Metacognition Mediates the Relationship Between Maladaptive Personality Traits and Levels of Personality Functioning: A General Investigation on a Nonclinical Sample.

ABSTRACT: The impaired ability to reflect on one's own state of mind and that of others (called metacognition or mentalization) is a central feature of personality disorders (PDs). Metacognition involves different specific abilities, which can be selectively impaired with different PDs and personality maladaptive traits. Moreover, research in the field of PDs has indicated that personality features and metacognitive abilities are associated with the severity of personality pathologies. In this study, we tested a mediation model of the interactions between these variables on predicting levels of personality functioning in a sample of adults taken from the general population (N = 775). Results showed that the relationship between personality traits and personality functioning is partially mediated by metacognitive abilities. These findings support the hypothesis that metacognition plays a significant role in predicting the levels of impairment in personality functioning. These findings have several clinical implications.

Neuro-Behçet's disease presenting as an isolated progressive cognitive and behavioral syndrome.

Behçet's disease is a chronic inflammatory disorder manifesting as a vasculitis that affects arteries and veins of any size. Up to 44% of cases may also present with neurological symptoms, thus defining Neuro-Behçet's disease. We describe a case of Neuro-Behçet's disease characterized by progressive behavioral and cognitive deterioration prevailing over other neurological symptoms, without evident systemic involvement.

The EDSS integration with the Brief International Cognitive Assessment for Multiple Sclerosis and orientation tests.

OBJECTIVE: Despite cognitive tests have been validated in multiple sclerosis (MS), a neuropsychological evaluation is not implemented in the Expanded Disability Status Scale (EDSS) scoring. METHODS: We used the Brief International Cognitive Assessment for Multiple Sclerosis (BICAMS) and orientation tests (OTs) to measure the cerebral functional system (CFS) score and to evaluate its impact on the EDSS. We compared EDSS calculated as usual (Native-EDSS) and after the use of the BICAMS and OT (NPS-EDSS). RESULTS: We tested 604 MS patients with BICAMS, OTs, and EDSS. In all, 384 patients (63.6%) had at least one altered test at the BICAMS. Older age, lower education, higher Native-EDSS, and male gender were independently associated with at least one impaired BICAMS test. Native-EDSS was different from NPS-EDSS (-0.112; p < 0.001) in 99 patients (16%). When considering patients with a Native-EDSS ⩽ 4.0, the proportion of miscalculated EDSS was 25%. CONCLUSION: The use of brief neuropsychological tests leads to a more accurate CFS assessment in two-thirds of MS patients, and a more accurate EDSS calculation in 25% of patients with a score ⩽4.0. This may help clinicians to better recognize cognitive impairment in everyday clinical practice, especially in the case of isolated cognitive worsening.

Exploring Guilt Differences in Grandiose Narcissism, Vulnerable Narcissism, and Malignant Self-Regard.

Narcissistic personality disorder is a heterogeneous and complex pathology which manifests itself very differently in individuals. The aim of the present study was to analyze differences and similarities in morality and sensitivity to feelings of guilt among grandiose narcissism (GN), vulnerable narcissism (VN), and malignant self-regard (MSR). We expected that MSR and VN would be most sensitive to deontological and altruistic guilt, and that MSR and VN would have higher levels of moral standards than GN. A nonclinical sample of 752 participants was evaluated. Results showed a significant association among MSR, VN, and GN. According to our hypothesis, GN turned out to be the one with the lowest association values to guilt measures. Our results demonstrated that MSR is strongly associated with all types of guilt, GN is associated with a substantial lack of guilt, and VN is associated with deontological guilt and self-hate, but not altruistic guilt. Results confirm the relevance of considering and understanding guilt when differentiating GN, VN, and MSR.

Neuropsychological correlates of theory of mind in patients with early Parkinson's disease.

