Predictors of Caregiver Burden in Huntington's Disease.

OBJECTIVE: Huntington's disease (HD) is a genetic neurodegenerative condition that is characterized by cognitive, motor, and psychiatric dysfunction. The purpose of this study was to explore which disease characteristics influence caregiver burden in HD. METHODS: Fifty participants with HD and 50 of their caregivers participated in the study at the University of South Florida. Participants were administered a neuropsychological battery, the Unified Huntington's Disease Rating Scale (UHDRS) motor exam, and the Frontal Systems Behavior Scale (FrSBe) self-report. Caregivers completed the Caregiving Appraisal Scale and the FrSBe family-report. RESULTS: There were significant correlations between caregiver burden and caregiver age and sex, UHDRS motor scores, cognitive functioning, and self and caregiver-reported FrSBe scores. The significant variables were entered into a regression model and explained 63.1% of the variance in caregiver burden scores. Caregiver age, cognitive functioning, and caregiver-reported FrSBe scores continued to be significant predictors of caregiver burden, whereas the other variables were no longer significant. CONCLUSIONS: There were significant relationships between caregiver burden, cognitive functioning, and frontally mediated behaviors, but not motor scores. The results suggest that possible interventions for caregivers may include education to caregivers on how to cope with apathy/executive dysfunction and cognitive decline. Caregiver age was associated with burden, with younger age being associated with increased burden when controlling for symptom severity. This has implications for this population in that HD typically has a younger age of onset than other neurodegenerative diseases and therefore, these caregivers may be particularly at risk for caregiver burden.

Evaluation of a performance-rating method to assess awareness of cognitive functioning in Huntington's disease.

Objective: Anosognosia, or lack of awareness of symptoms, is commonly observed in Huntington's disease (HD) using patient/informant-report discrepancy methods. The purpose of this study was to examine the utility of a performance-rating method for assessing awareness of cognitive performance in HD.Method: Persons with manifest HD (n = 54) rated their performance on the daily living tests from the Neuropsychological Assessment Battery (NAB) using a bell curve. We also examined patient/informant-report discrepancies of executive functioning (FrSBe) in a subset (n = 47) of patients and their informants.Results: Patients were able to reliably use our method of categorizing their performance as evidenced by significant correlations between verbal descriptions of their performance and the percentile ratings they indicated on the bell curve. Patients across the range of observed Unified HD Rating Scale motor scale scores rated their performance on the NAB tasks significantly higher than actual performance for both pretest prediction and posttest evaluation ratings. Only patients with more severe motor impairment underestimated their problems relative to informant report on the FrSBe.Conclusions: This is the first study to use a performance-rating method for examining awareness in HD. Patients could reliably use this performance-based bell curve method to provide accurate estimations of their performance and as a group they tended to overestimate performance as has been demonstrated in previous studies. Unlike previous studies using self-report methodology, we also demonstrated decreased awareness in the persons with HD with less severe motor impairment using the performance-rating method, suggesting the two methods may measure different constructs of awareness.

Awareness of Chorea in Huntington's Disease.

BACKGROUND: Anosognosia, or unawareness of illness of deficits, has been observed in Huntington's disease (HD) in relation to motor and cognitive signs and symptoms. Most studies of awareness in HD have used self-report questionnaire methodology rather than asking patients to report on their symptoms in real-time. The two studies in which patients were asked about their chorea in real-time had small sample sizes and only examined patients early in disease progression. OBJECTIVE: To examine awareness of chorea in real-time in HD patients across a broad range of disease progression. METHODS: Fifty HD patients across motor and cognitive impairment severity were asked if they noticed any involuntary movements after completing a simple working memory task used to elicit chorea. A movement disorders specialist rated the presence or absence of chorea while the patients completed the task. Disagreement between the patient and movement disorders specialist's ratings was considered to be an indicator of unawareness. RESULTS: Approximately 46% of patients who exhibited chorea did not report chorea. Eighty-eight percent of participants who acknowledged chorea did not report chorea in all parts of the body that chorea was observed. CONCLUSIONS: HD patients demonstrate unawareness of chorea across cognitive and motor sign severity.

