Objectives: Previous research has demonstrated an association between emotion recognition and apathy in several neurological conditions involving fronto-striatal pathology, including Parkinson’s disease and brain injury. In line with these findings, we aimed to determine whether apathetic participants with early Huntington’s disease (HD) were more impaired on an emotion recognition task compared to non-apathetic participants and healthy controls. Methods: We included 43 participants from the TRACK-HD study who reported apathy on the Problem Behaviours Assessment – short version (PBA-S), 67 participants who reported no apathy, and 107 controls matched for age, sex, and level of education. During their baseline TRACK-HD visit, participants completed a battery of cognitive and psychological tests including an emotion recognition task, the Hospital Depression and Anxiety Scale (HADS) and were assessed on the PBA-S. Results: Compared to the non-apathetic group and the control group, the apathetic group were impaired on the recognition of happy facial expressions, after controlling for depression symptomology on the HADS and general disease progression (Unified Huntington’s Disease Rating Scale total motor score). This was despite no difference between the apathetic and non-apathetic group on overall cognitive functioning assessed by a cognitive composite score. Conclusions: Impairment of the recognition of happy expressions may be part of the clinical picture of apathy in HD. While shared reliance on frontostriatal pathways may broadly explain associations between emotion recognition and apathy found across several patient groups, further work is needed to determine what relationships exist between recognition of specific emotions, distinct subtypes of apathy and underlying neuropathology. (JINS, 2019, 25, 453–461)

Objectives: The cognitive indicators of preclinical behavioral variant Frontotemporal Dementia (bvFTD) have not been identified. To investigate these indicators, we compared cross-sectional performance on a range of cognitive measures in 12 carriers of pathogenic MAPT mutations not meeting diagnostic criteria for bvFTD (i.e., preclinical) versus 32 demographically-matched familial non-carriers (n = 44). Studying preclinical carriers offers a rare glimpse into emergent disease, environmentally and genetically contextualized through comparison to familial controls. Methods: Evaluating personnel blinded to carrier status administered a standardized neuropsychological battery assessing attention, speed, executive function, language, memory, spatial ability, and social cognition. Results from mixed effect modeling were corrected for multiplicity of comparison by the false discovery rate method, and results were considered significant at p < .05. To control for potential interfamilial variation arising from enrollment of six families, family was treated as a random effect, while carrier status, age, gender, and education were treated as fixed effects. Results: Group differences were detected in 17 of 31 cognitive scores and spanned all domains except spatial ability. As hypothesized, carriers performed worse on specific measures of executive function, and social cognition, but also on measures of attention, speed, semantic processing, and memory storage and retrieval. Conclusions: Most notably, group differences arose on measures of memory storage, challenging long-standing ideas about the absence of amnestic features on neuropsychological testing in early bvFTD. Current findings provide important and clinically relevant information about specific measures that may be sensitive to early bvFTD, and advance understanding of neurocognitive changes that occur early in the disease. (JINS, 2019, 25, 184–194)

Objectives: Stroke could lead to deficits in organization of visual search. Cancellation tests are frequently used in standard neuropsychological assessment and appear suitable to measure search organization. The current aim was to evaluate which cognitive functions are associated with cancellation organization measures after stroke. Methods: Stroke patients admitted to inpatient rehabilitation were included in this retrospective study. We performed exploratory factor analyses to explore cognitive domains. A digital shape cancellation test (SC) was administered, and measures of search organization (intersections rate and best r) were computed. The following cognitive functions were measured by neuropsychological testing: neglect (SC, line bisection; LB, Catherine Bergego Scale; CBS, and Balloons Test), visuospatial perception and construction (Rey Complex Figure Test, RCFT), psychomotor speed (Trail Making Test; TMT-A), executive functioning/working memory (TMT-B), spatial planning (Tower Test), rule learning (Brixton Test), short-term auditory memory (Digit Span Forward; DSF), and verbal working memory (Digit Span Backward; DSB). Results: In total, 439 stroke patients were included in our analyses. Four clusters were separated: “Executive functioning” (TMT-A, TMT-B, Brixton Test, and Tower Test), “Verbal memory” (DSF and DSB), “Search organization” (intersections rate and best r), and “Neglect” (CBS, RCFT copy, Balloons Test, SC, and LB). Conclusions: Search organization during cancellation, as measured with intersections rate and best r, seems a distinct cognitive construct compared to existing cognitive domains that are tested during neuropsychological assessment. Administering cancellation tests and analyzing measures of search organization could provide useful additional insights into the visuospatial processes of stroke patients. (JINS, 2018, 24, 424–436)

