Background: The short version of the Problem Behaviours Assessment (PBA-s) is the recommended outcome measure for behavioural symptoms in Huntington's disease. Rasch analysis was used to further investigate the measurement limitations of the PBA-s.
Objectives: 1) To assess the psychometric properties of the 11 severity and frequency items within the PBA-s and 2) to determine the construct validity of using a total PBA-s score as a clinical outcome measure.
Methods: PBA-s data for 517 participants from Enroll-HD were included in the Rasch analysis. Separate analyses were conducted for the severity and frequency items of the PBA-s, using RUMM2030 software. Achieving fit to the model provides supporting evidence that all items contribute to a single underlying latent trait. This property is defined as internal construct validity.
Results: The total PBA-s severity score demonstrated several important limitations, including disordered response categories for all 11 severity items, local dependency and poor targeting. However, modifying the original five-point scoring system to a four-point system resulted in ordered response categories for seven of the severity items and achieved a good overall fit to the Rasch model. For the total PBA-s frequency score, fit to the model was not achieved even after amendments to the scoring system.
Conclusions: This study suggests that with reduction to a four-point scoring system, the total PBA-s severity score may be considered a valid clinical outcome measure. This study also suggests limitations in the use of a total PBA-s frequency score.
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http://dx.doi.org/10.3233/JHD-150164 | DOI Listing |
Neurol Ther
October 2024
Department of Neurology, Hospital Ramón y Cajal, IRYCIS, Madrid, Spain.
Background: Limited information is available on patients' experience living with Huntington's disease (HD). The primary objective of this study was to assess the health-related quality of life and well being of patients with HD.
Methods: A non-interventional, cross-sectional study was conducted in 17 hospitals-based movement disorders units in Spain.
Brain Behav
November 2023
Department of Neurology, Laboratory of Neurodegenerative Disorders, Rare Disease Center, West China Hospital, Sichuan University, Chengdu, China.
Background And Aim: Huntington's disease (HD) is an autosomal dominant inherited neurodegenerative disorder caused by CAG repeats expansion. Cognitive decline contributes to the loss of daily activity in manifest HD. We aimed to examine the cognition status in a Chinese HD cohort and explore factors influencing the diverse cognitive domains.
View Article and Find Full Text PDFJ Clin Med
December 2022
Department of Neurology, Rare Disease Center, West China Hospital, Sichuan University, Chengdu 610041, China.
Huntington’s disease (HD) is an autosomal dominant inherited neurodegenerative disorder caused by CAG repeats expansion. There is a paucity of comprehensive clinical analysis in Chinese HD patients due to the low prevalence of HD in Asia. We aimed to comprehensively describe the motor, neuropsychiatric symptoms, and functional assessment in patients with HD from China.
View Article and Find Full Text PDFBrain Behav
July 2021
Huntington and Rare Diseases Unit, Fondazione IRCCS Casa Sollievo della Sofferenza Hospital, San Giovanni Rotondo, Italy.
Introduction: Behavioral and cognitive changes can be observed across all Huntington disease (HD) stages. Our multicenter and retrospective study investigated the association between cognitive and behavioral scale scores in manifest HD, at three different yearly timepoints.
Methods: We analyzed cognitive and behavioral domains by the Unified Huntington's Disease Rating Scale (UHDRS) and by the Problem Behaviors Assessment Short Form (PBA-s), at three different yearly times of life (t0 or baseline, t1 after one year, t2 after two years), in 97 patients with manifest HD (mean age 48.
J Huntingtons Dis
August 2021
Neurology Department, Vanderbilt University Medical Center, Nashville, TN, USA.
Background: Psychiatric symptoms are widely prevalent in Huntington's disease (HD) and exert greater impact on quality of life than motor manifestations. Despite this, psychiatric symptoms are frequently underrecognized and undertreated. Lack of awareness, or anosognosia, has been observed at all stages of HD and may contribute to diminished patient self-reporting of psychiatric symptoms.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!