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Erdheim-Chester disease (ECD) is a rare disorder characterized by excessive production and accumulation of histiocytes within multiple tissues and organs. ECD primarily affects adults, and symptoms vary depending upon the specific location and severity. Etiology is not always known, but some patients with ECD may have a non-inherited genetic change that allows histiocytes to reproduce uncontrollably. Currently, the cognitive outcomes of ECD are not well understood, and there are no previous neuropsychological findings in the literature. Thus, the objective of this case study was to describe the neuropsychological presentation and findings of an ECD case to inform diagnosis and treatment better.
Participants and Methods:
The patient was a 64-year-old white, non-Hispanic, right-handed man diagnosed with ECD in 2017. ECD accounted for a constellation of medical problems, including diabetes insipidus, hypogonadism, and interstitial lung disease. A brain MRI in 2018 revealed orbital nodularities and pituitary infiltration thought to be consistent with ECD. The patient first noticed cognitive functioning difficulties in 2020 primarily related to short-term memory. Approximately two years later, he noted significant cognitive changes, including difficulties recalling recent events, dates, and conversations, problem-solving, and planning. He had difficulty driving and had two recent car accidents when leaving his driveway. Physically, he reported increased fatigue, unsteadiness, and occasional falls. In 2022, he had a brain MRI that demonstrated a progression of multiple diffuse cerebral, cerebellum, and brainstem lesions and cerebral volume loss compared to prior imaging. He was referred for a neuropsychological assessment to rule out a neurodegenerative disorder.
Results:
Neuropsychological data demonstrated moderate-to-severe deficits on tests of basic spatial working memory, visually based processing speed, visual memory, letter verbal fluency, and semantic verbal fluency. He demonstrated mild-to-moderate deficits on tests of basic auditory attention, verbal memory, higher-level visuospatial processing, abstract nonverbal reasoning, multistep, organization and planning, self-monitoring of performance quality, and fine hand and motor dexterity. He demonstrated variable initial learning of new information across modalities, although he did benefit from structured verbal material. Recognition was variable, with difficulty demonstrated in discriminating visual information presented, from similar competing information, along with availability recalling visual information. He showed average auditory-based divided attention, confrontational object naming, and abstract verbal reasoning. Regarding his emotional functioning, he reported mild depressive and anxiety symptoms.
Conclusions:
To the best of our knowledge, this is the first report documenting detailed neuropsychological data on this rare disease. The case study documents widespread cognitive deficits with greater difficulty with visually based abilities than verbal abilities. Specifically, patients with ECD may present with cognitive difficulties in visual learning and memory, processing speed, visuospatial processing, select areas of executive/frontal systems, letter and semantic verbal fluency, and fine motor dexterity.
Much of the pediatric concussion literature focuses on sports and recreation related injuries, while there is a relative paucity of research on the cognitive and psychological sequelae associated with assault. However, it is understood that children with assault-related injuries demonstrate a longer recovery time. This case series reviews the data of four teenagers who were administered cognitive, emotional, and behavioral screeners after sustaining an assault related concussion.
Participants and Methods:
Four pediatric female patients (ages 13,14,15,15) with a recent history of concussion due to physical assault presented for evaluation at a hospital-based concussion clinic. All four patients were administered a computerized cognitive screener as well as self-report measures for mood, anxiety, post-traumatic stress disorder (PTSD), and sleep disturbance.
Results:
All four of the pediatric cases reported significant symptoms of PTSD, depression, anxiety, and sleep disturbance. For all four patients, the most notable problems across cognitive performance measures were observed in the areas of simple and complex attention.
Conclusions:
Results of this case series revealed clinically significant anxiety, depression, PTSD-symptoms, and sleep disturbance in conjunction with poor simple and complex attention. These pediatric cases illustrate the potential link between assault-related concussions, emotional and behavioral symptoms, and cognitive functioning. Furthermore, assault-related concussions may elevate risk toward attentional problems associated with psychological distress. Early evaluation of PTSD and mood related symptomatology is suggested to best support treatment planning and recovery in this population. Future studies are needed to explore the nature of the relationship between cognitive and emotional sequelae in patients who present with assault-related concussions.
Individuals living with HIV may experience cognitive difficulties or marked declines known as HIV-Associated Neurocognitive Disorder (HAND). Cognitive difficulties have been associated with worse outcomes for people living with HIV, therefore, accurate cognitive screening and identification is critical. One potentially sensitive marker of cognitive impairment which has been underutilized, is intra-individual variability (IIV). Cognitive IIV is the dispersion of scores across tasks in neuropsychological assessment. In individuals living with HIV, greater cognitive IIV has been associated with cortical atrophy, poorer cognitive functioning, with more rapid declines, and greater difficulties in daily functioning. Studies examining the use of IIV in clinical neuropsychological testing are limited, and few have examined IIV in the context of a single neuropsychological battery designed for culturally diverse or at-risk populations. To address these gaps, this study aimed to examine IIV profiles of individuals living with HIV and who inject drugs, utilizing the Neuropsi, a standardized neuropsychological instrument for Spanish speaking populations.
Participants and Methods:
Spanish speaking adults residing in Puerto Rico (n=90) who are HIV positive and who inject drugs (HIV+I), HIV negative and who inject drugs (HIV-I), HIV positive who do not inject drugs (HIV+), or healthy controls (HC) completed the Neuropsi battery as part of a larger research protocol. The Neuropsi produces 3 index scores representing cognitive domains of memory, attention/memory, and attention/executive functioning. Total battery and within index IIV were calculated by dividing the standard deviation of T-scores by mean performance, resulting in a coefficient of variance (CoV). Group differences on overall test battery mean CoV (OTBMCoV) were investigated. To examine unique profiles of index specific IIV, a cluster analysis was performed for each group.
