Year: 2024
Type: conference-abstract
Abstract: Background Neuropsychological batteries can comprehensively evaluate cognitive dysfunction in early Huntington’s disease (HD). However, the extent to which these deficits correspond to the real-world experiences of patients and caregivers remains unclear. Thus, there is an ongoing need to develop sensitive and efficient cognitive assessments that capture the earliest self-reported cognitive symptoms that impact daily functioning in HD. Aims This study aims to pilot a questionnaire designed to assess perceived cognitive difficulties in individuals with HD. This questionnaire will be iteratively refined by selecting questions that most effectively differentiate between individuals with pre-symptomatic HD (preHD) and control participants. Methods Participants with known CAG repeat length and demographically-matched controls from the BIDMC HD research repository completed the initial 10-minute 44-item Likert-scale questionnaire (response range 1 (Never) to 5 (Almost Always)). Response frequency was tabulated for each question for each group. Independent samples t-tests were performed to compare group responses. Results 15 HD and 6 control participants were included in the analysis. The highest-rated items in controls, motor-manifest HD, and preHD (Total Motor Score (TMS) ≤8) are outlined. Conclusion This questionnaire will undergo rigorous refinement to better understand the nuances of self-reported cognitive difficulties in early HD. Although still in the pilot phase, this tool aims to enhance the ability to reliably capture cognitive changes, contributing to meaningful endpoint monitoring and outcome assessment in clinical research focused on improving early cognitive deficits in HD.
Institutions: Beth Israel Deaconess Medical Center, William James College
Language: English
Subfield: Cellular and Molecular Neuroscience
Field: Neuroscience
Domain: Life Sciences
Open Access status: gold
OpenAlex ID: W4402374427