linguistic data from our own study of seven patients with MND/dementia/aphasia syndrome focusing on the dissociation between noun and verb processing. To illustrate the clinical, neuropsychological and linguistic aspects of MND we describe in more detail the patient E.N., a pathologically confirmed case of MND/dementia. Finally, we attempt to characterise the nature of the linguistic impairment in MND in the light of current debates about the mechanisms underlying noun/verb dissociation.     

Sensitive questions in surveys

Psychologists have worried about the distortions introduced into standardized personality measures by social desirability bias. Survey researchers have had similar concerns about the accuracy of survey reports about such topics as illicit drug use, abortion, and sexual behavior. The article reviews the research done by survey methodologists on reporting errors in surveys on sensitive topics, noting parallels and differences from the psychological literature on social desirability. The findings from the survey studies suggest that misreporting about sensitive topics is quite common and that it is largely situational. The extent of misreporting depends on whether the respondent has anything embarrassing to report and on design features of the survey. The survey evidence also indicates that misreporting on sensitive topics is a more or less motivated process in which respondents edit the information they report to avoid embarrassing themselves in the presence of an interviewer or to avoid repercussions from third parties.     

Human classical conditioning. The status of the CS

Recent reports of failure to obtain blocking in human galvanic skin response (GSR) conditioning, together with our own equivocal results with eyelid conditioning, have motivated us to re-examine the status of the conditioned stimulus (CS) in human conditioning studies. The issues raised by compound stimuli, by contextual cues and occasion setting stimuli, and by cross-modal transfer are considered in the light of data from our laboratories. These data include observations on the interchangeability of stimulus modalities during acquisition, the use of varying information loads embedded in occasion setting displays, the comparison of alternative blocking designs and the analysis of response topography in relation to stimulus variability. They suggested that an adequate account of the CS in human conditioning studies must recognize that it is dynamically processed and reprocessed both during and after acquisition.     

Human classical conditioning

Recent reports of failure to obtain blocking in human galvanic skin response (GSR) conditioning, together with our own equivocal results with eyelid conditioning, have motivated us to re-examine the status of the conditioned stimulus (CS) in human conditioning studies. The issues raised by compound stimuli, by contextual cues and occasion setting stimuli, and by cross-modal transfer are considered in the light of data from our laboratories. These data include observations on the interchangeability of stimulus modalities during acquisition, the use of varying information loads embedded in occasion setting displays, the comparison of alternative blocking designs and the analysis of response topography in relation to stimulus variability. They suggested that an adequate account of the CS in human conditioning studies must recognize that it is dynamically processed and reprocessed both during and after acquisition.     

Fearing shades of grey: individual differences in fear responding towards generalisation stimuli

Individual differences in fear generalisation have been proposed to play a role in the aetiology and/or maintenance of anxiety disorders, but few data are available to directly support that claim. The research that is available has focused mostly on generalisation of peripheral and central physiological fear responses. Far less is known about the generalisation of avoidance, the behavioural component of fear. In two experiments, we evaluated how neuroticism, a known vulnerability factor for anxiety, modulates an array of fear responses, including avoidance tendencies, towards generalisation stimuli (GS). Participants underwent differential fear conditioning, in which one conditioned stimulus (CS+) was repeatedly paired with an aversive outcome (shock; unconditioned stimulus, US), whereas another was not (CS?). Fear generalisation was observed across measures in Experiment 1 (US expectancy and evaluative ratings) and Experiment 2 (US expectancy, evaluative ratings, skin conductance, startle responses, safety behaviours), with overall highest responding to the CS+, lowest to the CS? and intermediate responding to the GSs. Neuroticism had very little impact on fear generalisation (but did affect GS recognition rates in Experiment 1), in line with the idea that fear generalisation is largely an adaptive process.     

The Assessment and Treatment of Post-Abortion Syndrome: A Systematic Case Study From Southern Africa

This article reports a clinical case study of “Grace”, a black Zimbabwean woman with post-abortion syndrome (PAS), a form of post-traumatic stress disorder precipitated by aborting an unwanted pregnancy. She was treated by a middle class white South African trainee Clinical Psychologist. The case narrative documents the assessment and the course of treatment which was guided by ongoing case formulation based on current evidence-based models. Factors that made her vulnerable to developing PTSD included active suppression of the memory of the event and lack of social support. An understanding of these factors was used to guide an effective intervention. In spite of the differences in culture and background between client and therapist, there was considerable commonality in their experience as young women and students who each had to balance personal and occupational priorities. The narrative also highlights the commonalities of Grace's experiences with those reported in the literature on post-abortion syndrome, which is mostly from the U. S. A. and Europe.     