Results:
Results of a one-way ANOVA indicated significant between group differences on OTBMCoV (F[3,86]=6.54, p<.001). Post-hoc analyses revealed that HIV+I (M=.55, SE=.07, p=.003), HIV-I (M=.50, SE=.03, p=.001), and HIV+ (M=.48, SE=.02, p=.002) had greater OTBMCoV than the HC group (M=.30, SE=.02). To better understand sources of IIV within each group, cluster analysis of index specific IIV was conducted. For the HIV+ group, 3 distinct clusters were extracted: 1. High IIV in attention/memory and attention/executive functioning (n=3, 8%); 2. Elevated memory IIV (n=21, 52%); 3. Low IIV across all indices (n=16, 40%). For the HIV-I group, 2 distinct clusters were extracted: 1. High IIV across all 3 indices (n=7, 24%) and 2. Low IIV across all 3 indices (n=22, 76%). For the HC group, 3 distinct clusters were extracted: 1. Very low IIV across all 3 indices (n=5, 36%); 2. Elevated memory IIV (n=6, 43%); 3. Elevated attention/executive functioning IIV with very low attention/memory and memory IIV (n=3, 21%). Sample size of the HIV+I group was insufficient to extract clusters.
Conclusions:
Current findings support IIV in the Neuropsi test battery as clinically sensitive marker for cognitive impairment in Spanish speaking individuals living with HIV or who inject drugs. Furthermore, the distinct IIV cluster types identified between groups can help to better understand specific sources of variability. Implications for clinical assessment in prognosis and etiological considerations are discussed.
Patients with persistent post-concussion symptoms (PPCS) experience prolonged recovery (e.g., headache, fatigue, or dizziness) lasting >2 months post injury. These symptoms are thought to be maintained by several biopsychosocial factors including dysregulated stress responses, such as pain catastrophizing, that may drive behavioral avoidance and contribute to mood symptoms and cognitive difficulties. Conditions with similar symptomatology to PPCS (e.g., anxiety disorders, somatosensory disorders, chronic pain, etc.) also exhibit maladaptive thought patterns like pain catastrophizing as well as decrements in certain aspects of cognitive performance; however little is known about how pain catastrophizing might relate to neuropsychological performance in youth with PPCS. Therefore, the purpose of this study was to examine the relationship between pain catastrophizing and neuropsychological performance in youth participants with PPCS.
Participants and Methods:
A prospective case-control study design was used to examine 29 participants between the ages of 13 to 23. Participants were divided into two groups: 1) patients with PPCS (2-16 months post-injury; n = 15) and 2) age-matched, non-injured controls (n = 14). Participants completed the Pain Catastrophizing Scale (PCS) to determine degree of catastrophic thinking related to pain experience and the Beck Depression Inventory (BDI). Neuropsychological performance was assessed using the Repeatable Battery for the Assessment of Neuropsychological Status (RBANS) and a modified version of the Paced Auditory Serial Addition Test (PASAT) where performance was evaluated by total correct and error type (i.e., commission and omission) across 5 trials. ANCOVA was used to compare group differences in pain catastrophizing and neuropsychological tests scores while controlling for age and linear regressions examined the relationship between PCS total score and each neuropsychological test score while controlling for level of depression.
Results:
Overall, the PPCS group reported significantly higher levels of pain catastrophizing on the PCS compared to the control group (p < 0.01). For neuropsychological performance, the PPCS group scored significantly lower than the control group on List Learning (p < 0.01), Semantic Fluency (p < 0.05), and List Recall (p < 0.01) on the RBANS and made significantly higher omission errors (but not commission) on the PASAT(p <.01). Higher pain catastrophizing was also associated with poorer neuropsychological performance on the exact same tasks the PPCS group performed worse than controls. There was no significant interaction by group in the impact of PCS scores on neurocognitive performance.
Conclusions:
Compared to controls, youth PPCS patients reported higher levels of pain catastrophizing. Additionally, pain catastrophizing was associated with poorer neuropsychological performance. These findings suggest that increased pain catastrophizing after head injury could contribute to poorer cognitive performance in youth. As such, interventions that target maladaptive cognitive coping styles like pain catastrophizing may be especially helpful for patients with PPCS.
The purpose of the present study is to characterize the trajectory of internalizing and externalizing behaviors in children with NF1 longitudinally from the early childhood period to the school age period on a broad psychosocial norm-referenced measure using linear mixed model growth curves.
Participants and Methods:
Children with NF1 (n=28) were seen at least once between the ages of 3-8 years old and then again between the ages of 9-13 years old. Parents completed the Behavior Assessment System for Children (BASC) Second Edition; the version of the BASC administered depended on age (i.e., preschool form or child form). Linear mixed model growth curve analyses were used to examine the developmental trajectories of children with NF1 on the following scales, which were selected due to findings in the literature: Externalizing Problems, Internalizing Problems, Hyperactivity, Anxiety, Depression, Attention, and Executive Function. T-scores (M=50, SD=10) were used. Higher scores indicate more challenges.
Results:
By using loess lines to qualitatively describe the patterns of ratings across time, it is evident that most scales (Externalizing Problems, Internalizing Problems, Hyperactivity, Attention Problems, Executive Function) demonstrated curvilinear trajectory patterns, with scores peaking in the 8-10-year-old range, then decreasing again. However, there was no statistically significant effect of age on any of the scales. Notably, trajectories largely included standard scores within the normative range (T-scores between 45-55).
Conclusions:
Overall, the models also suggest that most children with NF1 are within the average range of functioning on all scales examined across the childhood period. Furthermore, with the exception of the Depression and Anxiety scales, ratings tend to peak around the 8-10-year period, and then decrease into early adolescence. Thus, when working with patients with NF1, it may be the case that clinicians note relative increases in challenges across these domains in late childhood, though these challenges may decrease over time during this age range. Linear growth curve modeling identified that the developmental trajectories of internalizing and externalizing behaviors of children with NF1, as rated by parents, remain stable across the childhood period. Importantly, low power may have contributed to the lack of observed age effects. Longitudinal research would be beneficial to capture patterns that may emerge in adolescence or adulthood.
This study examined false positive rates of performance validity test (PVT) failure in a group of monolingual (English-speaking) White non-Hispanic/Latinx (non-HL), monolingual (English-speaking) Hispanic/Latinx (HL), and bilingual (English- and Spanish-speaking) HL patients evaluated at an academic medical center. Research on classification accuracy of embedded performance validity tests (PVTs) is limited in HL and bilingual populations. Cultural test biases or language differences could inaccurately cause scores below PVT cutoff levels.