Taxonomy for Education and Training in Clinical Neuropsychology: past, present, and future.

OBJECTIVE: Historically, the clinical neuropsychology training community has not clearly or consistently defined education or training opportunities. The lack of consistency has limited students' and trainees' ability to accurately assess and compare the intensity of neuropsychology-specific training provided by programs. To address these issues and produce greater 'truth in advertising' across programs, CNS, with SCN's Education Advisory Committee (EAC), ADECN, AITCN, and APPCN constructed a specialty-specific taxonomy, namely, the Taxonomy for Education and Training in Clinical Neuropsychology. The taxonomy provides consensus in the description of training offered by doctoral, internship, and postdoctoral programs, as well as at the post-licensure stage. Although the CNS approved the taxonomy in February 2015, many programs have not adopted its language. Increased awareness of the taxonomy and the reasons behind its development and structure, as well as its potential benefits, are warranted. METHODS: In 2016, a working group of clinical neuropsychologists from the EAC and APPCN, all authors of this manuscript, was created and tasked with disseminating information about the taxonomy. Group members held regular conference calls, leading to the generation of this manuscript. RESULTS: This manuscript is the primary byproduct of the working group. Its purpose is to (1) outline the history behind the development of the taxonomy, (2) detail its structure and utility, (3) address the expected impact of its adoption, and (4) call for its adoption across training programs. CONCLUSIONS: This manuscript outlines the development and structure of the clinical neuropsychology taxonomy and addresses the need for its adoption across training programs.

The Parkinson's Active Living (PAL) Program.

BACKGROUND: Apathy, one of the most common neuropsychiatric symptoms in Parkinson's disease (PD), has been associated with reduced daily functioning, cognition, treatment compliance, quality of life, and increased caregiver burden and distress, among other outcomes. OBJECTIVES: The purpose of the present study was to develop and gather pilot data on the feasibility, acceptability, and efficacy of the Parkinson's Active Living (PAL) program, to our knowledge, the first behavioral treatment specifically designed to target apathy in patients with PD. The Parkinson's Active Living is a primarily telephone-based, 6-week activity scheduling and monitoring intervention that incorporates external cueing to target disease-related self-generational deficits to reduce levels of apathy in nondemented, highly apathetic patients with PD. METHODS: Participants aged 44 to 86 years (mean = 66, SD [standard deviation] = 10.7) ranging in disease duration from <1 to 23 years with elevated apathy (Apathy Evaluation Scale >35) were enrolled in a 1-arm trial and tested at 3 time points (baseline, posttest, and 1-month follow-up). RESULTS: Feasibility aspects (ie, acceptability, demand, implementation, practicality, adaptation, integration, and expansion) and efficacy of PAL program are reported. Matched pairs t tests showed a medium to large effect of treatment on patient apathy (52% showing ≥1 SD improvement), depression (33% showing ≥1 SD improvement), and quality of life at posttest, with improvements in apathy and depression maintained at follow-up. CONCLUSIONS: The program may hold promise as an effective nonpharmacological intervention for apathy in PD. Implications and future directions are discussed. Randomized controlled trials are needed.

Self-reported impulsivity in Huntington's disease patients and relationship to executive dysfunction and reward responsiveness.

