Comparability of different Z-score equations for aortic root dimensions in children with Marfan syndrome.

BACKGROUND: Aortic root dilation is a major complication of Marfan syndrome and is one of the most important criteria in establishing the diagnosis. Currently, different echocardiographic nomograms are used to calculate aortic root Z-scores. The aim of the present study was to assess the potential differences in aortic root measurements when aortic root Z-scores were obtained in a cohort of paediatric Marfan patients using several published nomograms. METHODS: In a cohort of 100 children with Marfan syndrome, Z-scores for aortic root dimensions were calculated according to the nomograms of Pettersen et al, Gautier et al, Colan et al, and Lopez et al. Bland-Altman plots were used to estimate mean differences in Z-scores and to establish limits of agreement. RESULTS: The mean Z-score of the sinus of Valsalva for Lopez et al was significantly higher compared to Gautier et al (p < 0.01) and Pettersen et al (p = 0.03). The nomogram of Lopez et al resulted in substantially higher Z-scores in patients with a large sinus of Valsalva diameter. Thirty-five percentage of the studied patients would have a Z-score ≥ 2 using Lopez et al compared to 20% for Pettersen et al, 21% for Gautier et al, and 33% for Colan et al. CONCLUSION: The currently available nomograms for calculating Z-scores of aortic dilation in children with Marfan syndrome lead to clinically relevant differences in Z-scores, especially in children with a relative large aortic root diameter. This could have impact on both the diagnosis and treatment of patients with Marfan syndrome.

Evidence for a relationship between mentalising deficits and paranoia over the psychosis continuum.

OBJECTIVE: Failing of mentalising has been suggested to underlie certain symptoms of psychosis. An as yet unresolved issue is whether mentalising deficits reflect a characteristic which can also be detected in people at risk for psychosis or people with evidence of subclinical expression of psychosis. This study wanted to assess an aspect of mentalising in four groups with different levels of psychosis vulnerability, and to examine associations between mentalising and symptoms of psychosis. METHOD: The study included i) 40 patients with psychosis, ii) 49 non-psychotic first-degree relatives (familial risk), iii) 41 subjects from the general population with a high level of positive psychotic experiences (psychometric risk) and iv) 54 healthy controls. All subjects performed the 'Hinting Task'. RESULTS: There was a significant association between psychosis risk and impairment on the Hinting Task (beta linear trend=0.37, p<0.001). Using the control group as the reference, the association with impairment on the Hinting Task was highest for the patient group (beta=0.46, p<0.001), whereas the familial risk group (beta=0.16, p=0.06) displayed an intermediate probability of failure. The psychometric risk group did not significantly differ from the control group (beta=0.04, p=0.653). In the patient group, impairment on the Hinting Task was associated with current hallucinations and paranoid symptoms. In the familial risk group, there was an association between the Hinting Task and paranoid symptoms. DISCUSSION: These results suggest that vulnerability to psychosis is expressed as an impairment in mentalising, which may have a mediating role in the formation of certain positive symptoms of psychosis.

Verbal self-monitoring in psychosis: a non-replication.

BACKGROUND: Current cognitive models of positive symptoms of psychosis suggest a mechanism of defective self-monitoring that may be relevant for (i) expression of psychosis at the clinical and subclinical level and (ii) transmission of risk for psychosis. METHOD: The study included 41 patients with psychosis, 39 non-psychotic first-degree relatives, 39 subjects from the general population with a high level of positive psychotic experiences, and 52 healthy controls with an average level of positive psychotic experiences. All subjects performed a speech attribution task in which single adjectives with a complimentary or derogatory meaning were presented to them on a computer screen; subjects had to read aloud and determine the source (self/other/uncertain) of the words they heard. In some of the trials, participants' speech was distorted, in others they heard someone else's voice (alien feedback condition) that could also be distorted. RESULTS: No large or significant differences in errors in the speech attribution task were found between the four groups in any of the conditions. CONCLUSIONS: Contrary to previous work using this paradigm, this study found no evidence that either expression of psychosis or risk for psychosis was associated with impairment in self-monitoring.