Participants and Methods:
The project involved secondary analysis of a deidentified dataset (N=391). Participants were included if they were between the ages of 18 and 64, had data from the initial visit, had an IQ greater than or equal to 70, were not diagnosed with dementia or major or mild cognitive impairment, and identified as either White non-HL or HL. Participants were required to have completed at least two PVTs. Participants who were not administered the Test of Memory Malingering (TOMM; n=95) or who scored below a highly sensitive Trial 1 cutoff (<46; n=86) were excluded. The final sample included 210 participants, which included monolingual non-HL participants (n=114), monolingual HL participants (n=44), and bilingual HL participants (n=52). Failure rates on eight PVTs were examined by participant group: Reliable Digit Span (RDS), Auditory Verbal Learning Test Recognition (AVLT), Logical Memory Recognition (LM), Visual Reproduction Recognition (VR), Trail Making Test Ratio (TMT rat), Rey Complex Figure Test Recognition (RCFT), Semantic Word Generation (animals; SWG), and Finger Tapping (TAP).
Results:
Groups were not significantly different in age. Monolingual non-HL participants had completed more years of education than monolingual and bilingual HL groups (13.7, 12.7, and 12.8 years respectively). In the whole sample, 8.6% (n=18) failed two or more PVTs. In the monolingual non-HL group, 8.8% (n=10) failed two or more PVTs, while 9.1% (n=4) of the monolingual HL group and 7.7% (n=4) of the bilingual HL group failed two or more PVTs (n.s.). Within the monolingual non-HL test set, failure rates were above 10% on SWG (12.73%) and TAP (17.7%). Failure rates above 10% in the monolingual HL set were found on SWG (11.6%). Failure rates above 10% in bilingual HL measures were observed on SWG (13.5%) and TAP (10.8%).
Conclusions:
Total PVT failure rates did not significantly differ between groups. Across groups, performance was above a common false positive threshold of 10% on SWG. Monolingual non-HL and bilingual participants also had elevated failure rates on TAP.
It has been well established that sustaining a head injury can result in cognitive impairments, but there is little research on the impact of head injuries within middle-aged and older adult samples. Given the two most common samples for head trauma research are athletes and military service members, most of this literature presents findings of individuals under 35 years old. It is important to study head injury outcomes in older samples because greater lengths of time may have passed since the injuries occurred, which may influence findings. Additionally, research indicates that head injuries can lead to measurable executive functioning difficulties - a cognitive domain previously established as susceptible to cognitive ageing-related decline. Therefore, the current investigation seeks to assess the connection between history of head injury and executive functioning performance in middle-aged and older adult participants.
Participants and Methods:
The current study examined 1150 participants from the 2nd wave of the Midlife in the United States cohort (MIDUS) and 801 participants of the MIDUS refresher panel. As a part of the biomarkers study, participants indicated how many head injuries they experienced (up to three) along with whether they were hospitalized and the year the injury occurred. As a part of the cognitive study, participants completed a battery of measures that were combined into a single Z-scored executive functioning measure. Regression was used to evaluate the association between self-reported head trauma and executive functioning, controlling for age and gender. Post hoc analyses examined hospitalization and recency of head injury.
Results:
Of the 1951 included participants, 70.7% reported zero head injuries, 20.8% reported one head injury, 5.8% reported two head injuries, and 2.7% reported at least three head injuries. History of head trauma was not associated with lower levels of executive functioning (F[3,1945]=2.68, p=.38). Furthermore, executive functioning performance was not associated with hospitalization for head injury (b=-.04, p=.31) or recent head injuries (b=-.04, p=.70).
Conclusions:
The current results do not provide evidence of decreased executive functioning performance linked to history of head injury in middle-aged and older adults. These findings are inconsistent with earlier literature suggesting that executive dysfunction is associated with prior head trauma. It is important to consider, however, that the operationalization of executive functioning as a cognitive domain is controversial and produces significant debate. Therefore, the present results solely indicate a lack of connection between previous head trauma and executive functioning, specifically as assessed by the current definition and measures. The present analyses were limited by the broad inclusion of all head injuries rather than a narrowed scope of specifically concussions or mild traumatic brain injuries. However, the study had the advantage of being adequately powered via a large sample size. The current results suggest that additional research is needed within middle-aged and older adult samples to investigate possible connections between head trauma and executive functioning performance using alternate definitions and assessments.
Adaptive emotional regulation strategies, such as cognitive reappraisal, are related to better neuropsychological functioning in the general population. Individuals with PTSD demonstrate difficulty with both emotional regulation and cognitive performance that contribute to clinical presentation (e.g., negative mood, irritability). However the extent to which neuropsychological functioning is associated with emotion regulation, alone and in concert with common comorbid symptoms like depression, remains understudied in this population. Better understanding how specific neuropsychological functions relate to cognitive reappraisal could point to novel treatment targets given preliminary evidence that certain cognitive training techniques can improves neuropsychological and affective outcomes. The present study aims to investigate the relationship between clinical symptoms of PTSD and depression, working memory capacity, and cognitive reappraisal in Veterans with PTSD. We hypothesized that clinical symptoms and working memory capacity would interact to predict cognitive reappraisal, such that elevated depression would relate to worse cognitive reappraisal, particularly for individuals with poor working memory capacity.
Participants and Methods:
Measures of working memory (symmetry span task), mental health symptomatology (Clinician-Administered PTSD Scale for DSM-5 (CAPS-5) and Beck Depression Inventory-II (BDI-II), and emotional regulation (Emotion Regulation Questionnaire (ERQ)) were collected in 38 Veterans. A regression analysis was conducted with total CAPS score, total BDI score, total symmetry span score, and the interaction between BDI score and symmetry span score as the independent variables and total ERQ cognitive reappraisal score as the dependent variable.
