An Exploratory Pilot Study of Neuropsychological Performance in Two Huntington Disease Centers of Excellence Clinics.

OBJECTIVES: To describe the characteristics of patients receiving a clinical referral for neuropsychological evaluation in two Huntington's Disease Society of America Centers of Excellence (HDSA COE). In this exploratory pilot study, we used an empirically supported clinical neuropsychological battery to assess differences in cognitive performance between premanifest and manifest HD patient groups (compared with each other and normative expectations). METHOD: Clinical data from 76 adult genetically confirmed patients referred for neuropsychological evaluations was retrospectively collected from two HDSA COEs. ANOVA and Chi-square tests were used to compare variables between pre-manifest (n = 14) and manifest (n = 62) groups for demographic, cognitive, neuropsychiatric, and disease severity variables. RESULTS: Our clinics serviced a disproportionate number of motor manifest patients. Six measures were excluded from analyses due to infrequent administration. The full WAIS-IV Digit Span was disproportionately administered to the manifest group. The premanifest group showed stronger cognitive performance with effect sizes in the large range on subtests of the WAIS-IV Digit Span, HVLT-R, SDMT, and verbal fluency. CONCLUSIONS: This is the first study to assess an empirically supported neuropsychological research battery in a clinical setting with a relatively large sample size given the rarity of HD. The battery adequately captured areas of impairment across the disease spectrum. Application of the current battery with larger premanifest samples is warranted.

Huntington Study Group's Neuropsychology Working Group: Implementing Non-Motor Diagnostic Criteria.

Background: The clinical diagnosis of manifest Huntington's disease (HD) relies on a high level of clinical confidence (99% confidence) of HD-consistent motor signs. Longitudinal data have reliably identified cognitive and behavioral dysfunction predating clinical motor diagnosis by up to 15 years. Reliance on motor signs to establish a diagnosis of HD increases risk of early misdiagnosis or delayed diagnosis. Clinical neuropsychologists are uniquely positioned to advise on the clinical application of the Movement Disorder Society Task Force's recently proposed non-motor diagnostic criteria for HD. Objectives: To provide (1) a recommended clinical approach toward non-motor diagnostic criteria in persons with HD and facilitation of accurate diagnosis; (2) recommended practices for medical treatment providers to screen and longitudinally monitor non-motor signs of HD. Methods: The Huntington Study Group re-established the Neuropsychology Working Group, then recruited a multi-disciplinary group of neuropsychologists, neurologists, and psychiatrists to conduct an unstructured literature review and discuss expert opinions on practice, to facilitate an informal consensus opinion to accomplish the objectives. Results: The opinion and an example protocol for medical treatment providers to screen, monitor, and triage non-motor signs and symptoms of Huntington's disease is provided. Conclusions: Clinical diagnosis of non-motor HD is empirically justified and clinically important. Screening and triage by non-neuropsychologist clinicians can aid in detecting and monitoring non-motor Huntington's disease manifestation. The Neuropsychology Working Group consensus advances good clinical practice, clinical research, and quality of life. A companion position paper presenting the details of our consensus opinion regarding evidence-based guidelines for neuropsychological practice is forthcoming.

Huntington study group's neuropsychology working group position on best practice recommendations for the clinical neuropsychological evaluation of patients with Huntington disease.

Objective: Neuropsychological evaluation is critical to detection and management of cognitive and neuropsychiatric changes associated with Huntington disease (HD). Accurate assessment of non-motor complications of HD is critical given the prominent impact on functional disability, frequently commensurate with or exceeding that of motor symptoms. The increasing emphasis on developing disease-modifying therapies targeting cognitive decline in HD requires consensus on clinical neuropsychological assessment methods. The Neuropsychology Working Group (NPWG) of the Huntington Study Group (HSG) sought to provide evidence and consensus-based, practical guidelines for the evaluation of cognitive and neuropsychiatric symptoms associated with HD. Method: The NPWG recruited a multi-disciplinary group of neuropsychologists, neurologists, and psychiatrists to inform best practices in assessing, diagnosing, and treating the non-motor symptoms in HD. A review was circulated among the NPWG, and in an iterative process informed by reviewed literature, best practices in neuropsychological evaluation of patients with HD were identified. Results: A brief review of the available literature and rational for a clinical consensus battery is offered. Conclusion: Clinical neuropsychologists are uniquely positioned to both detect and characterize the non-motor symptoms in HD, and further, provide neurologists and allied health professions with clinically meaningful information that impacts functional outcomes and quality of life. The NPWG provides guidance on best practices to clinical neuropsychologists in this statement. A companion paper operationalizing clinical application of previous research-based non-motor diagnostic criteria for HD is forthcoming, which also advises on non-motor symptom screening methods for the non-neuropsychologist working with HD.

