How different aspects of motor dysfunction influence day-to-day function in huntington's disease.

PURPOSE: This study examined the relationships between different aspects of motor dysfunction (chorea, dystonia, rigidity, incoordination, oculomotor dysfunction, dysarthria, and gait difficulties) and functional status in persons with Huntington's disease. METHODS: A total of 527 persons with Huntington's disease completed the Unified Huntington's Disease Rating Scale motor, total functional capacity, and functional assessments. RESULTS: Confirmatory factor analysis indicated that a 4-factor model provided a better model fit than the existing 5-factor model. Exploratory factor analysis identified the following 4 factors from the motor scale: dystonia, chorea, rigidity, and a general motor factor. Regression indicated that dystonia (ß = -0.47 and -0.79) and rigidity (ß = -0.28 and -0.59) had strong associations with function, whereas chorea had modest correlations (ß = -0.16 and -0.15). CONCLUSIONS: Dystonia and rigidity have stronger relationships with functional status than chorea in persons with Huntington's disease. The findings underscore the need for further research regarding the effects of dystonia and rigidity on functioning. © 2019 International Parkinson and Movement Disorder Society.

Self-report of pain in young people and adults with spastic cerebral palsy: interrater reliability of the revised Face, Legs, Activity, Cry, and Consolability (r-FLACC) scale ratings.

AIM: People with cerebral palsy (CP) are often unable to express pain owing to cognitive or speech impairments. Reports that rely on observation can be inaccurate, because behaviours such as grimacing, common in people with spastic CP, resemble pain expressions. We examined preliminary validity and reliability of the revised Face, Legs, Activity, Cry, and Consolability (r-FLACC) scale in people with spastic CP. METHOD: Forty-eight young people and adults (35 females, 13 males; mean [SD] age 29y 2mo [13y]) were video-recorded during a standard examination, rating their pain (0-10) afterwards. Two raters completed the r-FLACC using the video recordings. Interrater reliability was assessed with an unconditional cross-classified random-effects model and item response theory approach; Pearson correlations measured agreement between raters and participants. RESULTS: Mean (SD) participant (n=48) pain scores were 2.48 (2.5) and mean (SD) r-FLACC scores were 1.46 (1.68). There was moderate agreement between raters (intraclass coefficient 0.41 and 0.57 respectively) but low agreement between participants and raters (r=0.26). There were no significant effects for raters (lay observers, nurses, physicians, and inexperienced raters). INTERPRETATION: Results provide mixed support for the interrater reliability of the r-FLACC in people with spastic CP. WHAT THIS PAPER ADDS: The revised Face, Legs, Activity, Cry, and Consolability (r-FLACC) scale can be reliably used by experts and lay raters for people with spastic cerebral palsy (CP). Support is mixed for interrater reliability of the r-FLACC scale used with people with spastic CP.

Development of a rapid point-of-care patient reported outcome measure for cataract surgery in India.

BACKGROUND: For patient undergoing cataract surgery in India, existing patient-reported outcome (PRO) measures are either not culturally relevant, have not been adequately validated, or are too long to be used in a busy clinical setting. We sought to develop and validate a brief and culturally relevant point-of-care PRO measure to address this need. METHODS: Twelve items from the Indian Visual Functioning Questionnaire (IND-VFQ) were selected based on preliminary data. Patients 18 years and older were prospectively recruited at Aravind Eye Care System in Madurai, India. Clinical and sociodemographic data were collected and the 12-item short-form IND-VFQ (SF-IND-VFQ) was administered pre- and post-operatively to 225 patients; Factor analysis and Rasch modeling was performed to assess its psychometric properties. RESULTS: One item that did not fit a unidimensional scale and had poor fit with the Rasch model was eliminated from the questionnaire. The remaining 11 items represented a single construct (no residual correlations> 0.1) and were largely unaffected by differential item functioning. Five items had disordered thresholds resolved by collapsing the response scale from four to three categories. The survey had adequate reliability (0.80) and good construct (infit range, 0.77-1.29; outfit range, 0.56-1.30) and content (item separation index, 5.87 logits) validity. Measurement precision was fair (person separation index, 1.97). There was evidence that items were not optimally targeted to patients' visual ability (preoperatively, - 1.92 logits; overall, - 3.41 logits), though the survey measured a very large effect (Cohen's d 1.80). In a subset of patients, the average time to complete the questionnaire was 2 min 6.3 s. CONCLUSIONS: The SF-IND-VFQ is a valid, reliable, sensitive, and rapidly administered point-of-care PRO measure to assess changes in visual functioning in patients undergoing cataract surgery in India.