Results:
When examining each predictor independently, cognitive reappraisal was not associated with working memory (t=1.42, p=.165), but was significantly associated with PTSD (t=-3.17, p<.003) and depressive (t=-4.15, p<.001) symptom severity. The multiple regression model (F(4, 35)=4.45, p<.005, R2=.26) did not support the hypothesized relationship between working memory and depressive symptoms (t=0.56, p=.579). Depression independently predicted utilization of cognitive reappraisal in veterans with PTSD (t=2.43, p=.020) but PTSD symptom severity and working memory scores did not. However, additional investigation revealed that independently, greater PTSD symptom severity did predict less utilization of cognitive reappraisal (t=-3.17, p<.005).
Conclusions:
While depressive symptoms did not moderate the relationship between working memory and cognitive reappraisal, depressive symptoms did best predict utilization of cognitive reappraisal in a population with PTSD. Contrary to the hypothesized outcome, neither working memory nor PTSD symptoms were significant predictors of cognitive reappraisal when controlling for depressive symptoms. This suggests that the phenomenon of lower cognitive reappraisal rates in individuals with PTSD may be due to the presence of depressive symptoms rather than traumatic stress symptoms or neuropsychological ability. Due to the sample size and homogeneous diagnosis of the current study it is possible that the predicted relationship between working memory, PTSD severity, and cognitive reappraisal was not observed due to low power or a restricted range. Further investigation specifically in clinical populations on factors that predict utilization of cognitive reappraisal could expand on these findings in populations experiencing other stress-based disorders.
Youth with conduct problems (CP) may be more likely to exhibit aggression in the context of peer relations. Indices of dorsolateral prefrontal cortical functioning (DPCF), such as inhibitory control and planning, may be implicated in the behavioral presentation of CP. Further, youth living in violent communities may be more likely to use aggression as a means for problem-solving. However, little is known about how CP interact with DPCF indices to predict aggressive behaviors specifically in the context of environments with high levels of community violence. As such, the present study sought to use a multi-informant approach to examine relations between CP, DCPF, and different types of aggression among youth living in neighborhoods affected by community violence.
Participants and Methods:
Participants were 104 children (M=9.93±1.22 years; 50% male; 96% African American, 4% Latinx) who resided in neighborhoods with elevated rates of poverty and crime. DPCF was indexed by Stockings of Cambridge planning task and caregiver-reported inhibitory control from the Behavior Rating Inventory of Executive Function. Teachers completed the Child and Adolescent Symptom Inventory-4R to assess youth conduct disorder (CD) symptoms and the Teacher Checklist to assess proactive (bullying, planful aggression) and reactive (angry, emotionally labile aggression) aggression.
Results:
Multiple regression analyses were conducted with aggressive behaviors as the dependent variable, and child sex, age, CD symptoms, and the CD × DPCF (i.e., CD × inhibitory control; CD × planning) interaction terms as predictors. Inhibitory control moderated the relation between CD symptoms and reactive (CD × inhibitory control, B = -1.39, p = .004) but not proactive (CD × inhibitory control, B = -.64, p = .063) aggression. Post-hoc probing of the CD symptoms × inhibitory control interaction term predicting reactive aggression revealed a significant slope for youth with both high (B = 1.65, p < .001) and low (B = 4.48, p < .001) levels of inhibitory control. Planning does not significantly moderate the relation between CD symptoms and proactive or reactive aggression, though there is a positive main effect of teacher reported CD symptoms on both proactive (B = 2.44, p < .001) and reactive (B = 2.36, p < .001) aggression.
Conclusions:
This study is the first to consider the relations of CP, DPCF processes, and subtypes of aggression among youth living in environments with high rates of community violence. Among youth with CP, high levels of inhibitory control may be associated with lower rates of reactive aggression in the context of peer relations. As such, problem-solving interventions targeting processes related to DPCF, such as inhibitory control, may be useful among youth living in neighborhoods high in community violence who exhibit CP. Strengthening these cognitive processes may result in better behavioral outcomes and peer relations.
Approximately 70,000 people were displaced from Greece to Turkey and the Middle East during World War II. Following a presentation of the geography, statistics, and timeframe of the displacement, and Turkey’s interwar demographic policies, the article studies Turkey’s management of this refugee movement. Based on Greek, Turkish, and British archival material, the article argues that Turkish wartime refugee policy took shape in the intersection of two occasionally contradictory attributes. On the one hand, there were the state’s demographic desiderata and policies that differentiated incoming refugees on account of their ethnic and religious identities. On the other, Turkey’s reaction was necessarily conditioned by the military, political, and diplomatic conjuncture of the war. It was this conditionality that explains the inconsistences and shifts in refugee management and its rationale during the war, for instance, the differentiation in the treatment of Jewish and Christian refugees, but also in the management of Greek Muslims in 1941–1942 and in 1944–1945.
Japanese-Americans are the sixth largest Asian ethnicity in the United States. They represent a highly heterogeneous population due to their history of immigration dating back to the late 19th century. In comparison to the total Japanese-American population, there are very few neuropsychologists of Japanese descent who are familiar with the culture. The Asian Neuropsychological Association lists 16 licensed members of Japanese descent, and only 7 practice outside of Hawaii or California. These numbers suggest that unless non-Japanese neuropsychologists are knowledgeable of the culture, test translations, and appropriate norms, it would be challenging for many Japanese-Americans to receive culturally and linguistically competent neuropsychological services. The aim of the present study is to provide guidance for conducting neuropsychological assessments with Japanese-Americans with the goal of facilitating competent culturally-informed services to this population.
Participants and Methods:
Pertinent facets of Japanese culture as identified in the ECLECTIC framework and demographics of the U.S. Japanese-American population, and the available literature on neuropsychological tests that are translated into Japanese and normed with Japanese samples, was reviewed by authors with knowledge of Japanese language and culture. Literature published in both English and Japanese were included for review.