Impairment of self-monitoring: part of the endophenotypic risk for psychosis.

BACKGROUND: A disorder of self-monitoring may underlie the positive symptoms of psychosis. The cognitive mechanisms associated with these symptoms may also be detectable in individuals at risk of psychosis. AIMS: To investigate (a) whether patients with psychosis show impaired self-monitoring, (b) to what degree this is associated with positive symptoms, and (c) whether this is associated with liability to psychotic symptoms. METHOD: The sample included: individuals with a lifetime history of non-affective psychosis (n=37), a genetically defined risk group (n=41), a psychometrically defined risk group (n=40), and control group (n=49). All participants carried out an action-recognition task. RESULTS: Number of action-recognition errors was associated with psychosis risk (OR linear trend over 3 levels:1.12, 95% CI1.04-1.20) and differential error rate was associated with the degree of delusional ideation in a dose-response fashion (OR linear trend over 3 levels:1.13, 95% CI1.00-1.26). CONCLUSIONS: Alterations in self-monitoring are associated with psychosis with evidence of specificity for delusional ideation. In the risk state, this is expressed more as failure to recognise self-generated actions, whereas in illness failure to recognise alien sources come to the fore.

Data gathering: biased in psychosis?

This study examined whether the probabilistic reasoning bias referred to as a "jumping-to-conclusions" (JTC) style of reasoning, which, according to previous research, is associated with particular psychotic symptoms such as delusions, represents a trait that can also be detected in nonpsychotic relatives of patients with schizophrenia and in nonpsychotic individuals with a high level of psychotic experiences. Participants were, in order of level of psychosis liability, 40 patients with schizophrenia or a schizoaffective disorder, 40 first-degree nonpsychotic relatives, 41 participants from the general population with above average expression of psychotic experiences, and 53 participants from the general population with an average level of psychotic experiences. A "jumping-to-conclusions" bias was assessed using the beads task. A dose-response relationship was found in the association between level of psychosis liability and JTC (defined as needing only a single bead to complete the beads task) (odds ratio [OR] linear trend = 1.59, 95% CI: 1.13-2.24), and, independently, alinear association was apparent between JTC and level of delusional ideation (OR linear trend = 2.59, 95% CI: 1.18-5.69). In addition, the association between psychosis liability and JTC was generally much stronger as the level of delusional ideation was higher. JTC is associated with liability to psychosis (trait), in particular if the psychosis phenotype is characterized by delusional ideation (state).

Neuroticism and low self-esteem as risk factors for psychosis.

BACKGROUND: Low self-esteem and high neuroticism are common features in psychosis, but in the absence of longitudinal studies it is unclear whether they represent consequences of the illness or risk factors acting before illness onset. METHODS: A population sample of 3,929 individuals with no lifetime evidence of psychosis were interviewed with the Composite International Diagnostic Interview and were administered the Groningen Neuroticism Scale and the Rosenberg Self-Esteem Scale at baseline and 1 and 3 years later. At year 3, individuals with CIDI evidence of psychotic symptoms were interviewed by clinicians to identify incident psychotic or psychosis-like symptoms. RESULTS: Baseline neuroticism and self-esteem predicted first-ever onset of psychotic symptoms at year 3 (neuroticism, OR 1.16, 95% CI 1.09, 1.23; self-esteem, OR 1.09, 95% CI 1.01, 1.18).When adjusted for each other and for level of anxiety and depression, neuroticism was the strongest independent predictor for onset of psychotic symptoms (OR 1.16, 95% CI 1.07, 1.26). CONCLUSIONS: Neuroticism increases the risk for development of psychotic symptoms. Mechanisms of risk may involve certain cognitive styles associated with neuroticism, such as beliefs about the uncontrollability of certain events and experiences. The association between low self-esteem and psychosis may involve the area of overlap between self-esteem and neuroticism.