Distinguishing Alzheimer's disease from other dementias using pattern profile analysis in the Meyers Neuropsychological Battery: An exploratory study.

OBJECTIVE: This exploratory study aimed to assess the efficacy of pattern-matching statistical methods within the Meyers Neuropsychological Battery (MNB). It compared neuropsychological test data profiles of Alzheimer's disease (AD) patients from three independent samples against four MNB dementia groups: MNB-AD, MNB-Vascular Dementia (VaD), MNB-Dementia with Lewy bodies (DLB), and MNB-Parkinson's disease dementia (PDD). MATERIALS AND METHODS: Three AD-independent samples completed either the MNB (referred to as I-MNB-AD), Dementia Rating Scale-2 with additional testing (denoted as DRS-Plus-AD), or the Repeatable Battery for the Assessment of Neuropsychological Status (designated as RBANS-AD). Test data profiles were cross-validated with four MNB dementia comparison group datasets. Statistical methods included Pearson correlation, Kullback-Leibler (KL) divergence, pooled effect size (Cohen's d), Configuration, and MNB Code. RESULTS: Classification accuracy ranged from 40% (Pearson r) to 88% (Cohen's d) in the I-MNB-AD sample, 47% (Cohen's d) to 93% (KL) in the DRS-Plus-AD sample, and 47% (Pearson r) to 78% (Configuration) in the RBANS-AD sample. Some methods showed limited effectiveness depending on the sample and comparison group analyzed, while others demonstrated strong performance. Using a simple majority count of agreement, classification rates for selecting the MNB-AD comparison group were 80% (I-MNB-AD), 85% (DRS-Plus-AD), and 66% (RBANS-AD). CONCLUSIONS: This exploratory study demonstrates that specific statistical methods employed in the MNB for pattern-matching analysis effectively differentiated neuropsychological profiles of individuals with AD from other types of dementia, contributing to improved diagnostic precision. The findings underscore the potential advantages of pattern-matching analysis, advocating for further research to validate and refine its application.

Cross-validation and initial investigation of two abbreviated methods of the finger tapping test.

The Finger Tapping Test (FTT) is a widely utilized measure to assess lateralized motor speed and dexterity. The current study sought to cross-validate an abbreviated version of the FTT (i.e., M of Trials 3-5) and to evaluate a novel abbreviated method (i.e., M of three trials within five taps of each other; "3 within 5") to examine their respective effectiveness as a predictor of full-score performance based on a traditional administration procedure. The results showed that the abbreviated methods accurately predicted the full-test score, and any statistically significant differences that emerged were small based on effect size analysis and unlikely to be clinically meaningful. These findings were consistent across genders, among older adults, and among individuals displaying significant inter-trial tapping variability and thus requiring lengthier administration time. Classification accuracy statistics for the detection of impairment and performance validity status were high for both abbreviated methods. The results support two valid options for shortening the duration of the FTT. Both methods, used independently or in combination, are compatible with traditional administration procedures.

The Impact of Traumatic Brain Injury on Prison Health Services and Offender Management.

A large percentage of incarcerated offenders report a history of traumatic brain injury (TBI) with concomitant neuropsychiatric and social sequelae. However, research looking at the relationship between TBI and delivery of correctional health services and offender management is limited. In this study, the relationships between TBI and use of correctional medical/psychological services, chemical dependency (CD) treatment completion rates, in-prison rule infractions, and recidivism were investigated. Findings indicated that TBI history has a statistically significant association with increased usage of correctional medical/psychological services, including crisis interventions services, and with higher recidivism rates. Results also showed a trend toward offenders with TBI incurring higher rates of in-prison rule infractions and lower rates of CD treatment completion. Implications and future directions for correctional systems are discussed.