Understanding the need for assistance with survey completion in people with Huntington disease.

PURPOSE: In Huntington disease (HD), motor, cognitive, and psychiatric changes can have a detrimental impact on health-related quality of life (HRQOL). The purpose of this paper is to describe the extent and type of assistance needed to complete online HRQOL surveys, and the impact of assistance on HRQOL scores. METHODS: A patient-reported outcome measurement system was developed for HD-specific HRQOL. Individuals across the prodromal and diagnosed disease severity spectrum (n = 532) completed surveys by computer, and reported the amount and type of assistance they received. RESULTS: Some participants (n = 56; 10.5%) did not complete all surveys; this group had larger proportions with late stage disease, racial/ethnic minority status, low education and single marital status, and poorer motor, independence and cognitive function compared to those who completed all surveys (n = 476). Overall, 72% of individuals did not receive assistance, 11% received computer assistance only, and 17% received assistance answering the survey questions. The majority of late stage individuals (78%) received some assistance compared to early stage (29%) and prodromal individuals (< 1%). Those who received assistance had higher proportions with late stage disease, were older, had less education, and had poorer functional and cognitive skills. Before and after adjustment for sociodemographic and clinical characteristics, those who received assistance had poorer scores on some HRQOL outcomes than those who did not receive assistance. CONCLUSIONS: Computer-based assessments are feasible for many persons with HD, although other methods may also be needed. Clinicians and researchers should develop strategies to assist people with HD to complete HRQOL surveys.

Evaluating cognition in individuals with Huntington disease: Neuro-QoL cognitive functioning measures.

PURPOSE: Cognitive functioning impacts health-related quality of life (HRQOL) for individuals with Huntington disease (HD). The Neuro-QoL includes two patient-reported outcome (PRO) measures of cognition-Executive Function (EF) and General Concerns (GC). These measures have not previously been validated for use in HD. The purpose of this analysis is to evaluate the reliability and validity of the Neuro-QoL Cognitive Function measures for use in HD. METHODS: Five hundred ten individuals with prodromal or manifest HD completed the Neuro-QoL Cognition measures, two other PRO measures of HRQOL (WHODAS 2.0 and EQ5D), and a depression measure (PROMIS Depression). Measures of functioning The Total Functional Capacity and behavior (Problem Behaviors Assessment) were completed by clinician interview. Objective measures of cognition were obtained using clinician-administered Symbol Digit Modalities Test and the Stroop Test (Word, Color, and Interference). Self-rated, clinician-rated, and objective composite scores were developed. We examined the Neuro-QoL measures for reliability, convergent validity, discriminant validity, and known-groups validity. RESULTS: Excellent reliabilities (Cronbach's alphas ≥ 0.94) were found. Convergent validity was supported, with strong relationships between self-reported measures of cognition. Discriminant validity was supported by less robust correlations between self-reported cognition and other constructs. Prodromal participants reported fewer cognitive problems than manifest groups, and early-stage HD participants reported fewer problems than late-stage HD participants. CONCLUSIONS: The Neuro-QoL Cognition measures provide reliable and valid assessments of self-reported cognitive functioning for individuals with HD. Findings support the utility of these measures for assessing self-reported cognition.

Patient-reported outcomes in Huntington's disease: Quality of life in neurological disorders (Neuro-QoL) and Huntington's disease health-related quality of life (HDQLIFE) physical function measures.

BACKGROUND: There is a need for patient-reported outcome measures that capture the impact that motor impairments have on health-related quality of life in individuals with Huntington's disease. OBJECTIVES: The objectives of this study were to establish the reliability and validity of new physical functioning patient-reported outcome measures in Huntington's disease. METHODS: A total of 510 individuals with Huntington's disease completed 2 Quality of Life in Neurological Disorders (Lower Extremity Function and Upper Extremity Function) and 3 Huntington's Disease Health-Related Quality of Life (Chorea, Speech Difficulties, and Swallowing Difficulties) measures. Clinician-rated and generic self-report measures were also administered. RESULTS: Reliabilities for the new patient reported physical functioning measures were excellent (all Cronbach's α > .92). Convergent, discriminant validity and known group validity was supported. CONCLUSIONS: The results provide psychometric support for new patient-reported physical functioning measures and the fact that these measures can be used as clinically meaningful endpoints in Huntington's disease research and clinical practice. © 2017 International Parkinson and Movement Disorder Society.

