Emotion regulation across disease stages in Huntington's disease gene expansion carriers

OBJECTIVE: Emotion regulation difficulties are increasingly recognized in Huntington's disease (HD) yet remain understudied with inconsistent findings. While motor symptoms are prominent, patients and caregivers often describe emotional and behavioral changes as distressing. This study assessed perceived emotion regulation abilities and strategies across disease stages, with a focus on anger and irritability.

METHODS: We conducted a cross-sectional study including individuals with premanifest HD (n = 49), manifest HD (n = 41), and gene-negative family members previously at risk (n = 34). Participants completed the Difficulties in Emotion Regulation Scale (DERS) and the State-Trait Anger Expression Inventory (STAXI-2). Group differences were analyzed using one-way ANOVA or Kruskal-Wallis tests. Reference values for total DERS and STAXI-2 scores were derived from gene-negative family members, and percentile estimates were calculated.

RESULTS: Total scores on the DERS and STAXI-2 did not differ significantly between groups. However, both premanifest and manifest HD gene expansion carriers (HDGECs) scored significantly higher on the DERS Impulse Control subscale, indicating greater self-reported difficulties. One fourth of HDGECs scored within the 5th percentile on emotion regulation, and one third within the 10th percentile.

CONCLUSIONS: Total emotion regulation scores did not differ significantly between HDGECs and family controls. However, both premanifest and manifest HDGECs reported significantly greater difficulties with impulse control, alongside marked variability in overall emotion regulation. These findings highlight selective impulse control difficulties and a subset of individuals whose self-reported emotion regulation difficulties may be obscured by group-level analyses, underscoring the need for further research to clarify their clinical relevance.