Results:
Psychological testing is a Western technology fraught with the behavioral expectations and values of the culture in which it was developed. Thus, these tests may be biased against persons coming from cultures that differ from the West. Recommendations for providing neuropsychological services to Japanese-Americans are presented with an aim of maximizing test fairness by addressing the following issues: comfort with the testing situation, test biases, accessibility, and validity. Given the emphasis on education, Japanese-Americans should be familiar and comfortable with cognitive testing, although they may experience undue pressure to perform well to avoid shame. Japanese-Americans may experience discomfort disclosing personal information during the interview, particularly if the evaluation is perceived to be psychiatric in nature, as there is a strong stigma associated with mental illness that could bring shame to the family. Japanese communication styles are indirect in nature, where the message is implied and what is “not said” is just as important as what is directly conveyed. Accessibility issues will primarily impact first generation Japanese-Americans who are native Japanese speakers. Another characteristic that may impact responding is hesitancy for guessing when unsure. Recommendations for providing culturally competent neuropsychological assessment given these considerations will be presented and expanded upon in detail. Finally, an online database of translated and normed neuropsychological tests by cognitive domain has been created and will be presented.
Conclusions:
Providing neuropsychological services to Japanese-Americans can be challenging as Japanese culture is significantly different from western culture. In addition, Japanese-Americans are heterogenous with salient issues of English proficiency and acculturation. Information to individualize a conceptual understanding of Japanese-Americans, translated and normed tests, and recommendations to maximize fairness in testing are presented to assist clinical neuropsychologists provide competent services to Japanese-Americans.
Findings from cognitive screenings have resulted in lower-than-expected scores amongst late-career physicians (Moutier et al., 2013). Similar to healthy aging samples and those with mild cognitive impairment, inconsistencies in self-report and objective neuropsychological functioning have been noted in physicians (Nasreddine et al., 2005). Little research has focused on neuropsychological functioning of other highly educated groups, including PhD and JD degrees. We addressed a lack of normative cognitive performance data for populations with advanced degrees by exploring cognitive test scores in a mixed clinical sample of adults.
Participants and Methods:
Archival data are from 208 neuropsychology clinic outpatients with 20 years of education (Mage=67.7, SDage=12.3; 25% female; 95% White). Academic degrees were PhD (35.6%), JD (28.4%), MD/DO (21.6%), and 6% other. Referrals sources were physicians (93.8%), licensing boards/employers (3.8%), self-referrals (1.4%), and attorneys (1.0%). Employment status was 55.3% employed and 44.7% not employed. Final DSM-5 neurocognitive diagnosis (NCD) status was: no NCD (45.2%), mild NCD (35.6%), and major NCD (19.2%). Etiologies were: possible Alzheimer’s disease (41.2%), unspecified (13.2%), and possible vascular (12.3%). Chi-square tests denoted diagnostic status differences between degree type and employment status. ANOVAs denoted differences in global cognitive and intellectual functioning (on the Repeatable Battery for Neuropsychological Status [RBANS] Total Index, Weschler Adult Intelligence Scale-IV (WAIS-IV), Weschler Abbreviated Scale of Intelligence-II [WASI-II] FSIQ-4 and FSIQ-2) between degree types. Cumulative frequency rates for low scores in the entire sample on normally distributed tests of general intellectual and cognitive functioning were computed for -1.0, -1.5, -2.0, and -2.5 standard deviations (SDs) at or below the population mean.
Results:
NCD diagnosis did not differ by degree (X2[14]=8.73, p=.848) but did differ by employment status (x2[2]=40.98, p<.001, cp=0.44). Employment rate was highest for the no NCD group (66.0%), followed by mild NCD (37.8%), and major NCD (7.5%). For cases below retirement age (<65 years), employment status did not significantly differ between NCD diagnostic groups (x2[2]=5.97, p=.050). Low scores on an FSIQ measure were: -1 SD (7.0%), -1.5 SD (2.6%), -2.0 SD (0.9%), and -2.5 SD (0.0%) compared to general cognitive test scores which demonstrated 42.5% at -1 SD, 30.5% at -1.5 SD, 19.0% at -2.0 SD, and 9.2% at -2.5 SD below the population mean.
Conclusions:
The high-education literature is limited to medical degree samples. This sample included multiple degree types. Unsurprisingly, employment rates were higher for healthy versus impaired samples; however, employment rates were similar across these groups for people below retirement age. Our findings suggest that cognitively impaired people with 20 years of education often perform at or near the general population average on tests of general intellectual functioning but below the general population average on tests of general cognitive functioning. Future work should include base rates of low scores on a broader array of cognitive tests across diagnostic groups.
Periventricular nodular heterotopia (PVNH) is a malformation of cortical development (MCD) characterized by aggregates of gray matter adjacent to the lateral ventricular walls. Clinical presentation is heterogeneous with higher rates of seizures and reading impairments typically in the setting of average IQ (Felker et al. 2011). The majority of neuropsychological inquiry has focused on reading fluency, though a single case study showed a neurocognitive profile consistent with nonverbal learning deficits in a 7-year old boy with suspected autosomal dominant bilateral heterotopia (McCann et al., 2008). Given the periventricular focus and potential for unilateral presentations, non-linguistic neurocognitive sequelae may be expected in cases affecting the non-dominant hemisphere, yet this remains largely unexplored. Surgical ablation by laser interstitial thermal therapy (LITT) is increasingly used for epilepsy management in PVNH (Thompson et al., 2016, Whiting et al. 2020). However, there are no reported studies exploring cognitive outcomes following LITT of focal PVNH.
Participants and Methods:
A 46-year-old, right handed, Black female with 16 years of education presented for management of medically refractory epilepsy. Epilepsy monitoring captured intermittent slow waves in the right temporal lobe, interictal polyspikes in the right temporal lobe, and three epileptic events all emanating from the right temporal lobe. MRI showed extensive migrational anomalies involving the right hemisphere posteriorly consistent with PVNH with no associated mesial temporal sclerosis. Pre-surgical neuropsychological evaluation showed a significant split in IQ, with verbal IQ = 99 and performance IQ = 76. Testing indicated circumscribed deficits in visuoperceptual judgement, visuoconstruction, visuospatial reasoning, non-verbal recall, and several executive weaknesses in the context of otherwise average neurocognitive functioning. Fine motor speed was impaired bilaterally. Her profile was suggestive of non-dominant fronto-parieto-temporal dysfunction, concordant with the remainder of her work-up. By history she reported longstanding academic weaknesses in math and organization with strong verbal and reading abilities.
