Abstract

Background:Psychosis is considered rare in Huntington’s Disease, with an estimated prevalence of 3–11%. However, it has a profound impact on quality of life and disease burden. This study uses the Enroll-HD database to determine the prevalence, onset, and severity of psychosis in Huntington’s Disease and to determine demographic and disease characteristics associated with psychosis.Methods:Data were obtained from Enroll-HD. Adults with manifest Huntington’s Disease were included. Descriptive statistics were calculated. Simple logistic regression was used to calculate the odds ratio with 95% confidence interval for association with each characteristic.Results:7,966 manifest Huntington’s Disease participants were analyzed, and 12.95% had a history of psychosis. Mean age of psychosis onset (48.34 years, SD 13.26) mirrored Huntington’s Disease onset. Family history of psychosis in a first degree relative was documented in 23.6% of participants with psychosis. Variables significantly (p < 0.05) associated with presence of psychosis in manifest HD included lower education level, unemployment, single marital status, depression, decreased verbal fluency score, and decreased total functional capacity & functional assessment score.Discussion:Psychosis in Huntington’s Disease is more prevalent than many prior studies have reported. It is associated with several demographic & psychiatric features, decreased cognitive capacity, and worse functional outcomes.Highlights:Psychosis in HD is more prevalent than prior studies have reported. It is associated with a range of demographic and psychiatric variables, worse cognition, and worse functional outcomes suggesting several features that may be used to predict onset of psychosis and improve understanding and management of psychosis in HD.

Highlights

  • Huntington’s disease (HD) is an autosomal dominant ­neurodegenerative disease that has an incidence of about 0.38 per 100,000 individuals per year

  • Bivariate analysis was performed to evaluate the association between different demographic & disease characteristics and presence of psychosis in manifest HD

  • Our findings suggest that family history may be a better predictor for psychosis in HD as opposed to the general population, and supports prior research on familial aggregation of psychosis in HD [18]

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Summary

Introduction

Huntington’s disease (HD) is an autosomal dominant ­neurodegenerative disease that has an incidence of about 0.38 per 100,000 individuals per year. Psychosis is considered rare in Huntington’s Disease, with an estimated prevalence of 3–11% It has a profound impact on quality of life and disease burden. This study uses the Enroll-HD database to determine the prevalence, onset, and severity of psychosis in Huntington’s Disease and to determine demographic and disease characteristics associated with psychosis. Discussion: Psychosis in Huntington’s Disease is more prevalent than many prior studies have reported It is associated with several demographic & psychiatric features, decreased cognitive capacity, and worse functional outcomes. Highlights: Psychosis in HD is more prevalent than prior studies have reported It is associated with a range of demographic and psychiatric variables, worse cognition, and worse functional outcomes suggesting several features that may be used to predict onset of psychosis and improve understanding and management of psychosis in HD

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