Understanding the Relationship Between Perseveration, Comorbid Behavioral Symptoms, Motor Decline, Functional Decline, and Self-report Accuracy in Huntington Disease Can Help Inform Clinical Practice.

Abstract

Background: Perseveration is one of the most debilitating symptoms of Huntington disease (HD).

Objective: To study perseveration and its relationship to comorbid behavioral symptoms, motor decline, functional decline, and subject self-report accuracy by analyzing cross-sectional data tracking individuals who have or are at risk for HD and healthy controls (HC).

Method: We studied 96 individuals from HD families and 35 HC who were either family controls or gene negative. We used χ 2 tests to compare patient demographic and survey outcomes data and to analyze the presence of obsessions and compulsions (OC), depression, and apathy relative to the presence of perseveration.

Results: Individuals with HD and perseveration had a higher presence of OC, depression, and apathy compared with individuals with HD of the same stages without perseveration (19%, 47.6%, and 47.6% vs 15%, 40%, and 25%, respectively). In addition, individuals in HD Stages 1-3 with higher motor scores (showing a later stage of disease) displayed a significantly higher rate of perseveration than the HC ( P = 0.0476; P = 0.0499, respectively). The presence of an informant resulted in a significantly higher rate of perseveration reporting for individuals in HD Stages 1 and 2 (41.2% and 53.8% with informant vs 23.5% and 11.1% without informant, respectively).

Conclusion: Perseveration was seen across all motor and functional stages for the individuals with HD, without significant differences between the different stages. Additionally, informants were beneficial to obtaining accurate patient reports of perseveration. These findings should prove useful for physician evaluation and treatment considerations.