Tele-Neuropsychology: From Science to Policy to Practice.

OBJECTIVE: The primary aim of this paper is to accelerate the number of randomized experimental studies of the reliability and validity in-home tele-neuropsychological testing (tele-np-t). METHOD: We conducted a critical review of the tele-neuropsychology literature. We discuss this research in the context of the United States' public and private healthcare payer systems, including the Centers for Medicare & Medicaid Services (CMS) and Current Procedural Terminology (CPT) coding system's telehealth lists, and existing disparities in healthcare access. RESULTS: The number of tele-np publications has been stagnant since the onset of the COVID-19 pandemic. There are less published experimental studies of tele-neuropsychology (tele-np), and particularly in-home tele-np-t, than other tele-np publications. There is strong foundational evidence of the acceptability, feasibility, and reliability of tele-np-t, but relatively few studies of the reliability and validity of in-home tele-np-t using randomization methodology. CONCLUSIONS: More studies of the reliability and validity of in-home tele-np-t using randomization methodology are necessary to support inclusion of tele-np-t codes on the CMS and CPT telehealth lists, and subsequently, the integration and delivery of in-home tele-np-t services across providers and institutions. These actions are needed to maintain equitable reimbursement of in-home tele-np-t services and address the widespread disparities in healthcare access.

Integrating Lifestyle Factor Science into Neuropsychological Practice: A National Academy of Neuropsychology Education Paper.

OBJECTIVE: The primary aim of this paper is to review evidence and clinical implications related to lifestyle activities associated with promoting brain and cognitive health. Our review targets four key lifestyle factors: physical activity and exercise, social engagement, cognitively stimulating activity, and consuming Mediterranean-style diets. METHOD: We conducted a critical review of the lifestyle factor literature in the four domains listed earlier. We contextualize this literature review by translating findings, when possible, into evidence-based recommendations to consider when providing neuropsychological services. RESULTS: There is significant current evidence supporting the role of physical activity and exercise, social engagement, cognitively stimulating activity, and consuming Mediterranean-style diets on positive brain and cognitive health outcomes. While some null findings are present in all four areas reviewed, the weight of the evidence supports the notion that engaging in these activities may promote brain and cognitive functioning. CONCLUSIONS: Clinical neuropsychologists can have confidence in recommending engagement in physical activity, social activity, and cognitively stimulating activity, and adhering to a Mediterranean-style diet to promote brain and cognitive health. We discuss limitations in existing lifestyle factor research and future directions to enhance the existing evidence base, including additional research with historically underrepresented groups and individuals with neurological conditions.

Leveraging expertise and optimizing clinical research: Initial success of a pediatric epilepsy surgery collaborative.

OBJECTIVE: Improve data-driven research to inform clinical decision-making with pediatric epilepsy surgery patients by expanding the Pediatric Epilepsy Research Consortium Epilepsy Surgery (PERC-Surgery) Workgroup to include neuropsychological data. This article reports on the process and initial success of this effort and characterizes the cognitive functioning of the largest multi-site pediatric epilepsy surgery cohort in the United States. METHODS: Pediatric neuropsychologists from 18 institutions completed surveys regarding neuropsychological practice and the impact of involvement in the collaborative. Neuropsychological data were entered through an online database. Descriptive analyses examined the survey responses and cognitive functioning of the cohort. Statistical analyses examined which patients were evaluated and if composite scores differed by domain, demographics, measures used, or epilepsy characteristics. RESULTS: Positive impact of participation was evident by attendance, survey responses, and the neuropsychological data entry of 534 presurgical epilepsy patients. This cohort, ages 6 months to 21 years, were majority White and non-Hispanic, and more likely to have private insurance. Mean intelligence quotient (IQ) scores were below to low average, with weaknesses in working memory and processing speed. Full-scale IQ (FSIQ) was lowest for patients with younger age at seizure onset, daily seizures, and magnetic resonance imaging (MRI) abnormalities. SIGNIFICANCE: We established a collaborative network and fundamental infrastructure to address questions outlined by the Epilepsy Research Benchmarks. There is a wide range in the age and IQ of patients considered for pediatric epilepsy surgery, yet it appears that social determinants of health impact access to care. Consistent with other national cohorts, this US cohort has a downward shift in IQ associated with seizure severity.