Results:
The patient underwent partial LITT of right PVNH sparing areas involved in visual function. At 6-month follow-up she was seizure free (Engel outcome 1A). Post-surgical neuropsychological evaluation showed reliable improvements in perceptual reasoning, aspects of learning/memory, and verbal naming. Visuoconstruction remained impaired but qualitatively improved. She also reported subjective experience of improved mental clarity and was applying for jobs after regaining driving privileges.
Conclusions:
This case demonstrates symptoms and history concerning for a nonverbal learning disorder in an adult woman with epilepsy secondary to right hemisphere PVNH and underscores the importance of exploring the range of neurocognitive profiles in MCD. Her notable neurocognitive and functional improvements following surgical ablation and seizure freedom suggest a possible release of function in the absence of inhibitory neurophysiological influences. This raises interesting questions about the endurance of her developmental profile. This case report contributes to our understanding of neuroanatomical correlates of neurocognitive and neurodevelopmental presentations. Future investigations should explore neuropsychological changes following LITT for PVNH.
There is equivocal evidence that acculturation is associated with cognition. Various factors may contribute to ambiguous findings in the neuropsychology setting, including psychometric limitations of tools available for assessing acculturation as well as the frequent conflation of bilingualism with acculturation. Additionally, neuropsychological research on acculturation and cognition has largely failed to account for bidimensional models of acculturation, which have greater empirical support over unidimensional models. In response to these limitations and the theoretical literature on acculturation, we developed the Perceived Identity & Ethnicity Scales (PIES), a brief rating scale system to quickly (<2 minutes) capture an individual's perceived acculturative identity across several domains. In the current study, we sought to (1) provide initial psychometric support of the PIES and (2) examine how it relates to cognition in a culturally and linguistically diverse sample.
Participants and Methods:
We recruited 242 individuals from both university and community samples (age=23.7±7.6, range 18-72; <12 Years of Education=4%; 78% Female; 58% Hispanic/Latin American; 69% middle SES; 22% educated outside the USA). In addition to demographic questionnaires, participants completed the PIES; an established measure of acculturation (the Bicultural Involvement Questionnaire, BIQ) and bilingualism (Bilingual Language Profile, BLP); measures of mood (the Depression, Anxiety, Stress Scales, DASS; Apathy Evaluation Scale, AES); and of self-reported cognitive functioning (Everyday Cognition, ECog). A subsample of Spanish speakers (n=86) also completed a cognitive battery validated for use in this population (the Spanish English Neuropsychological Assessment Scales, SENAS). For the first aim, we examined the reliability, validity, and dimensionality of the PIES in the full sample. In the Spanish-speaking subsample, we examined the relationship between the tool and both subjective and objective cognition using linear regression controlling for age, education, sex/gender, and premorbid intellectual functioning.
Results:
Measures of internal consistency and dimensionality supported a bidimensional model of acculturation; identification with culture of family origin (PIES-O) was not related to identification with US American culture (PIES-U; r=0.036, p>0.05). Cultural preference scores from the BIQ were associated with PIES-O (r=-0.322) and PIES-U (r=0.277; both ps<0.001) in the expected directions. PIES-O (r=0.350) and PIES-U (r=-0.432) were associated with the ability to speak a language other than English on the BLP (both ps<0.001). PIES-U, but not PIES-O, was also strongly associated with other BIQ and BLP scores as well as with receiving education outside of the USA at medium to large effect sizes (rs=0.3 to 0.6; all ps<0.001). In the subsample, PIES-O and PIES-U were not associated with subjective cognition as measured by the ECog (AR2=0.016, p>0.05); global cognition as measured by the Montreal Cognitive Assessment (MoCA; AR2=0.046, p>0.05); or SENAS cognitive composite scores (AR2=0.016, p>0.05) after controlling for covariates.
Conclusions:
Findings provide strong initial psychometric support for the utility of the PIES in the assessment of acculturation. Moreover, these results further support the bidimensional model of acculturation. Acculturation as measured by the PIES was not associated with cognitive abilities in this highly educated and mainly female cross-sectional sample. Longitudinal research accounting for acculturation is needed to elucidate these relationships.
Traumatic brain injury (TBI), very common in the United States (US) and occurring at highest rates in older adulthood, is a documented risk factor for cognitive impairment and dementia. However, the full scope of the problem is unknown, as comparative incidence of TBI among older adults is poorly characterized. Moreover, the effect of demographics (race/ethnicity, sex) and cognitive and medical status, as well as education, socioeconomic status, and other social determinants of health (SDOH) on TBI risk is not well understood. We aimed to explore the impact of demographics, cognitive and medical status, and SDOH on vulnerability to new TBIs among older adults.
Participants and Methods:
Enrollees 65 and older in the nationally representative Health and Retirement Study (HRS) who consented to have their survey data linked to Medicare claims and had not experienced a head injury prior to HRS enrollment were studied. We used claims data 2000-2018 to obtain incident TBI diagnoses and harnessed the detailed demographic, cognitive, medical, and SDOH information available in the HRS. Incident TBI was defined using inpatient and outpatient International Classification of Disease (ICD 9 and 10) codes received the same day as an emergency room (ER) visit code and a computed tomography (CT) scan code, occurring after the enrollee’s baseline HRS interview. We calculated descriptive statistics and bivariate associations for TBI status with demographic and SDOH characteristics measured at baseline using sample weights to account for the complex survey design.
Results:
Of respondents meeting inclusion criteria (n=9273) during the study follow-up period of 18 years, 8.9% received emergency room treatment for a TBI. Older adults who experienced TBI during the study period were more likely to be female (p=0.0006), and white (p=0.0001), to have normal cognition (vs. cognitive impairment or dementia, p=0.0011), higher education (p<0.0001), and higher income (p=0.01). Having lung disease (p=0.0003) or functional impairment (p=0.03) at baseline were protective against experiencing a TBI.