INTRODUCTION: Few studies have directly investigated impulsivity in Huntington's disease (HD) despite known changes in dopaminergic and frontal functioning, changes that have been associated with impulsivity in other disorders and in the normal population. This study sought to further categorize impulsivity in HD through examining differences in self-reported impulsivity between community controls and HD patients, the relationship between executive dysfunction and impulsivity, and the relationship of a reward/punishment behavioral inhibition task in relation to these self-report measures. It was expected that HD patients would report higher impulsivity and executive dysfunction and that these measures would relate to a reward/punishment behavioral inhibition task. METHOD: The Barratt Impulsivity Scale (BIS-11) and Behavioral Inhibition/Behavioral Activation Scale (BIS/BAS) were completed, and the Mini-Mental State Examination (MMSE) and a reward-based flanker task with punishing and rewarding conditions were administered to 22 HD patients and 14 control participants. RESULTS: HD patients reported higher trait impulsivity (BIS-11) and executive dysfunction (Frontal Systems Behavior Scale, FrSBE) but not increased impulsivity on the BIS/BAS relative to controls. Higher BIS-11 scores were related to increased self-reported executive dysfunction and the attention/working memory factor of the MMSE. On a reward/punishment behavioral inhibition task, BAS was uniquely related to increased accuracy on rewarding trials of the flanker task, but was not related to punishing trials in HD patients. CONCLUSIONS: The relationships found suggest that trait impulsivity is reported higher in HD and may not be driven by altered reward evaluation and the appetitive nature of stimuli but rather by increased executive dysfunction and lack of sensitivity to punishment. Impulsivity in HD may represent a combination of trait impulsivity, altered dopaminergic circuitry, and executive dysfunction. Understanding impulsivity in HD is important as it is related to increased risk to the patient and difficult behaviors for the caregiver, and sheds light on the disease process.

Examining Huntington's disease patient and informant concordance on frontally mediated behaviors.

INTRODUCTION: Huntington's disease (HD) is a genetic, neurodegenerative disease that affects cognitive, psychiatric and motor functioning. Frontal subcortical circuits are impacted by disease pathology, resulting in frontally mediated behavioral dysfunction. The purpose of this study was (a) to examine the relationship between both patient and informant reports of frontally mediated behaviors and disease progression and (b) to determine rate of agreement between patient and informant reports of these behaviors in relation to disease progression. METHOD: Twenty-six HD patients and their informants participated in the study at the University of South Florida. Patient-informant pairs completed the Frontal Systems Behavior Scale (FrSBe) Self and Family ratings forms, respectively. UHDRS (Unified Huntington's Disease Rating Scale) motor scores were obtained from medical records as an index of disease progression. RESULTS: Only informant report of frontally mediated behaviors of apathy, disinhibition, and executive dysfunction was related to neurological examination results. On average, ratings by patients with less severe motor symptoms were comparable to informant ratings, suggesting intact awareness of deficits. In contrast, ratings of patients with more severe motor symptoms were discrepant from informant data, with informants providing more severe ratings than patients. CONCLUSIONS: HD patients may show intact awareness of frontally mediated behaviors in less severe stages but become increasingly unaware in more severe stages of the disease. This underscores the importance of clinical decisions regarding patient versus informant report at various stages of the disease.

Postdoctoral recruitment in neuropsychology: a review and call for inter-organizational action.

The history of centralized matches for postgraduate selection is briefly discussed with a focus on the match instituted by the Association of Postdoctoral Programs in Clinical Neuropsychology (APPCN) in 2001] Survey data, conducted both by APPCN and independently, are summarized. In general, despite incomplete participation and an estimated 30% rate of "exploding offers", applicants are somewhat satisfied with the match according to recent surveys (although satisfaction varies depending on whether an applicant matched). Given the high rate of withdrawal, the history of other specialties with suboptimal participation, and the concerns most commonly expressed by participants about this issue in survey data, there is cause for concern. We assert that incomplete participation in the match hurts applicants and programs. We propose that focused efforts are needed involving multiple organizations to enhance the match, including the Clinical Neuropsychology Synarchy (CNS) as our specialty council and the multiple organizations represented on the CNS.

Dynamic associations of change in physical activity and change in cognitive function: coordinated analyses of four longitudinal studies.

The present study used a coordinated analyses approach to examine the association of physical activity and cognitive change in four longitudinal studies. A series of multilevel growth models with physical activity included both as a fixed (between-person) and time-varying (within-person) predictor of four domains of cognitive function (reasoning, memory, fluency, and semantic knowledge) was used. Baseline physical activity predicted fluency, reasoning and memory in two studies. However, there was a consistent pattern of positive relationships between time-specific changes in physical activity and time-specific changes in cognition, controlling for expected linear trajectories over time, across all four studies. This pattern was most evident for the domains of reasoning and fluency.