Longitudinal Clinical and Biological Characteristics in Juvenile-Onset Huntington's Disease.

BACKGROUND: Juvenile-onset Huntington's disease (JOHD) is a rare form of Huntington's disease (HD) characterized by symptom onset before the age of 21 years. Observational data in this cohort is lacking. OBJECTIVES: Quantify measures of disease progression for use in clinical trials of patients with JOHD. METHODS: Participants who received a motor diagnosis of HD before the age of 21 were included in the Kids-JOHD study. The comparator group consisted of children and young adults who were at-risk for inheriting the genetic mutation that causes HD, but who were found to have a CAG repeat in the non-expanded range (gene non-expanded [GNE]). RESULTS: Data were obtained between March 17, 2006, and February 13, 2020. There were 26 JOHD participants and 78 GNE participants who were comparable on age (16.03 vs. 14.43, respectively) and sex (53.8% female vs. 57.7% female, respectively). The mean annualized decrease in striatal volume in the JOHD group was -3.99% compared to -0.06% in the GNE (mean difference [MD], -3.93%; 95% confidence intervals [CI], [-4.98 to -2.80], FDR < 0.0001). The mean increase in the Unified Huntington's Disease Rating Scale Total Motor Score per year in the JOHD group was 7.29 points compared to a mean decrease of -0.21 point in the GNE (MD, 7.5; 95% CI, [5.71-9.28], FDR < 0·0001). CONCLUSIONS: These findings demonstrate that structural brain imaging and clinical measures in JOHD may be potential biomarkers of disease progression for use in clinical trials. Collaborative efforts are required to validate these results in a larger cohort of patients with JOHD. © 2022 International Parkinson and Movement Disorder Society.

Neuropsychological Profiles of Athletes and Views of Parents Choosing Flag Versus Tackle Football Participation.

Background: Previous studies have found that injury rates are slightly higher in children who play flag football versus tackle football. It is unclear if this difference is due to the way each type is played or taught or whether there are intrinsic differences in attitudes or neuropsychological characteristics in children and their parents. Purpose: To determine whether children who play flag football score differently from those who play tackle football on validated neuropsychological tests. Study Design: Cross-sectional study; Level of evidence, 3. Methods: Each participating athlete (aged 8-12 years) was recruited in 2018 and 2019 by email through local youth football leagues and the local university. Each athlete was administered a 1-time multidimensional assessment battery. The battery included the Wechsler Abbreviated Scale of Intelligence-2nd Edition, the children's version of the Trail Making Test, the Integrated Digit Span and Spatial Span subtests of the Wechsler Intelligence Scale for Children-4th Edition (WISC-IV), and the Beck Self-Concept Inventory for Youth. The parent/guardian of each athlete completed the Achenbach Child Behavior Checklist-Parent Report Form, the Behavior Rating Inventory of Executive Function (BRIEF)-Parent Form, and a custom survey. These tests were used to determine IQ estimates and standardized scores, measuring verbal comprehension, matrix reasoning, mental set-shifting, attention, cognitive processing speed, working memory, spatial processing, perception of self-concept, behavioral regulation index, metacognition index, and global executive composite. Scores were compared between flag football and tackle football groups by 2-sample t test, with the Wilcoxon rank-sum test used for nonparametric data. Results: A total of 64 athletes (41 tackle football, 23 flag football) were enrolled from youth football leagues (grades 4-6). Flag players scored significantly higher on the WISC-IV Spatial Span-Backward subtest (scaled mean, 12.0 vs 10.6; P = .046), while tackle players had significantly higher BRIEF-Inhibit subscores (mean t-score, 45 vs 42; P = .026). There were no significant differences in any of the other tests, including socioeconomic status and perceived concussion risks. Conclusion: Concerns that injury epidemiologic studies comparing flag with tackle football could be confounded by intrinsic differences in the children who choose to play each type seem to be unfounded.

Behavioral Deficits in Juvenile Onset Huntington's Disease.