Validity of the 12-item World Health Organization Disability Assessment Schedule 2.0 (WHODAS 2.0) in individuals with Huntington disease (HD).

PURPOSE: The reliability and construct validity of the 12-item World Health Organization Disability Assessment Schedule 2.0 (WHODAS 2.0) were examined in individuals with Huntington disease (HD). METHODS: We examined factor structure (confirmatory factor analysis), internal consistency reliability (Cronbach's alpha), floor and ceiling effects, convergent validity (Pearson correlations), and known-groups validity (multivariate analysis). RESULTS: Results of a confirmatory factor analysis replicated the six-factor latent model that reflects the six separate scales within the WHODAS 2.0 (understanding and communicating; getting around; self-care; getting along with others; life activities; participation). Cronbach's alpha for the scale was 0.94, suggesting good internal consistency reliability. The WHODAS demonstrated a ceiling effect for 19.5 % of participants; there were no floor effects. There was evidence for convergent validity; the WHODAS demonstrated moderate significant correlations with other general measures of health-related quality of life (HRQOL; i.e., RAND-12, EQ5D). Multivariate analyses indicated that late-stage HD participants indicated poorer HRQOL than both early-stage HD and prodromal HD participants for all HRQOL measures. CONCLUSIONS: Findings provide support for both the reliability and validity of the WHODAS 2.0 in individuals with HD.

Validation of Neuro-QoL and PROMIS Mental Health Patient Reported Outcome Measures in Persons with Huntington Disease.

BACKGROUND: Patient-reported outcomes (PROs) for mental health are important for persons with Huntington disease (HD) who commonly experience symptoms of depression, anxiety, irritability, anger, aggression, and apathy. Given this, there is a need for reliable and valid patient-reported outcomes measures of mental health for use as patient-centered outcomes in clinical trials. OBJECTIVE: Thus, the purpose of this study was to establish the psychometric properties (i.e., reliability and validity) of six Neuro-QoL and PROMIS mental health measures to support their clinical utility in persons with HD. METHODS: 294 individuals with premanifest (n = 102) or manifest HD (n = 131 early HD; n = 61 late HD) completed Neuro-QoL/PROMIS measures of Emotional and Behavioral Dyscontrol, Positive Affect and Well-Being, Stigma, Anger, Anxiety, and Depression, legacy measures of self-reported mental health, and clinician-rated assessments of functioning. RESULTS: Convergent validity and discriminant validity for the Neuro-QoL and PROMIS measures of Emotional and Behavioral Dyscontrol, Positive Affect and Well-Being, Stigma, Anger, Anxiety, and Depression, were supported in persons with HD. Neuro-QoL measures of Anxiety and Depression also demonstrated moderate sensitivity and specificity (i.e., they were able to distinguish between individuals with and without clinically significant anxiety and depression). CONCLUSIONS: Findings provide psychometric support for the clinical utility of the Neuro-QoL/PROMIS measures of mental health measures in persons with HD. As such, these measures should be considered for the standardized assessment of health-related quality of life in persons with HD.

Factors Associated With End-of-Life Planning in Huntington Disease.

OBJECTIVE: Knowledge of one's gene status for adult onset conditions provides opportunity to make advance end-of-life (EOL) plans. The purposes of these analyses were to (1) determine the prevalence of EOL plans, including advance directives (ADs) among persons across 3 stages of Huntington disease (HD) and (2) examine factors associated with having ADs in this sample. METHODS: Data are from 503 participants in the HD Quality of Life study. Participants completed an online health-related quality-of-life survey that included questions regarding EOL planning and self-reported HD symptoms. Frequencies were calculated for EOL planning by the HD stage. Bivariate analysis and logistic regression were used to identify variables associated with having ADs. RESULTS: A total of 38.2% of participants stated they had ADs and fewer than half had other EOL plans. Being older, increased HD stage, more years of education, lower anxiety, more swallowing symptoms, and higher meaning and purpose were associated with having ADs. CONCLUSION: The prevalence of ADs in our sample is comparable to the general US population, but surprisingly low, considering the severity and long disease course of HD. PRACTICE IMPLICATIONS: Health-care providers should develop specific interventions early in the disease process to increase ADs in this population.