Objectives: Cognitive dysfunction is a key feature of major depressive (MDD) and bipolar (BD) disorders. However, rather than a single cognitive profile corresponding to each diagnostic categories, recent studies have identified significant intra- and cross-diagnostic variability in patterns of cognitive impairment. The goal of this study was to contribute to the literature on cognitive heterogeneity in mood disorders by identifying cognitive subprofiles in a population of patients with MDD, BD type I, BD type II, and healthy adults. Methods: Participants completed a neuropsychological battery; scores were converted into Z-scores using normative data and submitted to hierarchical cluster analysis. Results: Three distinct neuropsychological clusters were identified: (1) a large cluster containing mostly control participants, as well as some patients with BD and MDD, who performed at above-average levels on all neuropsychological domains; (2) a cluster containing some patients from all diagnostic groups, as well as healthy controls, who performed worse than cluster 1 on most tasks, and showed impairments in motor inhibition and verbal fluency; (3) a cluster containing mostly patients with mood disorders with severe impairments in verbal inhibition and cognitive flexibility. Conclusions: These findings revealed multiple cognitive profiles within diagnostic categories, as well as significant cross-diagnostic overlap, highlighting the importance of developing more specific treatment approaches which consider patients’ demographic and cognitive profiles in addition to their diagnosis. (JINS, 2017, 23, 584–593)

Background: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a recently described life-threatening autoimmune disorder associated with a characteristic multi-stage neuropsychiatric syndrome. Although it is known that the majority of patients experience neuropsychological disturbance post-treatment, some aspects of the cognitive profile remain unclear. Methods: This study sought to investigate patterns of cognitive functioning in a sample of anti-NMDAR encephalitis patients. Seven (6F:1M; mean age, 26.4 years; range, 16–37 years) treated patients completed a comprehensive set of neurocognitive and social functioning measures. Performance was analyzed using normative data (where available), and comparison with matched controls (10F:4M; mean age, 25.8 years; range, 16–38 years). Results: Individual cognitive profiles ranged from within normal limits to extensive dysfunction. Relative to controls, the patient group’s performance was affected in the domains of verbal/ visual memory, working memory, attention, processing speed, executive functioning, and social cognition. The patient group also reported significantly higher levels of anxiety compared to controls. Conclusions: These results add to the accumulating evidence that neurocognitive deficits, consistent with the distribution and functions of the NMDAR system can persist during recovery from anti-NMDAR encephalitis. This is the first study to provide evidence of performance decrements on measures of social cognition, including some involving theory of mind. (JINS, 2016, 22, 828–838)

Naming or word-finding tasks are a mainstay of the typical neuropsychological evaluation, particularly with older adults. However, many older adults have significant visual impairment and there are currently no such word-finding tasks developed for use with older visually impaired populations. This study presents a verbal, non-visual measure of word-finding for use in the evaluation of older adults with possible dysnomia. Stimuli were chosen based on their frequency of usage in everyday spoken language. A 60-item scale was created and given to 131 older Veterans. Rasch analyses were conducted and differential item functioning assessed to eliminate poorly-performing items. The final 55-item scale had a coefficient alpha of 0.84 and correlated with the Neuropsychological Assessment Battery Naming test, r=0.84, p<.01, Delis-Kaplan Executive Function System (D-KEFS) Category Fluency, r=0.45, p<.01, and the D-KEFS Letter Fluency, r=0.40, p<.01. ROC analyses found the measure to have sensitivity of 79% and specificity of 85% for detecting dysnomia. Patients with dysnomia performed worse on the measure than patients with intact word-finding, t(84)=8.2, p<.001. Patients with no cognitive impairment performed significantly better than patients with mild cognitive impairment, who performed significantly better than patients with dementia. This new measure shows promise in the neuropsychological evaluation of word-finding ability in older adults with or without visual impairment. Future directions include the development of a shorter version and the generation of additional normative data. (JINS, 2015, 21, 1–10)

The base rates of abnormal test scores in cognitively normal samples have been a focus of recent research. The goal of the current study is to illustrate how Bayes’ theorem uses these base rates—along with the same base rates in cognitively impaired samples and prevalence rates of cognitive impairment—to yield probability values that are more useful for making judgments about the absence or presence of cognitive impairment. Correlation matrices, means, and standard deviations were obtained from the Wechsler Memory Scale –4th Edition (WMS-IV) Technical and Interpretive Manual and used in Monte Carlo simulations to estimate the base rates of abnormal test scores in the standardization and special groups (mixed clinical) samples. Bayes’ theorem was applied to these estimates to identify probabilities of normal cognition based on the number of abnormal test scores observed. Abnormal scores were common in the standardization sample (65.4% scoring below a scaled score of 7 on at least one subtest) and more common in the mixed clinical sample (85.6% scoring below a scaled score of 7 on at least one subtest). Probabilities varied according to the number of abnormal test scores, base rates of normal cognition, and cutoff scores. The results suggest that interpretation of base rates obtained from cognitively healthy samples must also account for data from cognitively impaired samples. Bayes’ theorem can help neuropsychologists answer questions about the probability that an individual examinee is cognitively healthy based on the number of abnormal test scores observed. (JINS, 2015, 21, 1–9)