Reports of behavioral disturbance in Juvenile-Onset Huntington's Disease (JOHD) have been based primarily on qualitative caregiver reports or retrospective medical record reviews. This study aims to quantify differences in behavior in patients with JOHD using informant- and self-report questionnaires. Informants of 21 children/young adults (12 female) with JOHD and 115 children/young adults (64 female) with a family history of Huntington's Disease, but who did not inherit the disease themselves (Gene-Non-Expanded; GNE) completed the Behavior Rating Inventory of Executive Function (BRIEF) and the Pediatric Behavior Scale (PBS). Mixed linear regression models (age/sex adjusted) were conducted to assess group differences on these measures. The JOHD group had significantly higher scores, indicating more problems, than the GNE group on all BRIEF subscales, and measures of Aggression/Opposition and Hyperactivity/Inattention of the PBS (all p < 0.05). There were no group differences in Depression/Anxiety. Inhibit, Plan/Organize, Initiate, and Aggression/Opposition had significant negative correlations with Cytosine-Adenine-Guanine (CAG) repeat length (all p < 0.05) meaning that individuals with higher CAG repeats scored lower on these measures. There was greater discrepancy between higher informant-vs. lower self-reported scores in the JOHD group, supporting the notion of lack of insight for the JOHD-affected group. These results provide quantitative evidence of behavioral characteristics of JOHD.

Return on Investment and Value Research in Neuropsychology: A Call to Arms†.

There is substantial empirical evidence to support the clinical value of neuropsychological evaluation and the incremental value of neuropsychological assessment, suggesting such evaluation is beneficial in the prediction and management of clinical outcomes. However, in the cost-conscious and evolving era of healthcare reform, neuropsychologists must also establish the economic value, or return on investment, of their services. There is already a modest body of literature that demonstrates the economic benefits of neuropsychological evaluation, which is reviewed in the current paper. Neuropsychologists will need to be able to communicate, and develop evidence of, economic value of their services; thus, this paper also discusses common concepts, terms, and models used in healthcare valuation studies. Finally, neuropsychologists are urged to incorporate these financial concepts in their clinical practice and research.

Brain structure in juvenile-onset Huntington disease.

OBJECTIVE: To assess brain morphometry in a sample of patients with juvenile-onset Huntington disease (JOHD) and several mouse models of Huntington disease (HD) that likely represent the human JOHD phenotype. METHODS: Despite sharing the mutation in the Huntingtin gene, adult-onset HD characteristically presents as a hyperkinetic motor disorder, while JOHD typically presents as a hypokinetic motor disease. The University of Iowa Kids-JHD program enrolls individuals 5 to 25 years of age who have already received the clinical diagnosis. A total of 19 children with juvenile HD (JHD) (mean CAG = 72) were studied. Patients with JHD were compared to healthy controls (n = 234) using a cross-sectional study design. Volumetric data from structural MRI was compared between groups. In addition, we used the same procedure to evaluate brain morphology of R6/2, zQ175, HdhQ250 HD mice models. RESULTS: Participants with JHD had substantially reduced intracranial volumes. After controlling for the small intracranial volume size, the volumes of subcortical regions (caudate, putamen, globus pallidus, and thalamus) and of cortical white matter were significantly decreased in patients with JHD. However, the cerebellum was proportionately enlarged in the JHD sample. The cerebral cortex was largely unaffected. Likewise, HD mice had a lower volume of striatum and a higher volume of cerebellum, mirroring the human MRI results. CONCLUSIONS: The primary pathology of JOHD extends beyond changes in the striatal volume. Brain morphology in both mice and human patients with JHD shows proportional cerebellar enlargement. This pattern of brain changes may explain the unique picture of hypokinetic motor symptoms in JHD, which is not seen in the hyperkinetic chorea-like phenotype of adult-onset HD.

A survey-based study identifies common but unrecognized symptoms in a large series of juvenile Huntington's disease.