Cognitively Stimulating Activities: Effects on Cognition across Four Studies with up to 21 Years of Longitudinal Data.

Engagement in cognitively stimulating activities has been considered to maintain or strengthen cognitive skills, thereby minimizing age-related cognitive decline. While the idea that there may be a modifiable behavior that could lower risk for cognitive decline is appealing and potentially empowering for older adults, research findings have not consistently supported the beneficial effects of engaging in cognitively stimulating tasks. Using observational studies of naturalistic cognitive activities, we report a series of mixed effects models that include baseline and change in cognitive activity predicting cognitive outcomes over up to 21 years in four longitudinal studies of aging. Consistent evidence was found for cross-sectional relationships between level of cognitive activity and cognitive test performance. Baseline activity at an earlier age did not, however, predict rate of decline later in life, thus not supporting the concept that engaging in cognitive activity at an earlier point in time increases one's ability to mitigate future age-related cognitive decline. In contrast, change in activity was associated with relative change in cognitive performance. Results therefore suggest that change in cognitive activity from one's previous level has at least a transitory association with cognitive performance measured at the same point in time.

Social activity and cognitive functioning over time: a coordinated analysis of four longitudinal studies.

Social activity is typically viewed as part of an engaged lifestyle that may help mitigate the deleterious effects of advanced age on cognitive function. As such, social activity has been examined in relation to cognitive abilities later in life. However, longitudinal evidence for this hypothesis thus far remains inconclusive. The current study sought to clarify the relationship between social activity and cognitive function over time using a coordinated data analysis approach across four longitudinal studies. A series of multilevel growth models with social activity included as a covariate is presented. Four domains of cognitive function were assessed: reasoning, memory, fluency, and semantic knowledge. Results suggest that baseline social activity is related to some, but not all, cognitive functions. Baseline social activity levels failed to predict rate of decline in most cognitive abilities. Changes in social activity were not consistently associated with cognitive functioning. Our findings do not provide consistent evidence that changes in social activity correspond to immediate benefits in cognitive functioning, except perhaps for verbal fluency.

Depressive symptoms in Parkinson disease: degree of association and rate of agreement of clinician-based and self-report measures.

Depression in Parkinson disease (PD) is associated with faster disease progression, lower activities of daily living, and more severe cognitive impairment. Even mild symptoms of depression may impact outcomes in patients with PD. Nevertheless, a low rate of agreement has been reported between patient and clinician ratings of depression, suggesting that clinicians may underestimate depression in patients with PD. However, to accurately compare the rates of agreement, comparable estimates are needed so that patient and clinician ratings have similar meaning (eg, mild, moderate, severe, etc). The purpose of this study was to examine this question by investigating the degree of association and rate of agreement of levels of symptom severity among self-report and clinician ratings using established cutoffs that correspond to more comparable estimates of these levels for both patient and clinician. Our findings suggest that patient's self-report of depressive symptoms was significantly correlated with clinician-based report irrespective of the stage of disease. Moreover, patients demonstrated a 72% rate of agreement with clinicians in classifying symptoms as asymptomatic, mildly symptomatic, or fully symptomatic, a rate significantly higher than the rate of 35% previously reported. This difference in rate of agreement may be accounted for using varying criteria for severity levels across the studies. Findings suggest that clinician and patient reports show a high rate of agreement across a range of depressive symptoms and that self-report measures may provide a relatively efficient means of detecting depressive symptoms especially if patients are disinclined to initiate their report.

The independent influence of apathy and depression on cognitive functioning in Parkinson's disease.