Recent reports suggest that cognition is relatively preserved in some schizophrenia patients. However, little is known about the functional advantage these patients may demonstrate. The purpose of this study was to identify cognitively normal patients with a recently developed test battery and to determine the functional benefit of this normality relative to cognitively impaired patients. Average-range cognitive ability was defined by the Measurement and Treatment Research to Improve Cognition in Schizophrenia (MATRICS) Consensus Cognitive Battery (MCCB) composite score (T≥40) and applied to 100 patients with schizophrenia or schizoaffective disorder and to 81 non-psychiatric research participants. With group assignment adjusted for demographic variables, this procedure yielded 14 cognitively normal patients, 21 cognitively impaired patients, and 21 healthy adults with normal-range MCCB scores. Cognitively normal patients were indistinguishable from controls across all MCCB scales. Furthermore, their performance was superior to impaired patients on all scales except Social Cognition. Cognitively normal patients were also superior to impaired patients on a summary index of simulated life skills and functional competence. Nevertheless, both patient groups were equally disadvantaged relative to controls in independent community living. These findings suggest that normal-range cognition exists in schizophrenia, but fails to translate into enhanced community outcome. (JINS, 2014, 20, 1–7)

Background: Globally, falls in older people are a leading cause of injury-related mortality and morbidity. Cognitive impairment is a well-known risk factor for falls in this population group. While there is now a large body of evidence to support effective interventions for falls reduction across care settings, very little is known about interventions in the vulnerable, but increasing population of cognitively impaired community-dwelling older people. Therefore, the purpose of this systematic review is to investigate interventions designed to reduce falls in community-dwelling, cognitively impaired older adults.

Methods: A literature search of databases was conducted to identify original research published in English, which met predefined inclusion and exclusion criteria for effective (non-pharmacological) falls prevention interventions in cognitively impaired community-dwelling people over 65 years of age. Data from the selected papers were extracted into data extraction tables and analyzed according to study characteristics, measures, results, and quality.

Results: The review identified 11 studies providing data from 1,928 participants. Interventions included exercise, health assessment and management of risk, multi-component and cognitive behavioral programs, and hip protectors as falls risk reduction strategies. Seven of the selected studies showed an intervention effect in decreasing falls risk; however, only two of these showed a significant improvement in physical performance measures specifically in a cognitively impaired group.

Conclusions: The diversity of interventions, study designs, populations, and quality of the studies, which met inclusion criteria, resulted in conflicting evidence and inconclusive results for falls prevention interventions in this highly complex population.

The association of cerebral white matter lesions (WMLs) with cognitive status is not well understood in middle-aged individuals. Our aim was to determine the specific contribution of periventricular hyperintensities (PVHs) and deep white matter hyperintensities (DWMHs) to cognitive function in a community sample of asymptomatic participants aged 50 to 65 years. One hundred stroke- and dementia-free adults completed a comprehensive neuropsychological battery and brain MRI protocol. Participants were classified according to PVH and DWMH scores (Fazekas scale). We dichotomized our sample into low grade WMLs (participants without or with mild lesions) and high grade WMLs (participants with moderate or severe lesions). Analyses were performed separately in PVH and DWMH groups. High grade DWMHs were associated with significantly lower scores in executive functioning (−0.45 standard deviations [SD]), attention (−0.42 SD), verbal fluency (−0.68 SD), visual memory (−0.52 SD), visuospatial skills (−0.79 SD), and psychomotor speed (−0.46 SD). Further analyses revealed that high grade DWMHs were also associated with a three- to fourfold increased risk of impaired scores (i.e.,<1.5 SD) in executive functioning, verbal fluency, visuospatial skills, and psychomotor speed. Our findings suggest that only DWMHs, not PVHs, are related to diminished cognitive function in middle-aged individuals. (JINS, 2012, 18, 1–12)

The CAMCOG, ADAS-cog, and MMSE, designed to grade global cognitive ability in dementia have inadequate precision and accuracy in distinguishing mild dementia from normal ageing. Adding neuropsychological tests to their scale might improve precision and accuracy in mild dementia. We, therefore, pooled neuropsychological test-batteries from two memory clinics (ns = 135 and 186) with CAMCOG data from a population study and 2 memory clinics (n = 829) and ADAS-cog data from 3 randomized controlled trials (n = 713) to estimate a common dimension of global cognitive ability using Rasch analysis. Item difficulties and individuals’ global cognitive ability levels were estimated. Difficulties of 57 items (of 64) could be validly estimated. Neuropsychological tests were more difficult than the CAMCOG, ADAS-cog, and MMSE items. Most neuropsychological tests had difficulties in the ability range of normal ageing to mild dementia. Higher than average ability levels were more precisely measured when neuropsychological tests were added to the MMSE than when these were measured with the MMSE alone. Diagnostic accuracy in mild dementia was consistently better after adding neuropsychological tests to the MMSE. We conclude that extending dementia specific instruments with neuropsychological tests improves measurement precision and accuracy of cognitive impairment in mild dementia. (JINS, 2012, 18, 314–322)