Conclusions:
Our results suggest that almost 9% of US older adults received ER treatment for a new TBI during the 18-year study period, and that race, sex, and SDOH factors may increase risk for, or be protective against, TBI. This novel investigation into the impact of demographics and SDOH on incident TBI suggests access to care may impact who gets treatment for TBI. Further study is indicated and may lead to opportunities for both targeted intervention (e.g., primary TBI prevention) to groups most at risk as well as identification and mollification of the most relevant structural and contextual factors (e.g., access to care) to reduce risk of TBI among older adults.
As word finding or "naming" impairment is a symptom of multiple neurological conditions, naming assessment is an integral component of most neuropsychological evaluations. For decades, the Boston Naming Test (BNT) has been, and remains, the most widely used measure of naming. Although it has been shown that naming is generally stable from young adulthood through middle age, we have observed, clinically, that young adults tend to have greater difficulty on the BNT than older adults. Considering that the BNT was developed more than 50 years ago, and that language and culture change over time, we hypothesized that 1) increasing age would be associated with stronger performance on the BNT, whereas 2) there would be no association between age and naming performance on more recently developed naming measures.
Participants and Methods:
Participants were healthy adults who served as normative subjects in the revision study of the Auditory (ANT) and Visual Naming (VNT) Tests. Due to known effects of education level on BNT performance, we excluded those with less than 16 years of education, targeting young adults through middle age, resulting in 118 adults, 20 through 50 years of age (mean age: 32.9 ± 9.2 years; mean education: 16.8 ± 1.2 years; mean FSIQ: 106.0 ± 12. 6). All participants were native English speakers or learned English by age 5 and were fully educated in English. Untimed accuracy (i.e., response within 20 seconds) is the standard performance measure for the BNT; the ANT and VNT additionally include tip-of-the-tongue (TOT) scores, which incorporate response time and reliance on phonemic cueing (TOT score = number of items named in > 2 seconds but < 20 seconds, plus items named correctly after 20 seconds, following a phonemic cue). Pearson correlations examined the relation between age and naming performance on the BNT, ANT and VNT.
Results:
Pearson correlations revealed a small but significant, positive correlation between age and BNT performance (r =.22, p = .017), yet no correlation between age and performance on the ANT (ANT Accuracy: r = .05, p=.60, ANT-TOT: r = -.14, p = .12) or VNT (VNT Accuracy: r = .04, p=.67, VNT-TOT; r = -.03, p = .72).
Conclusions:
In this sample of healthy adults, naming performance improved with increasing age on the BNT; however, while vocabulary knowledge may broaden, naming efficiency should not improve with age. By contrast, we found no relation between age and naming performance on the more recently developed ANT and VNT. Results underscore the need to revise test stimuli on verbal measures, particularly those that assess naming, and suggest caution in interpreting BNT performance in young adults, as poor BNT performance might not accurately represent their true naming ability.
Multiple sclerosis (MS) has historically been considered a syndrome that primarily affects White persons of northern European ancestry. This has been strongly disproven in recent decades with prevalence/incidence studies showing that MS impacts individuals from diverse backgrounds. The few studies available investigating clinical characteristics of MS across diverse groups have shown that Hispanic/Latinx/e (Latinx) and non-Hispanic Black/African American (NHB) persons with MS (pwMS) have more severe disease trajectories compared to non-Hispanic Whites (NHW), including an earlier age of disease onset, greater disability, and more severe symptoms overall. Changes in brain structure have been linked outcomes and MS-itself, but what remains understudied is how brain structure differs across race/ethnicity. As such, the current study aims to investigate volumetric brain differences in a diverse sample of pwMS.
Participants and Methods:
The sample (n=79) was compiled from multiple neuroimaging datasets and divided into three groups- Latinx (n=19), NHB (n=29), and NHW (n=32)- based on self-reported race/ethnicity. Participants completed demographic interviews and structural magnetic resonance imaging (MRI) scans. Neuroimaging data was visually inspected and processed in FreeSurfer (7.3.2). Volumetric measures for total gray matter, cortical gray matter, total white matter, and subcortical gray matter were used as the primary outcome measures.
Results:
A multivariate general linear model was used to examine volumetric brain differences across groups. Age and total intracranial volume were included as covariates. Results showed a significant effect of race/ethnicity (Pillai’s Trace=0.175, F(6, 148)=2.36, p=.033), indicating significant differences in volumetric brain metrics across race/ethnicity, namely for subcortical gray matter, total gray matter, and total white matter volumes. Post-hoc testing showed the Latinx group to have less subcortical gray matter, total gray matter, and total white matter than NHWs. There was a trend for the NHB versus NHW, with NHBs having less brain volume. No significant differences were observed between the Latinx and NHB groups. Lesion volume and regional gray matter volumes were also examined.
Conclusions:
To the authors’ knowledge, this is among the first studies to investigate structural brain differences across race/ethnicity in pwMS. Results point to disparities in brain volume across racial/ethnic groups with MS. These differences may partially underlie the differing trajectories observed in clinical characteristics across race/ethnicity. Future studies should include larger samples of diverse pwMS and examine the intersection of psychosocial and systemic factors (i.e., social determinants of health) and brain metrics to better understand the divergent disease trajectories observed across groups.
The COVID-19 pandemic has been a catalyst for a shift towards virtual forms of working, and specific support for the provision of virtual therapy (BPS, 2020). Remotely delivered therapy has known potential benefits, such as improving access to people living in underserved areas and for those who struggle to attend face-to-face appointments due to psychosocial, physical or geographical constraints. Those with neurological conditions frequently fall in these groups.
Acceptance and Commitment Therapy (ACT) is an evidence-based psychological intervention which aims to improve the physical and mental health of people living with neurological conditions. However, there are several considerations to translating this into a virtual therapy for this clinical group that have not yet been thoroughly empirically evaluated. Such considerations include: fatigue, cognitive difficulties in the context of virtual communication, adaptations within the therapeutic alliance, and translation of dynamic and interactive therapeutic exercises within a virtual medium.
This study aimed to describe the experiences of Clinical Psychologists delivering ACT remotely in the context of two randomised controlled feasibility and acceptability clinical trials for young people (aged 11 to 24) who have experienced a brain tumour or Tuberous Sclerosis Complex. Exploring therapist perceptions and experiences provides valuable insights into potential barriers and facilitators to engagement with remote ACT delivery.
