Executive Impairment Is Associated With Unawareness of Neuropsychiatric Symptoms in Premanifest and Early Huntington's Disease

摘要

Objective: Unawareness of neuropsychiatric symptoms appears to be common in Huntington's disease (HD), but the clinical correlates of unawareness are unclear. Identifying predictors of unawareness is important for improving diagnosis of neuropsychiatric symptoms, and cognitive impairment, specifically executive impairment, may be a potential important predictor of unawareness. The authors examined whether unawareness of neuropsychiatric symptoms is more common in early HD compared to premanifest HD, and whether executive task performance was associated with awareness, independent of demographic, motor or mood variables. Method: One hundred thirty-two gene-positive participants (60 premanifest and 72 early diagnosed) from the multicenter TRACK-HD study were included. Participants and their informants completed self and informant versions of the Frontal Systems Behavior Scale, which measures executive dysfunction, apathy, and disinhibition symptoms. Awareness was measured as the discrepancy between self- and informant-reports across premanifest and early HD groups. Participants' executive task performance was then assessed as a predictor of unawareness across the whole group.Results: Premanifest participants reported higher levels of executive dysfunction, apathy and disinhibition than their informants, whereas early HD participants reported less executive dysfunction and apathy than their informants, indicating that unawareness is more common after diagnosis. Impaired executive task performance was related to unawareness of executive dysfunction and apathy, independent of demographic, motor and mood variables. Conclusions: Executive impairment is a useful early predictor of unawareness of neuropsychiatric symptoms in HD. Clinicians should closely monitor HD patients with executive impairment for unawareness, and consider this when assessing neuropsychiatric symptoms in HD and providing education to patients and families.