The theory of mind is the ability to attribute mental states to oneself and others and to understand that others have beliefs, desires and intentions different from one's own. The aim of the study was to explore the neuropsychological correlates of theory of mind in patients affected by early Parkinson's disease (PD). Thirty-three PD patients and 33 age-, sex-, and education-matched control subjects underwent the Frontal Assessment Battery, as well as tasks assessing "cognitive" and "affective" theory of mind, and memory abilities; questionnaires evaluating behavioral disorders and quality of life were also administrated. Although the 2 groups did not differ on neuropsychological tasks, PD patients' performance on tasks assessing cognitive and affective theory of mind was significantly worse than controls. Moreover, PD patients had more behavioral disorders and worse quality of life than controls. After covarying for behavioral and quality of life scores, the differences between patients and controls on theory of mind tasks remained significant. "Cognitive" theory of mind was associated with Frontal Assessment Battery score and 2 domains of quality of life scale, whereas "affective" theory of mind scores correlated only with behavioral scales such as the Frontal Behavioral Inventory and Apathy Evaluation Scale. The results demonstrate that both affective and cognitive aspects of theory of mind are simultaneously impaired in early PD and suggest that deficits in the 2 subcomponents of theory of mind may be linked to dysfunction of different frontosubcortical circuitries in early PD.

A progranulin mutation associated with cortico-basal syndrome in an Italian family expressing different phenotypes of fronto-temporal lobar degeneration.

Cortico-basal syndrome (CBS) is a rare neurodegenerative disease characterised by movement and cognitive disorders. It occurs along the spectrum of fronto-temporal lobar degeneration (FTLD), which also includes fronto-temporal dementia (FTD) and progressive supranuclear palsy (PSP). FTLD has recently been shown to be associated with mutations in GRN gene, coding for progranulin, a multifunctional secreted glycoprotein involved in cell cycle, inflammation and tissue repair. We describe the case of a 73-year-old man suffering from CBS with a family history of cognitive disorders belonging to the clinical spectrum of FTLD. Sequencing analysis of GRN in this patient revealed that the C157KfsX97 null mutation has been already described by Le Ber et al. in a French patient affected by an apparently sporadic form of FTD. This report confirms the variability of clinical phenotypes associated with the same mutation and emphasises the importance of genetic analysis in cases with a clear familiarity, as well as in apparently sporadic forms.

Brain-behavior relationship in a case of successful ECT for drug refractory catatonic OCD.

This case report describes the successful response to electroconvulsive therapy (ECT) in a patient with an unusual presentation of catatonia, whose onset occurred in the context of an extremely severe form of refractory obsessive-compulsive disorder (OCD). We correlated the clinical improvement in catatonic and OCD symptoms with specific changes in brain function as shown by regional cerebral blood flow scans, neurological soft signs examination, and neuropsychological testing. All assessments were conducted before and after the ECT course. The results strongly suggest that a right hemisphere dysfunction was the neural correlate of our patient's symptoms, and that ECT, by reverting this abnormality, may serve as an effective therapeutic approach for refractory catatonic OCD.

A common processing system for duration, order and spatial information: evidence from a time estimation task.

The aim of the present study was to verify whether duration, order and space representations share common mechanisms. A two-alternative time estimation paradigm was implemented in two experiments in which subjects had to judge whether the first or the second tone in a pair was shorter (or longer) and to respond by pressing a left or a right key. In Experiment 1, subjects were more accurate in conditions where the first tone was shorter or the second tone was longer, with no effects of spatial information. In Experiment 2, a modification of the paradigm allowed us to demonstrate the presence of a SNARC-like effect, as evidenced by the interaction between order and response key, and of a second-order interaction among duration, order and space. These findings seem consistent with the hypothesis that processing of these three mental categories is subserved by a common mechanism, representing duration and order information according to a spatially-defined magnitude system.

Rehabilitation of gesture imitation: a case study with fMRI.

Acquired disorders of gesture imitation are amenable to treatment, but with poor generalisation toward gestures not included in the training program. We investigated the neural basis of this item-specific recovery in a patient with a slowly progressive posterior cortical atrophy, by means of an fMRI study comparing imitation of rehabilitated and not-rehabilitated gestures. Results suggested that in our patient gesture imitation recruited the mirror system and additional areas relevant to gesture analysis and preparation. Imitation of rehabilitated gestures activated the mirror neuron system, and also left dorsolateral prefrontal cortex and putamen, and the right anterior temporal cortex. This suggests that item-specific recovery was based on interaction of circuitry of imitation with neural systems involved in emotional and motivational processing.

On the genesis of unilateral micrographia of the progressive type.