AIM: The symptoms of Huntington's disease are well known, yet the symptoms of juvenile Huntington's disease (JHD) are less established due to its rarity. The study examined a cluster of symptoms considered to be common, but under-recognized in JHD: pain, itching, sleeping difficulties, psychosis and tics. MATERIALS & METHODS: A symptom survey was constructed using the online tool Qualtrics and dispersed to JHD caregivers through websites. RESULTS: A total of 33 surveys were completed. Disrupted sleep was the most prevalent symptom (87%), followed by tics (78%), pain (69%), itching (60%) and psychosis (39%). CONCLUSION: Despite limitations, the study supports that there are symptoms in the JHD population that are not considered classic, however, are common and significant for patients and caregivers.

Manipulative and antisocial behavior in an 11-year-old boy with epilepsy.

Brian is an 11-year-old boy who presented to the emergency room with suicidal ideation and hearing voices. In the preceding weeks, he had escalating symptoms of oppositional defiant disorder, attention-deficit hyperactivity disorder (ADHD), and bipolar disorder. His medical history was notable for complex partial epilepsy with onset at age 4 that had been well controlled with divalproate. He had several mental health diagnoses by various practitioners including oppositional defiant disorder, ADHD, and bipolar disorder. Brian's family and social history was notable for the absence of identifiable risk factors for seizures or psychiatric problems. Over the course of a week-long psychiatric hospitalization, his complaints of depression and hearing voices seemed incongruent with his behavior. His parents endorsed a long history of Brian manipulating family and friends, such as conning his friends into stealing money and giving it to him. There was increasing suspicion that Brian was contriving his presenting symptoms for secondary gains. When his parents visited, he consistently bargained for prized items such as a long sought after cell phone and his own bedroom to improve his mood. His prior diagnoses (ADHD, a mood disorder, and oppositional defiant disorder) did not capture what seemed to be his core problem--an ability and willingness to manipulate others for his own self-serving purposes. Three months later, he was seen in the pediatric neurology clinic for increased seizure frequency. In the interim, he had several very serious altercations including setting fire to his family church, an attempted break-in-and-entry, assaulting his principal and resisting the arresting officer, and a malicious planned attack on his father where he struck him in the head with a crescent wrench "in cold blood, without any emotion."

Pre- and post-electroconvulsive therapy multidomain cognitive assessment in psychotic depression: relationship to premorbid abilities and symptom improvement.

OBJECTIVES: Cognitive changes have been reported in patients after electroconvulsive therapy (ECT), but few studies have investigated post-ECT changes across multiple cognitive domains. Because cognitive dysfunction is presumed to be more salient in psychotic depression, we propose a brief pre-ECT multidomain cognitive assessment battery, assessing neurocognitive function in this population before and after ECT. We also compared performance to estimated premorbid levels and determined if neuropsychological functioning was related to symptom improvement. METHODS: Twenty participants with psychotic depression (12 females, 8 males) undergoing ECT for severe depression received the repeatable battery for the assessment of neuropsychological status (RBANS) and additional tasks. The wide range achievement test reading test provided an estimate of premorbid intellectual functioning. Depressive symptoms were assessed with the Hamilton Depression Scale-28, whereas negative and positive symptoms were assessed with the Scale for Assessing Negative and Positive Symptoms. RESULTS: There was a significant improvement in depressive symptoms with most measures of cognitive function showing net gains. When cognitive performances were compared with estimated premorbid abilities, findings indicated significant movement toward normalization in overall RBANS score, particularly involving the language index and attention index. Considered individually, 6 (30%) participants showed pre-ECT cognitive dysfunction (RBANS total score

Bilingualism, brain injury, and recovery: implications for understanding the bilingual and for therapy.

Psychologists and other therapists are seeing an increasingly large number of bilingual individuals. Such clients are a special challenge when there has been some type of brain injury or disease because of the seemingly unpredictable effect such disorders may have on language skills, impacting either or both of the client's languages and interfering with internal speech that plays a role in higher cognitive functions such as insight and awareness. While there are many clinical assumptions about which language will show the least impairment or recover the best, such suppositions based on clinical lore are often contradictory. A review of the literature finds that the outcome of brain injury may be influenced by factors such as cerebral representation of a secondary language, method of language acquisition, age of acquisition, premorbid language proficiency, and style of learning in an individual. Neuropsychological concepts that can explain these findings are examined, along with their implications for therapy, and rehabilitation.