Patient-reported outcome measures in Huntington disease: Quality of life in neurological disorders (Neuro-QoL) social functioning measures.

Social functioning is an essential but poorly understood component of health-related quality of life (HRQOL) for people with Huntington disease (HD). We report on the psychometric properties of 2 Neuro-QoL patient-reported outcome measures to assess social functioning in HD. Persons with prodromal (n = 198) or manifest HD (n = 195 early and n = 117 late) completed Neuro-QoL Ability to Participate in Social Roles and Activities, and Satisfaction with Social Roles and Activities. Items from 2 generic HRQOL patient-reported outcome measures were used to create a social functioning composite score; items from the Unified Huntington's Disease Rating Scale and Problem Behaviors Assessment Scale were used to create a clinician-rated composite score of social function. Internal consistencies for the scores on the Neuro-QoL measures were excellent (> .88). Computer adaptive test administration had some advantages over computer-administered static Short Forms. Validity was supported by significant associations between the scores on the Neuro-QoL measures and other self- and clinician-reports of social function. Individuals with prodromal HD had better social functioning than the manifest HD groups; individuals with late-HD had less satisfaction and ability to participate in social roles and activities than the other 2 groups. Neuro-QoL provides brief, reliable scores of social functioning that measure ability to participate in, and satisfaction with, social roles and activities in persons with prodromal and manifest HD. In addition, test score interpretations of these measures support their validity in people with prodromal and manifest HD. These measurement tools add breadth to treatment outcome measures in HD and can increase understanding of the social implications of living with HD. (PsycINFO Database Record

Motor-free composites from the National Institutes of Health Toolbox Cognition Battery (NIHTB-CB) for people with disabilities.

PURPOSE/OBJECTIVE: The National Institutes of Health Toolbox Cognition Battery (NIHTB-CB) includes a group of brief measures (i.e., 30 min) designed to assess language, processing speed, working memory, episodic memory, and executive functioning. These subtests can be combined to create composite scores that reflect fluid and crystallized cognition, as well as overall cognition. The battery is of limited utility with individuals who have impaired upper extremity motor functioning. This manuscript examines the accuracy of the Oral Symbol Digit Modalities Test as a substitute for the Pattern Comparison Processing Speed Test for computing motor-free composite scores. Research Method/Design: Individuals with spinal cord injury (SCI; n = 188), traumatic brain injury (TBI; n = 159), or stroke (n = 180) completed the NIHTB-CB. We used the Oral Symbol Digit Modalities Test to create a Motor-Free Pattern Comparison score; this was used to create revised, Motor-Free Composite scores for Fluid Cognition and Overall Cognition. RESULTS: Although there were statistically significant overall differences between the two Fluid and Overall Cognition composite scores for some of the clinical groups (scores based on the motor-free approach were significantly higher than the original score), these differences were small and partly because of overclassification of impaired processing speed in participants with motor impairment. There was good to substantial agreement with regard to "impairment" classification between the two sets of Original and Motor-Free composite scores. CONCLUSIONS/IMPLICATIONS: Although the Motor-Free scores are not a perfect match for the Original Composite scores, they provide a reliable and valid way to examine overall and fluid cognition in individuals with upper extremity motor impairments. (PsycINFO Database Record

Construct validity of the NIH Toolbox Cognition Battery in individuals with stroke.

OBJECTIVE: The National Institutes of Health (NIH) Toolbox (NIHTB) for the Assessment of Behavior and Neurological Function Cognition Battery (NIHTB-CB) provides a brief assessment (approximately 30 min) of key components of cognition. This article examines construct validity to support the clinical utility of the NIHTB-CB in individuals with stroke. RESEARCH METHOD: A total of 131 individuals with stroke (n = 71 mild stroke; n = 60 moderate/severe stroke) completed the NIHTB-CB. Univariate analyses were conducted to examine the cognitive profiles of the two different stroke groups (mild vs. moderate/severe stroke) on NIHTB-CB measures and composite scores. Pearson correlations were conducted between NIHTB-CB and established measures to examine convergent and discriminant validity. Effect sizes and clinical impairment rates for the different NIHTB-CB measures and composite scores were also examined. RESULTS: Participants experiencing moderate to severe stroke had poorer performance than did individuals with mild stroke on several of the NIHTB cognition measures. Evidence of convergent validity was provided by moderate to strong correlations between the NIHTB measures and the corresponding standard neuropsychological test (Pearson rs ranged from 0.31 to 0.88; median = .60). Evidence of discriminant validity was provided by smaller correlations between different cognitive domains than correlations of measures within the same domain. Effect sizes for composite and subtest scores regarding stroke severity were generally moderate-to-large. In addition, 42% of the sample were exhibiting mild cognitive impairment (i.e., ≥2 low scores on fluid tests). CONCLUSIONS: Findings provide support for the construct validity of the NIHTB-CB in individuals with stroke. (PsycINFO Database Record