OBJECTIVE: The purpose of the present study was to examine the independent influence of symptoms of depression and apathy, two of the most common neuropsychiatric symptoms in Parkinson's disease (PD), on executive functioning and memory in PD patients using measures designed to discriminate between these symptoms. METHOD: Participants included 68 nondemented, idiopathic PD patients, ages 56-82 years. The Apathy Evaluation Scale-Self-Rating and select items of the Beck Depression Inventory II were used to assess symptoms of apathy and depression, respectively. Cognitive function was assessed using the Wisconsin Card Sorting Test and Hopkins Verbal Learning Test-Revised. Correlations and hierarchical regressions were conducted to investigate the relationships between apathy, depression, and cognitive function. Hierarchical regression analyses were conducted to evaluate the degree of influence of depression and apathy on cognitive function. RESULTS: Results revealed that symptoms of apathy, but not depression, were significantly and negatively associated with executive functioning. Immediate memory was significantly and negatively associated with both apathy and depression. However, apathy accounted for additional variance in memory performance after controlling for depression at a level approaching significance. CONCLUSIONS: Apathy is not only associated with cognitive impairment, but also with impaired daily functioning, caregiver burden and distress, medication noncompliance, and increased mortality. Differentiating apathy and depression, understanding their unique effects, and appropriately identifying apathy symptoms in patients have robust implications for the development of neuropsychological models of these effects in PD as well as practical implications in guiding improvements to patient care and enhancing quality of life in patients and caregivers.

Alteration of expected hemispheric asymmetries: valence and arousal effects in neuropsychological models of emotion.

The relative advantage of the left (LH) over the right hemisphere (RH) in processing of verbal material for most individuals is well established. Nevertheless, several studies have reported the ability of positively and negatively valenced stimuli to enhance and reverse, respectively, the usual LH>RH asymmetry. These studies, however, have used baseline stimuli that differed from emotional stimuli on two dimensions (i.e., valence and verbal/nonverbal nature), creating interpretive difficulties as to whether differences across these conditions are due to differences in valence or the verbal/nonverbal nature of the primes used in the baseline condition. In addition, these studies, along with many others in the literature, have failed to control for potential confounding effects of arousal. Emotional stimuli vary on dimensions of valence as well as arousal and arousal may be asymmetrically presented in the brain therefore contributing to observed asymmetries. Taken together, these considerations underscore the importance of controlling for both valence as well as arousal in any investigation of the effects of emotional stimuli. The objectives of the present study were twofold: (1) to employ an appropriate baseline condition to render emotional stimuli vs. baseline stimuli comparisons meaningful and (2) to examine the extent to which emotional verbal stimuli, equated for arousal level, alter the expected LH>RH asymmetry in a consonant trigram task. Results demonstrated that when LH lateralized consonant trigram presentations were preceded by a positive prime, an enhancement of the expected LH>RH asymmetry was observed. In contrast, when trigram presentations lateralized to the RH were preceded by a negative prime, a complete reversal of the typical asymmetry was found with RH>LH performance. These results are analogous to the pattern of relative hemispheric activations observed for various mood states. Controlling for arousal in studies investigating asymmetries associated with emotional processing may allow more clear interpretation of data intended to test predictions of neuropsychological models of emotion. Moreover, equating stimuli on the dimension of arousal as well as valence may shed more light on conflicting findings with regard to perception vs. expression of emotion.

The lateralized stroop: a meta-analysis and its implications for models of semantic processing.

The prevailing theory in the literature concerning the lateralization of Stroop effects involves a speed of processing account (see ). Because the left hemisphere (LH) demonstrates an overall advantage relative to the right hemisphere (RH) on most verbal tasks, interference effects are hypothesized to be greater in the LH than in the RH. Inspection of the data, however, reveals that many studies find no difference in magnitude of Stroop interference between the hemispheres. Given findings within the lateralized semantic priming literature, this is not surprising. A meta-analysis on a subset of lateralized Stroop experiments was conducted to determine whether or not the LH produces significantly more interference than the RH in this paradigm. Based on a number of exclusionary criteria, a total of 19 different studies were included, representing a potential 26 effect size estimates of differential interference. The effect size representing interference using congruent conditions as the baseline (d+=.06) reveals that there is no significant difference between the hemispheres in magnitude of the Stroop interference effect. The LH interference effect was d+=.73, which is significant. Likewise, the RH interference effect, d+=.67, was significant. In summary, while there was no significant difference between the hemispheres, each hemisphere, when examined independently, did exhibit significant within hemisphere interference effects. These findings are presented in light of the lateralized semantic priming literature.