Which neuropsychiatric and behavioural features distinguish frontal and temporal variants of frontotemporal dementia from Alzheimer's disease?
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OBJECTIVES: To investigate the prevalence of changes in mood, personality, and behaviour in frontotemporal dementia (FTD) and Alzheimer's disease (AD) and hence, which features reliably distinguish between them. To establish whether the frontal and temporal variants of FTD are characterised by different behavioural changes. METHODS: A questionnaire was designed to assess a wide range of neuropsychiatric changes; it incorporated features reported in previous studies of FTD and components of the neuropsychiatric inventory.(1) This was completed by 37 carers of patients with Alzheimer's disease (AD) and 33 patients with frontotemporal dementia (FTD), comprising 20 with temporal variant FTD (tv FTD) or semantic dementia and 13 with frontal variant FTD (fv FTD). An exploratory principal components factor analysis and discriminant function analysis was applied. RESULTS: Factor analysis showed four robust and meaningful symptom clusters: factor 1-stereotypic and eating behaviour; factor 2-executive dysfunction and self care; factor 3-mood changes; factor 4-loss of social awareness. Only stereotypic and altered eating behaviour and loss of social awareness reliably differentiated AD from FTD with no effect of disease severity. By contrast, executive dysfunction, poor self care, and restlessness showed a significant effect of disease severity only, with the more impaired patients scoring more highly. Changes in mood were found to be equally prevalent in the three patient groups. Analysis of individual symptoms showed increased rates of mental rigidity and depression in the patients with semantic dementia compared with those with fv FTD. Conversely, the latter group showed greater disinhibition. Discriminant function analysis correctly classified 71.4% overall and 86.5% of the patients with AD. CONCLUSIONS: This questionnaire disclosed striking differences between patients with FTD and AD, but only stereotypic behaviour, changes in eating preference, disinhibition, and features of poor social awareness reliably separated the groups. The patients with fv FTD and semantic dementia were behaviourally very similar, reflecting the involvement of a common network, the ventral frontal lobe, temporal pole, and amygdala. Dysexecutive symptoms and poor self care were found to be affected by the severity of the disease, reflecting perhaps spread to dorsolateral prefrontal areas relatively late in the course of both FTD and AD. This questionnaire may be of value in the diagnosis and the monitoring of therapies. (+info)
The Wheels mutation in the mouse causes vascular, hindbrain, and inner ear defects.
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In a screen for mouse mutations with dominant behavioral anomalies, we identified Wheels, a mutation associated with circling and hyperactivity in heterozygotes and embryonic lethality in homozygotes. Mutant Wheels embryos die at E10.5-E11.5 and exhibit a host of morphological anomalies which include growth retardation and anomalies in vascular and hindbrain development. The latter includes perturbation of rhombomeric boundaries as detected by Krox20 and Hoxb1. PECAM-1 staining of embryos revealed normal formation of the primary vascular plexus. However, subsequent stages of branching and remodeling do not proceed normally in the yolk sac and in the embryo proper. To obtain insights into the circling behavior, we examined development of the inner ear by paint-filling of membranous labyrinths of Whl/+ embryos. This analysis revealed smaller posterior and lateral semicircular canal primordia and a delay in the canal fusion process at E12.5. By E13.5, the lateral canal was truncated and the posterior canal was small or absent altogether. Marker analysis revealed an early molecular phenotype in heterozygous embryos characterized by perturbed expression of Bmp4 and Msx1 in prospective lateral and posterior cristae at E11.5. We have constructed a genetic and radiation hybrid map of the centromeric portion of mouse Chromosome 4 across the Wheels region and refined the position of the Wheels locus to the approximately 1.1-cM region between D4Mit104 and D4Mit181. We have placed the locus encoding Epha7, in the Wheels candidate region; however, further analysis showed no mutations in the Epha7-coding region and no detectable changes in mRNA expression pattern. In summary, our findings indicate that Wheels, a gene which is essential for the survival of the embryo, may link diverse processes involved in vascular, hindbrain, and inner ear development. (+info)
Family settings and children's adjustment: differential adjustment within and across families.
