Reflections on the Gerstmann syndrome.

There has been much debate about whether or not the Gerstmann aggregate is a syndrome in any meaningful sense. Some investigators have marshalled evidence suggesting that the syndrome is an artifact of selective observation and that its components are merely particular expressions of aphasic disorder or mental impairment. Other investigators have attacked the design and logic of these negative studies and have contended that the syndrome is an index of focal brain disease. In this paper, aspects of the early history of the syndrome that are relevant to the question are reviewed, the nature of the components of the syndrome is discussed from a cognitive standpoint, and the findings and logic of recent major studies dealing with the question are analyzed. On this basis, the neuropsychological significance of the syndrome is assessed and some persisting questions are considered.     

Rubinstein-Taybi syndrome.

Rubinstein-Taybi Syndrome was first described in 1957 but only in 1963 as a distinguishable type of moderate mental retardation. The cause is still unknown although some type of genetic origin is possible. No consistent chromosomal abnormality has been demonstrated, the chromosomal analysis is normal, and cases are generally not considered hereditary. The recurrent risk for siblings is approximately 0.1% but may be as high as 50% for offspring of patients with the syndrome. The Rubinstein-Taybi syndrome is not at present detectable before birth and is evenly found in both males and females. The syndrome was thought to be rare but an increase in the number of reported cases each year suggests it is not as rare as estimated. It is thought to occur in about 1 in 300,000 births, and since it has become readily identifiable, more than 400 cases have been reported worldwide.     

Gilles de la Tourette syndrome in India: two cases.

Two cases of Gilles de la Tourette syndrome from India are presented. The symptomatology of Tourette syndrome is the same as that documented in western populations which suggests biological factors in the aetiology of the syndrome.     

Relationships between self-regulation and personality scores of persons with Down syndrome.

This study examined the associations of self-regulation (scores on self-assertion and self control) with personality traits for 76 persons with Down syndrome. Analysis shows self-assertion scores were correlated with scores for all personality traits. The correlations were significant with Emotionality and Playfulness for people with Down syndrome but not for those without Down syndrome (n=40). Self-control scores significantly correlated with scores on controlling and attachment for both groups. Emotionality was related to scores on self-control for students without Down syndrome but not for those with Down syndrome.     

A developmental study of handedness in Down syndrome pupils.

Assessed hand preference in 7- to 9-yr.-old and 13- to 15-yr.-old pupils with Down syndrome (n = 41) was compared with control schoolchildren (n = 50) of the same ages. A significant increase in left-handedness and mixed-handedness was noted in Down syndrome groups compared with the normal population. Younger Down syndrome pupils were less consistent in their hand preference than the older pupils and the normal controls.     

Ethnic Pride and Cardiovascular Health Among Mexican American Adults Along the U.S.-Mexico Border

This study addressed the association between items from the General Acculturation Index (GAI) and cardiovascular health. Specifically, we assessed whether ethnic pride was associated with health outcomes after controlling for items regarding language, place where the childhood was spent and ethnic interaction. The study was a cross sectional analysis of demographic and clinical data from a border population of Mexican American adults (n=316) at risk for cardiovascular disease (CVD). Outcomes included smoking and diabetes status, Framingham risk, and metabolic syndrome. Ethnic pride was associated with lower diabetes prevalence, lower Framingham risk, and fewer risk factors for metabolic syndrome, but was not associated with smoking status. Ethnic pride was not associated with the other acculturation items of the GAI. Among an at-risk border population, ethnic pride functioned independently of other acculturation indicators. Ethnic pride may act as a protective factor for diabetes, metabolic syndrome and CVD risk status.     

Cross-Cultural Examination of Psychopathy Network in Chinese and U.S. Prisoners

Journal of Psychopathology and Behavioral Assessment - Although psychopathic personality has been described as a syndrome comprising three or four distinct personality domains, there is still no...     

Hostility predicts metabolic syndrome risk factors in children and adolescents.

The authors tested in 134 African American and European American children whether hostility measured at study entry predicted the metabolic syndrome risk factors an average of 3 years later. Hostility was measured with the Cook-Medley Hostility Scale (W. W. Cook & D. M. Medley, 1954) and with ratings of Potential for Hostility from interview responses. Metabolic syndrome was based on having at least 2 of the following risk factors above the 75th percentile of scores for their age, race, and gender group: body mass index, insulin resistance index, ratio of triglycerides to high-density lipoprotein cholesterol, and mean arterial blood pressure. Children who exhibited high hostility scores at baseline were likely to exhibit the metabolic syndrome at the follow-up. The results highlight the potential importance of early prevention and intervention of behavioral risk factors for cardiovascular disease.     

Perceived age and sex characteristics of voices of institutionalized children with Down's syndrome.

It was hypothesized that the voices of children with Down's syndrome would be perceived as younger than their true chronological age and that perceptual auditors would have considerable difficulty in identifying the correct sex of these children by voice alone. Perceived age and sex were investigated in 20 institutional children with Down's syndrome who were matched for sex and age with a control group of 20 normal children. Randomized matched verbal samples were played backwards to a group of 16 listener/judges who rated the samples as to age and sex. The results indicated that the voices of these Down syndrome children were perceived as being more than 2 yr. younger than their group mean age and that there was a negative correlation between the biological sex of such children and their perceived sex.     