The nature and prevalence of disability in a Ghanaian community as measured by the Language Independent Functional Evaluation.

INTRODUCTION: The current study uses the Language Independent Functional Evaluation (L.I.F.E.) to evaluate disability in a smaller Ghanaian coastal town to characterize the extent and nature of disability. The L.I.F.E. is a video animated, language free equivalent of the standard 10-item verbal/written Barthel Index functional assessment. METHODS: Over a four-month period, the L.I.F.E. survey was given to members of the village of Anomabo in a preliminary survey which consisted of recruitment in an un-controlled manner, followed by a systematic, comprehensive survey of three neighborhood clusters. Basic demographics were also collected, along with the observer's assessment of disability. RESULTS: 541 inhabitants (264 in the preliminary survey and 277 in systematic survey) completed the L.I.F.E. Participants ranged from 7-100 years old (mean age 32.88, s.d. 20.64) and were 55.9% female. In the systematic study, 16.6% of participants had a less than perfect score on the L.I.F.E., indicating some degree of impairment. Significant differences were found between age groups, but not between sexes, the preliminary and systematic survey, and study location (a=.05). CONCLUSION: The L.I.F.E. and this study methodology can be used to measure the prevalence of disability in African communities. Disability in this community was higher than the frequently cited estimate of 10%. African policymakers can use the L.I.F.E. to measure disability and thus more rationally allocate resources for medical rehabilitation.

Construct validity of language independent functional evaluation as used by Spanish-speaking clinicians to evaluate persons with developmental delays.

OBJECTIVE: This study aimed to establish the utility of Language Independent Functional Evaluation (LIFE) as used by clinicians. DESIGN: This study was a prospective trial involving 100 Spanish-speaking rehabilitation clients between the ages of 9 and 45 yrs with cognitive disability and 2 rehabilitation clinicians who cared for them at a Colombian inpatient and outpatient rehabilitation facility. Clinicians who had worked with clients for more than 2 mos rated their function using the LIFE and the Spanish-translated Barthel index. RESULTS: Overall LIFE scores correlated well with Barthel scores (r[98] = 0.793, P < 0.0001) and also on an item-to-item basis in all activities except grooming, bowel continence, and bladder continence. CONCLUSIONS: The LIFE is a reliable tool for clinicians to use in the assessment of persons with cognitive disability, regardless of the clinician's language.

Body mass index, but not blood pressure is related to the level of pain in persons with chronic pain.

OBJECTIVE: To determine whether obesity or blood pressure relate to the level of pain experienced by persons with chronic back pain. DESIGN: Retrospective Study. SETTING: University spine program. PATIENTS (OR PARTICIPANTS): Adults seeking care for back pain of more than 3 months duration. METHODS OR INTERVENTIONS (OR ASSESSMENT OF RISK FACTORS): Measurement of weight, blood pressure, and pain on a numeric scale. MAIN OUTCOME MEASUREMENTS: Relationships between body mass index (BMI) and pain, blood pressure and pain. RESULTS: 198 subjects had a mean BMI of 29.50 ± 7.12, with 33.3% obese. The relationship between pain as a continuous variable and BMI approached significance (r= 0.125, n= 175, p= 0.098). The BMI of persons with lower pain scores of 0-3 was significantly different from those with high pain scores of 7-10. No significant relationship was found between pain and systolic (r=0.126, p=0.078) or diastolic (r= 0.099, p=0.165) blood pressure. CONCLUSIONS: Contrary to assumptions about acute pain, increased chronic pain does not appear to relate to increased blood pressure. The relationship of obesity with increased pain level among persons who have chronic pain raises the possibility that psychological or physiological mechanisms may be important above and beyond the biomechanical impact of obesity.