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BACKGROUND: Children in stepfamilies and single-parent families exhibit elevated levels of behavioural and emotional problems compared with children in intact (biological) families, but there is variation within and across these family types. AIMS: To examine the sources of variation in children's behavioural and emotional problems across diverse family settings. METHOD: Levels of behavioural and emotional problems in children from diverse stepfamilies and single-parent families were compared with children living with both biological parents. Psychosocial risks were measured at the individual child and family levels. RESULTS: Behavioural and emotional problems were elevated in children in stepmother/complex stepfamilies and single-parent families, but not in simple stepfather families, relative to 'biological' families. Psychopathology associated with family type was explained by compromised quality of the parent-child relationship, parental depression and socio-economic adversity. Sibling similarity in behavioural and emotional problems was most pronounced in high-risk family settings. CONCLUSIONS: Family type is a proxy for exposure to psychosocial risks; the extent of family-wide influence on children's development may be strongest in high-stress settings. (+info)
Emotional and behavioral symptoms in children with acute leukemia.
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BACKGROUND AND OBJECTIVES: The diagnosis of leukemia is probably one of the most severe stressors that children can experience and may be associated with emotional and behavioral symptoms indicating comorbidity with mental health disorders. This study aims to evaluate the presence of emotional and behavioral symptoms in children with acute leukemia exposed to chemotherapy from outpatient services at two university hospitals in Brazil. DESIGN AND METHODS: In this cross-sectional study, emotional and behavioral symptoms were assessed using the Children Behavior Checklist (CBCL) in three groups of children aged 5-14 years: a) children with acute leukemia (n = 21); b) children with blood dyscrasias (n = 21); c) children evaluated or treated in a pediatric outpatient service (n = 33). RESULTS: Children with blood dyscrasias had significantly few symptoms of externalization (delinquent and aggressive behavior) than pediatric controls (p< 0.05). Children with leukemia did not differ from the two other groups regarding symptoms of externalization. No significant difference on the scores of the CBCL internalization dimension (anxiety, depression, somatic symptoms and withdrawn) was found among the three groups. INTERPRETATION AND CONCLUSIONS: These findings seem to indicate that children with acute leukemia do not have more emotional or behavioral symptoms than children with benign hematologic or physical diseases suggesting that comorbidity with mental disorders is not higher in children with acute leukemia than in children in the other two groups. (+info)
Genomewide scan of hoarding in sib pairs in which both sibs have Gilles de la Tourette syndrome.
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A genome scan of the hoarding phenotype (a component of obsessive-compulsive disorder) was conducted on 77 sib pairs collected by the Tourette Syndrome Association International Consortium for Genetics (TSAICG). All sib pairs were concordant for a diagnosis of Gilles de la Tourette syndrome (GTS). However, the analyses reported here were conducted for hoarding as both a dichotomous trait and a quantitative trait. Not all sib pairs in the sample were concordant for hoarding. Standard linkage analyses were performed using GENEHUNTER and Haseman-Elston methods. In addition, novel analyses with a recursive-partitioning technique were employed. Significant allele sharing was observed for both the dichotomous and the quantitative hoarding phenotypes for markers at 4q34-35 (P=.0007), by use of GENEHUNTER, and at 5q35.2-35.3 (P=.000002) and 17q25 (P=.00002), by use of the revisited Haseman-Elston method. The 4q site is in proximity to D4S1625, which was identified by the TSAICG as a region linked to the GTS phenotype. The recursive-partitioning technique examined multiple markers simultaneously. Results suggest joint effects of specific loci on 5q and 4q, with an overall P value of.000003. Although P values were not adjusted for multiple comparison, nearly all were much smaller than the customary significance level of.0001 for genomewide scans. (+info)
Behavioural abnormalities contribute to functional decline in Huntington's disease.
