Hyperorality in Frontotemporal Dementia: Cognitive and Psychiatric Symptom Profiles in Early-Stage Disease.

Hyperorality is a distinctive feature of the behavioral variant of frontotemporal dementia (bvFTD), but little is known about its significance in early-stage disease. This study examined the cognitive and psychiatric symptom profiles associated with hyperorality, using data from subjects with early-stage bvFTD enrolled in Alzheimer's Disease Research Centers. We found that hyperorality was not associated with cognitive performance, but was associated with psychosis, elation, and disinhibition. Hyperorality may share neurobiology with a subset of early psychiatric symptoms, a finding which could help identify targets for future treatment.

A Meta-Analysis of Neuropsychological Functioning in the Logopenic Variant of Primary Progressive Aphasia: Comparison with the Semantic and Non-Fluent Variants.

OBJECTIVES: The logopenic variant of primary progressive aphasia (lvPPA) has disparate pathological and anatomical features when compared to the semantic (svPPA) and non-fluent (nfvPPA) variants of PPA. As such, there is increasing need for measures that improve diagnostic accuracy particularly when etiology-specific treatments become available. In the current study, we used meta-analytic methods to establish the neuropsychological profile of lvPPA and compare it to recent findings in svPPA and nfvPPA. METHODS: We extracted neuropsychological data from 51 publications representing 663 lvPPA patients and 1379 controls. We calculated Hedges' g effect sizes for nine domains of neuropsychological functioning in lvPPA and assessed the influence of demographic, disease, and task characteristics on effect size magnitude. Results obtained in lvPPA were compared to findings in svPPA and nfvPPA. RESULTS: In lvPPA, the magnitude of deficits in attention, math, visuospatial memory, and executive functioning were as prominent as language deficits. Within the language domain, lvPPA patients demonstrated greater naming than repetition deficits. Compared to svPPA and nfvPPA, lvPPA patients demonstrated greater neuropsychological deficits overall and greater impairment on attention, math, and visual set-shifting tests. CONCLUSIONS: Tests of attention, delayed visuospatial memory, visual set-shifting, and math distinguish lvPPA from svPPA and nfvPPA likely reflecting the posterior temporoparietal atrophy observed early in the course of lvPPA. These findings support the inclusion of these measures in the clinical neuropsychological assessment of lvPPA and underscore the need for additional clinicopathological and longitudinal studies of arithmetic and visuospatial memory across the PPA variants.

A meta-analysis of neuropsychological, social cognitive, and olfactory functioning in the behavioral and language variants of frontotemporal dementia.

BACKGROUND: Though meta-analyses of neuropsychological and social cognitive deficits in behavioral variant frontotemporal dementia (bvFTD) have been conducted, no study has comprehensively characterized and compared the neuropsychological, social cognitive, and olfactory profiles in the behavioral and language variants of FTD. METHODS: Our search yielded 470 publications meeting inclusion criteria representing 11 782 FTD patients and 19 451 controls. For each domain, we calculated Hedges' g effect sizes, which represent the mean difference between the patient and control group divided by the pooled standard deviation. The heterogeneity of these effects was assessed with Cochran's Q-statistic using a random-effects model. Meta-regressions were employed to analyze the influence of demographic and disease characteristics. RESULTS: Though semantic variant primary progressive aphasia patients showed the greatest impairment across all task types, the three groups showed similar cognitive effect sizes once contributions from the language subdomain were excluded from analysis. Contrary to expectation, the magnitude of deficits in executive functioning, social cognition and olfaction were comparable between the three subgroups. Among indices, a metric of executive errors distinguished the behavioral variant of FTD from the language phenotypes. CONCLUSIONS: These data indicate that social cognitive and traditional executive functioning measures may not capture differences between FTD syndromes. These results have important implications for the interpretation of neuropsychological assessments, particularly when applied to the differential diagnosis of FTD. It is hoped that these findings will guide clinical and research assessments and spur new studies focused on improving the measurement of FTD syndromes.

Self-appraisal: Estimates of intellectual performance for persons with Acquired Brain Injury.

BACKGROUND: Acquired Brain Injury (ABI) limits a survivor's ability to appraise their task performance. There are, however, few measures of self-appraisal. OBJECTIVE: This study developed a technique for measuring self-appraisal originally proposed by Wilbur, Wilk, Silver, and Parente (2008). METHODS: A multivariate model of self-appraisal that includes measures of predicted performance as well as measures of over- and under-estimation of performance was evaluated with ABI survivors, participants with diagnosed learning disabilities, and others with emotional impairments to determine which measures were the most sensitive to the differences among the groups. RESULTS: This model provided a more accurate assessment of self-appraisal than the one previously proposed by Wilbur et al. (2008). The two measures of self-appraisal measure different psychological processes, and the overall model measures aspects of performance that are unrelated to an individual's IQ. A measure of over- or under-estimation of performance was the most sensitive component of the model. CONCLUSIONS: Self-appraisal is a multi-dimensional concept, with at least two main components. The findings corroborate previous literature suggesting that persons with ABI have difficulty accurately assessing their task performance resulting in inflated performance judgments. This self-appraisal technique can be applied to most assessments of performance.