We report a patient who, following a focal ischemic lesion of the left basal ganglia, developed right hand micrographia characterised by progressive reduction of letter size during writing (progressive micrographia). The patient did not show relevant cognitive impairments, but achieved pathological scores in tests for verbal fluency, and cognitive flexibility and monitoring. A systematic investigation of the writing performances demonstrated that micrographia showed a clear length effect in whatever writing style or task, while it was not observed in drawing, or in left hand writing to a comparable extent. Right hand progressive micrographia was not affected by a concurrent motor and cognitive load; instead, switching between two kinds of allographic responses and presenting one letter at a time in copying tasks reduced severity of micrographia significantly. These findings support the view that progressive micrographia in our patient could be ascribed to a defect in regulating the motor output on the basis of self-generated strategies. This conclusion would be consistent with neuroimaging evidence about the role of the basal ganglia in the control of motor sequencing, and could suggest that progressive micrographia might be associated with specific executive defects.

Autobiographical recall training in elderly adults with subjective memory complaint: a pilot study.

Subjective memory complaint is a self-reported memory impairment which affects elderly people. This problem does not interfere with daily living activities but could decrease quality of life. This study's purpose was to verify whether a specific, newly developed, autobiographical recall training could modify self-perception of memory of subjects with subjective memory complaint. Seven elderly subjects (4 women and 3 men; mean age 65.5 yr., SD=11) with such complaint, evidenced through a specific questionnaire, attended the training course and were prospectively assessed on standard neuropsychological tests, depressive symptomatology, and self-perception of memory. Self-perception of memory, as assessed by scores on a formalized questionnaire, improved significantly after the training, while depressive symptoms did not change. Neuropsychological performances were normal before and after the training, but a statistically significant improvement was observed only on the phonological fluency test. Thus the present pilot study suggested that the training may be effective in improving self-perception of memory and metamnestic capacity in elderly people with subjective memory complaints but not in changing subjects' depressive symptoms. This requires replication of this work with a much larger sample so statistical power is adequate.

Apathy and related executive syndromes in dementia associated with Parkinson's disease and in Alzheimer's disease.

Apathy is defined as a lack of motivation and has been reported to be common in Alzheimer's disease (AD) and Parkinson's disease (PD). To explore the neuropsychological correlates of apathy in patients with PD related dementia (PDD) and AD and to identify the specific cognitive profile of apathy in the two forms of neurodegenerative disease, 61 non-depressed patients (29 PDD and 32 AD) were selected. Out of these, 29 patients (47.5%) were detected as apathetic (14 PDD-A+ and 15 AD-A+), and 32 patients as non-apathetic (15 PDD-A- and 17 AD-A-). All patients underwent cognitive tasks tapping memory, visuospatial and executive functions, behavioral rating scales and Clinical Judgment for Apathy Syndrome (CJ-AS), an inventory developed to measure severity of apathy. The four subgroups differed significantly on memory and frontal tasks. The PDD-A+ performed significantly worse than PDD-A- on frontal tasks. The AD-A+ had poorer performance than AD-A- on frontal tasks. Last, PDD-A+ achieved significantly higher scores than AD-A+ on memory tasks. The four groups differed significantly on CJ-AS and behavioral rating scales.The results showed that apathetic patients with both forms of dementia showed a common neuropsychological and behavioral picture, characterized by defects on frontal tasks, thus strongly supporting the existence of an 'apathetic syndrome', characterized by specific cognitive and psychological symptoms.

The ADAMTS18 gene is responsible for autosomal recessive early onset severe retinal dystrophy.

BACKGROUND: Inherited retinal dystrophies, including Retinitis Pigmentosa and Leber Congenital Amaurosis among others, are a group of genetically heterogeneous disorders that lead to variable degrees of visual deficits. They can be caused by mutations in over 100 genes and there is evidence for the presence of as yet unidentified genes in a significant proportion of patients. We aimed at identifying a novel gene for an autosomal recessive form of early onset severe retinal dystrophy in a patient carrying no previously described mutations in known genes. METHODS: An integrated strategy including homozygosity mapping and whole exome sequencing was used to identify the responsible mutation. Functional tests were performed in the medaka fish (Oryzias latipes) model organism to gain further insight into the pathogenic role of the ADAMTS18 gene in eye and central nervous system (CNS) dysfunction. RESULTS: This study identified, in the analyzed patient, a homozygous missense mutation in the ADAMTS18 gene, which was recently linked to Knobloch syndrome, a rare developmental disorder that affects the eye and the occipital skull. In vivo gene knockdown performed in medaka fish confirmed both that the mutation has a pathogenic role and that the inactivation of this gene has a deleterious effect on photoreceptor cell function. CONCLUSION: This study reveals that mutations in the ADAMTS18 gene can cause a broad phenotypic spectrum of eye disorders and contribute to shed further light on the complexity of retinal diseases.