Effects of COMT genotype on behavioral symptomatology in the 22q11.2 Deletion Syndrome.

The 22q11.2 Deletion Syndrome (DiGeorge/velocardiofacial syndrome) is associated with elevated rates of psychosis, and is also characterized by severe attentional difficulties and executive dysfunction. Behavioral manifestations of this syndrome could result from haploinsufficiency of the catechol-O-methyltransferase (COMT) gene, located within the 22q11 region. The goal of the present study was to examine COMT genotype in relation to behavioral symptomatology in this syndrome. Val158/108Met was genotyped in 38 patients (16 Met/-, 22 Val/-) with confirmed 22q11.2 deletions who had received the Child Behavior Checklist (CBCL) as part of a comprehensive evaluation. Results indicated that the Val genotype was associated with significantly greater internalizing and externalizing behavioral symptomatology in children with 22q11.2 deletions. Val allele status was associated with a greater-than-four-fold increase in risk for clinically significant behavior problems in children with this syndrome. These data are consistent with previous findings of increased psychopathology associated with the Val genotype in normal individuals and suggest that a functional genetic polymorphism in the 22q11 region may influence behavior in individuals with COMT haploinsufficiency.     

Changes in depressive-symptom experiences among older women.

Changes in depressive-symptom experiences over a 5-year period were investigated in a community sample of 251 women between the ages of 51 and 92 years. Findings from a confirmatory factor analysis of the SCL-90-R Depression and Additional Symptoms scales (Derogatis, 1983) indicate that two phenomenally different syndromes underlie symptom-reporting patterns. A depressive syndrome, more classic in form, shows decreasing levels with increasing age, although a depletion syndrome, marked by feelings of enervation and a loss of interest in things, shows increasing levels with advancing age. The relative independence of the two syndromes, as well as their differing relations to four more delimited forms of distress that were identified in the analyses, have important implications for future research on the age-depression relation.     

The Charles Bonnet syndrome: 'phantom visual images'.

The Charles Bonnet syndrome is a condition in which individuals experience complex visual hallucinations without demonstrable psychopathology or disturbance of normal consciousness. An analysis of the sixty-four cases described in the literature reveals that the syndrome can occur at any age though it is more common in elderly people. Reduction in vision, due to peripheral eye pathology as well as pathology within the brain, is associated with the syndrome. Individual hallucinatory episodes can last from a few seconds to most of the day. Episodes can occur for periods of time ranging from days to years, with the hallucinations changing both in frequency and in complexity during this time. The hallucinations may be triggered or stopped by a number of factors which may exert their effect through a general arousal mechanism. People, animals, buildings, and scenery are reported most often. These images may appear static, moving in the visual field, or animated. Emotional reaction to the hallucinations may be positive or negative. Several theories have been proposed to account for the hallucinations. This paper highlights the sensory deprivation framework, with particular emphasis on the activity in the visual system after sensory loss that produces patterns of nerve impulses that, in turn, give rise to visual experience.     

Linguistic profile of individuals with Down syndrome: comparing the linguistic performance of three developmental disorders.

An increasing number of studies, addressing the linguistic abilities of individuals with Down syndrome (DS) suggest that they exhibit strengths and weaknesses within the linguistic domain. This article critically reviews the literature on the linguistic profile of individuals with DS, with particular emphasis on the expression and reception of vocabulary and grammar, including nonverbal linguistic expression during infant development. In doing so, attention is given to recent comparative studies of the linguistic abilities of individuals with DS, Specific Language Impairment (SLI), and Williams syndrome (WS). The possibility that deficits in one cognitive system may have consequences in another cognitive system, and that these consequences may define the nature of the impairment in each clinical syndrome is further discussed with suggestions for future research.     

Habituation in normal and Down's syndrome fetuses.

The ability of ten normal and two Down's syndrome fetuses to habituate to a repeated auditory stimulus was assessed. The normal fetuses were assessed on two separate occasions during the final trimester of pregnancy. The number of trials to habituate and latency to respond were recorded. The results demonstrated that all normal fetuses exhibited a decrement in response to repeated presentations of the stimulus, a 250-Hz sine wave. This was the result of habituation and not fatigue since fetuses recovered responding upon presentation of a new (500 Hz) stimulus and habituated faster on re-presentation of the original stimulus. The performance of fetuses remained stable over the two testing periods. The response of the Down's syndrome fetuses was different to that of normal fetuses rarely overlapping indices of habituation exhibited by normal fetuses. The study concludes that habituation may be a powerful tool to examine the behavioural and sensory development of the fetus and may be of use in the antenatal assessment of the existence and severity of neural abnormality.     

Hostility, the metabolic syndrome, and incident coronary heart disease.