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.

Glaucoma-Associated Visual Task Performance and Vision-Related Quality of Life in South India.

Objective/Purpose: Performance-based measures may provide an objective assessment of how glaucoma affects daily functioning. We sought to validate a clinically-applicable performance-based measure of visual ability for patients with glaucoma in south India and to describe its relationship to clinical and patient-reported outcomes. Design: Cross-sectional validation study. Subjects/Participants/Controls: 145 participants with glaucoma were recruited at Aravind Eye Hospital. Methods/Intervention/Testing: We modified the compressed assessment of activities related to vision (CAARV), a performance-based measure validated in the U.S., to be culturally relevant in south India. Participants underwent a series of tests, including the Indian CAARV (I-CAARV), Indian Visual Functioning Questionnaire (IND-VFQ), Spaeth/Richman Contrast Sensitivity (SPARCS) test, standard automated perimetry, and visual acuity (VA). Factor analysis and Rasch modeling were used to validate the I-CAARV. Correlations between the I-CAARV and other outcomes were evaluated. Main Outcome Measure: Psychometric properties of the I-CAARV for individuals with glaucoma in south India. Results: The study included 142 participants (51.7% female, mean age 56.4 years). Average presenting visual acuity and visual field mean deviation (MD) in the better-seeing eye were 0.26 logMAR and -6.57 dB, respectively. The four tasks of the I-CAARV were found to measure a single underlying construct. Rasch analysis of the I-CAARV revealed that the outcome measure had moderate reliability, good construct and content validity, and fair measurement precision. Tasks were well-targeted to the study sample. Rasch-calibrated scores on the I-CAARV were significantly correlated with Rasch-calibrated IND-VFQ scores (r=-0.54) and with visual field MD, presenting VA, best-corrected VA, and SPARCS contrast sensitivity in both the better-seeing eye (r=0.60, -0.51, -0.53, 0.76, respectively) and worse-seeing eye (r=0.48, -0.61, -0.46, 0.69, respectively). Conclusions: The I-CAARV is a valid performance-based measure of vision-dependent functioning in glaucoma in south India. This study also found that I-CAARV task performance was strongly correlated with contrast sensitivity and suggests that performance-based and patient-reported outcomes are related but distinct measures of the impact of glaucoma on functioning and vision-related quality of life. Future studies are needed to determine the sensitivity of the I-CAARV to detect changes due to disease progression that are relevant to functioning and vision-related quality of life.

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

Development and initial validation of a brief self-report measure of cognitive dysfunction in fibromyalgia.

UNLABELLED: Pain is often the focus of research and clinical care in fibromyalgia (FM); however, cognitive dysfunction is also a common, distressing, and disabling symptom in FM. Current efforts to address this problem are limited by the lack of a comprehensive, valid measure of subjective cognitive dysfunction in FM that is easily interpretable, accessible, and brief. The purpose of this study was to leverage cognitive functioning item banks that were developed as part of the Patient Reported Outcomes Measurement Information System (PROMIS) to devise a 10-item short form measure of cognitive functioning for use in FM. In study 1, a nationwide (U.S.) sample of 1,035 adults with FM (age range = 18-82, 95.2% female) completed 2 cognitive item pools. Factor analyses and item response theory analyses were used to identify dimensionality and optimally performing items. A recommended 10-item measure, called the Multidimensional Inventory of Subjective Cognitive Impairment (MISCI) was created. In study 2, 232 adults with FM completed the MISCI and a legacy measure of cognitive functioning that is used in FM clinical trials, the Multiple Ability Self-Report Questionnaire (MASQ). The MISCI showed excellent internal reliability, low ceiling/floor effects, and good convergent validity with the MASQ (r = -.82). PERSPECTIVE: This paper presents the MISCI, a 10-item measure of cognitive dysfunction in FM, developed through classical test theory and item response theory. This brief but comprehensive measure shows evidence of excellent construct validity through large correlations with a lengthy legacy measure of cognitive functioning.