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The independent and relative contributions of motor, cognitive, and behavioural deficits to functional decline in patients with Huntington's disease are examined. Twenty two patients with Huntington's disease were assessed with rating scales for motor dysfunction, cognitive measures of executive functions, and behavioural measures of apathy, executive dysfunction, and disinhibition. Their functional status was assessed with informant based and clinician based ratings of activities of daily living (ADL). A composite apathy/executive dysfunction behavioural index was strongly related to decline in ADL independently and after controlling for motor and cognitive deficits. These results suggest that behavioural dysfunction contributes to functional decline in patients with Huntington's disease and may impede their ability to utilise motor or cognitive skills that remain available in the early stages of the disease. (+info)
Neurobehavioral effects during experimental exposure to 1-octanol and isopropanol.
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OBJECTIVES: The study examined acute neurobehavioral effects provoked by controlled exposure to 1-octanol and isopropanol among male volunteers. METHODS: In a 29-m3 exposure laboratory, 24 male students (mean age 25.8 years) were exposed to 1-octanol and isopropanol. Each substance was used in two concentrations (0.1 and 6.4 ppm for 1-octanol; 34.9 and 189.9 ppm for isopropanol:). In a crossover design, each subject was exposed for 4 hours to the conditions. Twelve subjects reported enhanced chemical sensitivity; the other 12 were age-matched controls. At the onset and end of the exposures neurobehavioral tests were administered and symptoms were rated. RESULTS: At the end of the high and low isopropanol exposures the tiredness ratings were elevated, but no dose-dependence could be confirmed. For both substances and concentrations, the annoyance ratings increased during the exposure, but only for isopropanol did the increase show a dose-response relation. The subjects reported olfactory symptoms during the exposure to the high isopropanol and both 1-octanol concentrations. Isopropanol provoked no sensory irritation, whereas high 1-octanol exposure slightly enhanced it. Only among the subjects with enhanced chemical sensitivity were both 1-octanol concentrations associated with a stronger increase in annoyance, and lower detection rates were observed in a divided attention task. CONCLUSIONS: Previous studies reporting no neurobehavioral effects for isopropanol (up to 400 ppm) were confirmed. The results obtained for 1-octanol lacked dose-dependency, and their evaluation, is difficult. The annoying odor of 1-octanol may mask sensory irritation and prevent subjects with enhanced chemical sensitivity from concentrating on performance in a demanding task. (+info)
Relationship between ethanol-induced changes in brain regional metabolism and its motor, behavioural and cognitive effects.
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AIMS AND METHODS: Acute alcohol administration induces marked decreases in glucose metabolism throughout the human brain. However, the relationship between alcohol's effects on brain metabolism and the behavioural changes that occur with intoxication are still unclear. Here we assessed this association using principal component analysis for dimension reduction and canonical correlations to gauge inter-class relationships. We also used canonical correlations in the polynomial space to assess for possible nonlinear relationships. RESULTS: After normalizing the regional measures to account for the large whole brain decreases observed with intoxication we show that the largest decreases occurred in occipital cortex and that there were relative increases in basal ganglia. Principal component analysis of the changes in the normalized measures revealed that 60% of the variance was accounted for by two factors; one that contrasted cerebellum versus frontal and anterior cingulate metabolism, and another that contrasted basal ganglia and insula. The square of the first factor was significantly correlated with the deterioration in cognitive performance. The second factor showed a significant linear correlation with self-reports of intoxication and with deterioration in cognitive and motor performance. CONCLUSIONS: These findings suggest that the contrasting effects of alcohol in basal ganglia versus the insula are involved in the perception of 'feeling drunk' and that its contrasting effects in cerebellum versus those in frontal and parietal cortices are involved in its motor incoordinating effects. On the other hand alcohol's impact on cognitive performance implicates a more complex pattern of brain effects that includes linear as well as non-linear associations. (+info)