This invesgation examined the impact of hostility and the metabolic syndrome on coronary heart disease (CHD) using prospective data from the Normative Aging Study. Seven hundred seventy-four older, unmedicated men free of cardiovascular disease were included in the study. The total Cook-Medley Hostility (Ho) Scale score, anthropometric data, serum lipids, fasting insulin concentrations, blood pressure, cigarette smoking, alcohol consumption, and total dietary calories were used to predict incident CHD during a 3-year follow-up interval. Multivariate analysis indicated that only Ho positively predicted and high-density lipoprotein cholesterol level negatively predicted incident CHD. Ho's effects on CHD may be mediated though mechanisms other than factors that constitute the metabolic syndrome.     

Metachromatic leukodystrophy and nonverbal learning disability: neuropsychological and neuroradiological findings in heterozygous carriers.

Metachromatic leukodystrophy (MLD) is an autosomal recessive neurodegenerative disorder due to deficiency of the enzyme arylsulfatase A that leads to progressive, diffuse demyelination. The syndrome of nonverbal learning disability has been attributed to white matter abnormality and has been reported in children with this disorder and in some healthy family member carriers of gene. We examined the neuropsychologic profiles and MRIs of eight members of the family of a 7-year-old girl with this disease, all of whom were heterozygous carriers of the mutation and five of whom were also carriers of the MLD pseudodeficiency gene. All had low normal levels of arylsulfatase A, and seven of the eight had average or better profiles across all assessed neuropsychological domains. The patient's younger sister had a profile with features of the syndrome of nonverbal learning disability despite a normal MRI, whereas two members with minor white matter findings did not. This family does not provide evidence for the syndrome of nonverbal learning disability in heterozygous carriers of the gene for MLD, even when associated with the MLD pseudodeficiency gene.     

Socio-communicative deficits in young children with Williams syndrome: performance on the Autism Diagnostic Observation Schedule.

In this investigation, the socio-communicative skills of 29 children with Williams syndrome aged 2 (1/2) to 5 (1/2) years were examined using the Autism Diagnostic Observation Schedule (ADOS) Module 1. Most of the participants showed socio-communicative difficulties. Approximately half of the participants were classified by the ADOS algorithm as "autism spectrum." Three participants were classified "autism." Difficulties with pointing, gestures, giving, showing, and eye contact were present for more than half of the participants, with many also showing difficulties with initiation and response to joint attention and with integration of gaze with other behaviors. Expressive and receptive language abilities of the children with Williams syndrome classified "autism spectrum" were weaker than for children classified nonspectrum, but expressive and receptive language level did not account for the socio-communicative difficulties. Implications for our understanding of the socio-communicative abilities of young children with Williams syndrome and diagnostic practices regarding dual diagnosis are discussed.     

Aphasia syndromes in brain tumors. Clinico-pathological correlations]

A clinica-psychological and pathoanatomical analysis of 40 observations with neoplasms of the brain in the left dominant hemisphere and 4 observations with neoplasms in the right subdominant hemisphere, has been carried out. In 36 cases, a desintegration in the language in a form of aphasia, has been established, while in 4 cases this was not present. In 22 observations the established neuro-psychological syndrome completely indicated and coincided with the localization of the neoplasm in the corresponding part or parts of the brain, in 10 it only partialy coincided, while in 4 cases it did not coincide with the localization of the neoplasm in the brain. The established neuro-psychological syndrome in observations with neoplasms in the right subdominant hemisphere possesses only a tentative topico-diagnostical significance. In conclusion the authors consider that the established syndromes of aphasia in patients with neoplasms of the brain may be of a considerable significance and help in substantiating a precise topical diagnosis.     

Étude du style interactif maternel et des compétences sociocommunicatives des enfants. Une étude comparative entre enfants porteurs de trisomie et enfants typiques

The goal is to explain Down syndrome social language disturbances. Down's syndrome and non-handicapped children were observed interacting with their mothers. We compared 22 dyads with Down syndrome children and 22 dyads with non-handicapped children. Down's syndrome children were matched on mental age with non-handicapped children (4 to 24 months). Social context, mothers' behaviours and children's social communicative competences were evaluated. Interaction behaviours in the mothers of DS children differ from those of mothers of non-handicapped children. DS communicative competences also are different from those in non-handicapped children. We argue that mothers' behavioural interaction in the dyads with DS children can be an aspect of social communicative disturbances of DS children.     

If at first you don't succeed. False hopes of self-change

Despite repeated failure at attempts to change aspects of their behavior, people make frequent attempts at self-change. The generally negative outcome of many such self-change efforts makes it difficult to understand why so many individuals persist at these attempts. The authors have described this cycle of failure and renewed effort as a "false hope syndrome" characterized by unrealistic expectations about the likely speed, amount, ease, and consequences of self-change attempts. In this article, the authors further develop their conceptualization of this syndrome and review its evidential basis. They review the reasons why so many people tend to fail in their self-change attempts and then examine how people interpret these failures in such a way that they are led to keep trying repeatedly despite apparently overwhelming odds. Finally, the authors discuss the psychological consequences of repeated failure and analyze the distinction between confidence and overconfidence.