Conditioned inhibition of fear: Evidence for a competing response mechanism

In two experiments, inhibitory conditioning was attempted by presenting a discrete CS in a neutral stimulus environment shortly following the termination of either shock (Experiment 1) or a second discrete CS which had been paired in a forward manner with shock (Experiment 2). Evidence of successful inhibitory conditioning was mixed in Experiment 1, where the properties of the CS were assessed within an escape-from-fear procedure. Postresponse presentations of the CS enhanced performance, whereas the presentation of the CS prior to responding did not have the expected degrading effect on performance. In Experiment 2, the inhibitory properties of the CS were assessed by combining this stimulus with an excitatory CS and presenting the compound to rats engaged in a water-reinforced licking response. Less response suppression was found in reaction to this compound relative to three separate comparison conditions, thus witnessing the success of the inhibitory-conditioning procedure used. The common assumption that inhibitory conditioning results from the nonreinforcement of a CS in a situation where reinforcement is expected, i.e., one which contains previously reinforced cues, is not supported by these data, for no previously reinforced cues were simultaneously presented with the CS during inhibitory training. The data are in agreement with a conditioned antagonistic-response interpretation of inhibitory conditioning.     

Breast cancer and cerebral laterality

In reviewing and summarizing work at our institution, we have identified some surprising consistencies in patients with breast cancer. Breast cancer shows four features of cerebral laterality: excess left-handedness, atypical season-of-birth patterns, the risk factor of advanced maternal age, and an association with prenatal sex hormones. Since hypopigmentation also relates to cerebral laterality, a consequence of the association of breast cancer and cerebral laterality is that eye color should be measured as a function of side of disease and age at diagnosis in women with breast cancer.     

Folie à Famille: A Family Therapist's Perspective

Over the last century there have been reports in the psychiatric journals of an unusual disorder, folie ä famille, yet the syndrome has been neglected by family theorists. In this paper the literature on delusions shared by families is examined from the perspective of a family therapist. The role the disorder plays in defining the continuum of functional/dysfunctional shared familial beliefs is described, and a case example demonstrating that folie ä famille may be underreported is presented.     

Segmenting the stream of consciousness: the psychological correlates of temporal structures in the time series data of a continuous performance task

Using principal component analysis, we examined whether structural properties in the time series of response time would identify different mental states during a continuous performance task. We examined whether it was possible to identify regular patterns which were present in blocks classified as lacking controlled processing, either behaviourally (as a failure to withhold a response to a target) or subjectively (as an off task report at a thought probe). Principal component analysis identified three components present in response times accounting for 58.8% of the variance in the data. Of these components, the second largest factor showed two features that implied it was a marker for mind wandering. First, it was stronger under slow relative to fast stimulus presentation conditions, and so paralleled the distribution of mind wandering reports. Second, it was more powerful prior to behaviour markers of mind wandering (failures in response inhibition) and less powerful prior to reports of task focused thinking (on task reports). Taken together, the use of principal components analysis on response times seem a viable tool for differentiating different mental states and so could help identify the neural substrates which underpin mind wandering and other subjective states.     

Measuring the role of conditioning and stimulus generalisation in common fears and worries

Common and persistent fears may emerge through learning mechanisms such as fear conditioning and generalisation. Although there have been extensive studies of these learning processes in healthy but also psychiatric samples, many of the tasks used to produce conditioning and assess generalisation either use painful and aversive stimuli as the unconditioned stimuli (UCS), or suffer from poor belongingness between the conditioned stimuli and the UCS. Here, we present novel data from a paradigm designed to examine fear conditioning and generalisation in healthy individuals. Two female faces served as conditioned threat cue (CS+) and conditioned safety cue (CS?) respectively. The CS+ was paired repeatedly with a fearful, screaming face (unconditioned stimulus). Generalisation included intermediate faces which varied in their similarity to the CS+ and CS?. We measured eyeblink startle reflex and self-reported ratings. Acquired fear of the CS+ generalised to intermediate stimuli in proportion to their perceptual similarity to the CS+. Our findings demonstrate how fears of new individuals may develop based on resemblance to others with whom an individual has had negative experiences. The paradigm offers new opportunities for probing the role of generalisation in the emergence of common and persistent fears.     

Examiner Errors on the WAIS-R: A Source of Concern

Munchausen syndrome (MS) is characterized by patients' chronic and relentless pursuit of medical treatment for combinations of symptoms of consciously self-inflicted injury and falsely reported symptomatology. MS patients are adults, as are perpetrators of Munchausen syndrome by proxy (MSBP). MSBP is an unusual form of child abuse in which a parent, usually the mother, brings a child for medical attention with symptoms falsified or directly induced by the mother. Most reports of MSBP have been of a few cases appearing in medical and pediatric literature. Motivation in both disorders is unclear, and diagnosis and treatment present difficult problems that require more research.     

Developmental prosopagnosia: a review

This article reviews the published literature on developmental prosopagnosia, a condition in which the ability to recognize other persons by facial information alone has never been acquired. Due to the very low incidence of this syndrome, case reports are sparse. We review the available data and suggest assessment strategies for patients suffering from developmental prosopagnosia. It is suggested that developmental prosopagnosia is not a unitary condition but rather consists of different subforms that can be dissociated on the grounds of functional impairments. On the basis of the available evidence, hypotheses about the aetiology of developmental prosopagnosia as well as about the selectivity of deficits related to face recognition are discussed.