Participants and Methods:
Clinical Psychologists who were trained to deliver ACT via videoconferencing were invited to take part in virtual interviews. The semi-structured interview guide aimed to draw on their experience of ACT training, supervision, remote delivery, and perceived impact of ACT. Interviews were audio-recorded, transcribed verbatim and analysed using inductive thematic analysis.
Results:
Five Clinical Psychologists were interviewed. Interview data were coded into broad themes around the experience of delivering ACT, engaging with remote systems, the wider perceived impact of ACT and the adaptability of ACT. ACT training was reported to be an effective, positive experience and the opportunity for regular peer and group supervision worked to solidify the training and support fidelity to the ACT processes. It was largely acknowledged that while remote delivery of the intervention did create some initial challenges to the therapeutic process, this approach created opportunity for the young people to access psychological support that they might not have been able to engage with due to logistical considerations. Clinical Psychologists also shared that training in and experience of delivering ACT had a positive and meaningful impact on their clinical practice and personal conduct. They also reported that ACT was adaptable to the needs of the young people in the clinical trials and felt appropriate to deliver in this context.
Conclusions:
This in-depth, qualitative study demonstrated the clinical promise of remotely delivered ACT. Clinical Psychologists viewed remotely delivered ACT as an appropriate, acceptable and feasible intervention for young people with experience of brain tumour and Tuberous Sclerosis Complex within the context of research, that would be generalisable to everyday clinical practice.
Interpretation of neuropsychological (NP) tests depends on the quality of the normative standards available for the tests. Co-norming across tests is necessary when interpreting differences between scores on different tests. The relevance of specific norms for an individual examinee further depends on multiple design features of the standardization studies, including: when the studies were conducted, sampling strategy, inclusion/exclusion criteria, age, sex/gender, education, race and ethnicity, socioeconomic status, and region. This paper examines the standardization studies of the most widely used NP tests, identifies their strengths and weaknesses, and makes recommendations for interpretive caveats based on these analyses.
Participants and Methods:
We reviewed the standardization strategies and coded information about the sampling frames, inclusion/exclusion criteria, stratification methods, demographic characteristics, and sample sizes overall and within each stratum where relevant. These methods were applied to the WAIS-IV, WMS-IV, CVLT3, D-KEFS, Pearson Advanced Clinical Solutions (ACS), Rey Complex Figure Test, WCST, Symbol Digit Modalities Test, RBANS, BVMT-R, HVLT, Halstead-Reitan (“Heaton et al”) Norms for Boston Naming, Finger Tapping, Grooved Pegboard), MOANS, and MOAANS (Boston Naming, Trail Making Test, Judgement of Line Orientation). We calculated multiple indexes for each test, including standard errors and confidence intervals for scaled scores.
Results:
Most tests used age only as a stratification factor, providing “age corrected” scores for selected age bands. The sample sizes for the age strata range from 1 to ∼200 but were usually less than 100 participants/stratum. Sex differences were rarely reported and some studies had markedly uneven distributions of sex. Education was not used as a stratification factor in any study, and few norms attempted corrections for education. The possible interactions of age and education on test scores are seldom reported and cell sizes for combinations of age and education may be too small to enable robust estimates of scores, especially at lower levels of education and older ages. The possible impact of race and ethnicity are rarely interrogated except in ACS, Heaton and MOAANS norms, which all focus on “African American” participants. Discrepancies in scores across ACS, Heaton and MOAANS suggest marked sampling differences.
Conclusions:
Existing norms have major limitations which may impact the clinical assessment of individuals and result in inappropriate treatment recommendations as well as lead to inappropriate classification in clinical trials, which may include score “cutoffs” based on widely used normative standards. Most norms use only age as a stratification factor, despite robust impacts of education on scores. Race and ethnicity are poorly represented, fail to reflect current demographic characteristics of the United States, and existing norms present major conflicts for African American groups, with the same raw scores differing by a full standard deviation depending only on the source of normative data. Sex differences are examined infrequently and it remains unclear to what extent sex or gender differences may affect some scores. There is an urgent need for new, preferably “dynamic” normative standards, that include sampling by socially and demographically meaningful metrics, to provide greater precision in assessment of neuropsychological scores and score discrepancies, and for evaluating the inclusion/exclusion criteria, and criteria for efficacy in clinical trials that use neurocognitive endpoints.
Studies suggest that internally oriented cognitive processes are central to creativity. Here, we distinguish between intentional and unintentional forms of mind wandering and explore their component contributions to creativity. Furthermore, we describe resting-state connectivity profiles associated with these trait-level measures of mind wandering. Bearing in mind the role of the DMN in internally directed cognitive processes, we hypothesize that, in general, mind wandering will be associated with increased connectivity of voxels in the medial prefrontal and posterior cingulate cortices, key nodes in the DMN. We further hypothesize that intentional and unintentional mind wandering will be associated with distinct patterns of connectivity across DMN core regions.
Participants and Methods:
We used a sample of 155 healthy adults from the mind-brain-body dataset, all of whom completed resting-state fMRI scans and trait-level measures of mind wandering. We analyzed intentional and unintentional mind wandering tendencies using self-report measures. Next, we explored the relationship between mind wandering tendencies and creativity, as measured by a divergent thinking task. Additionally, we describe patterns of resting-state network connectivity associated with mind wandering, using graph theory (weighted degree) functional connectivity analysis.
Results:
At the behavioral level, results showed a significant positive association between creativity and both intentional and unintentional mind wandering. Neuroimaging analysis revealed higher weighted degree connectivity associated with both forms of mind wandering, implicating core regions of the default network and the left temporal pole. We observed topological connectivity differences within the default network: intentional mind wandering was associated with degree connectivity in posterior regions, whereas unintentional mind wandering showed greater involvement of prefrontal areas.
Conclusions:
In this study, we highlight patterns of resting-state network connectivity associated with intentional and unintentional mind wandering, and provide novel evidence of a link between mind wandering and creativity. These findings represent a promising step towards understanding the neurocognitive mechanisms that underlie productive mind wandering and demonstrate its relevance for the study of creative thinking.