PDF(Pubmed)
Abstract：
Although cognitive and motor symptoms of Huntington\'s disease (HD) are associated with disease progression, the underlying causes of psychological symptoms are not as clearly understood. Recent evidence suggests that some mental health difficulties experienced by people with HD are shared by noncarriers within HD families. Accordingly, there is a need to evaluate potential systemic contributors to HD mental distress, to support meaningful interventions for psychological symptoms in people with HD and affected families.
We used short-form Problem Behaviors Assessment mental health symptom data from the international Enroll-HD data set to characterize mental health symptoms across eight HD groups: Stages 1-5, premanifest and genotype-negative individuals, and family controls (n = 8567) using chi-square analysis with post hoc comparisons.
We identified that people with later-stage HD (Stages 2-5) had significantly higher apathy, obsessive-compulsiveness, and (from Stage 3) disorientation than the remaining groups at a medium effect size, and that these findings largely held across three measure administrations over time.
These findings highlight the critical symptoms in manifest HD from Stage 2 onward, but also demonstrate that crucial symptoms such as depression, anxiety, and irritability are present across HD-affected groups (including noncarriers of the gene expansion). The outcomes highlight a need for specific clinical management of later-stage HD psychological symptoms, and for systemic support across affected families.
We used short-form Problem Behaviors Assessment mental health symptom data from the international Enroll-HD data set to characterize mental health symptoms across eight HD groups: Stages 1-5, premanifest and genotype-negative individuals, and family controls (n = 8567) using chi-square analysis with post hoc comparisons.
We identified that people with later-stage HD (Stages 2-5) had significantly higher apathy, obsessive-compulsiveness, and (from Stage 3) disorientation than the remaining groups at a medium effect size, and that these findings largely held across three measure administrations over time.
These findings highlight the critical symptoms in manifest HD from Stage 2 onward, but also demonstrate that crucial symptoms such as depression, anxiety, and irritability are present across HD-affected groups (including noncarriers of the gene expansion). The outcomes highlight a need for specific clinical management of later-stage HD psychological symptoms, and for systemic support across affected families.
摘要：
背景：尽管亨廷顿病（HD）的认知和运动症状与疾病进展有关，心理症状的根本原因并不清楚。最近的证据表明，HD患者经历的一些心理健康困难是HD家庭中的非携带者共有的。因此，有必要评估HD精神困扰的潜在系统性贡献者，支持对HD患者和受影响家庭的心理症状进行有意义的干预。
方法：我们使用了来自国际注册HD数据集的简短形式的问题行为评估心理健康症状数据来表征八个HD组的心理健康症状：1-5阶段，入列前和基因型阴性个体，和家庭对照(n=8567)使用卡方分析和事后比较。
结果：我们发现晚期HD（2-5期）患者的冷漠程度明显更高，强迫症,并且（从第3阶段开始）比其余组具有中等效果大小的迷失方向，随着时间的推移，这些发现在三个措施管理部门中基本保持不变。
结论：这些发现强调了从第2阶段开始，表现为HD的危重症状，但也证明了抑郁症等关键症状，焦虑,和烦躁不安存在于受HD影响的人群中(包括基因扩增的非携带者)。结果强调需要对晚期HD心理症状进行特定的临床管理，以及受影响家庭的系统性支持。
方法：我们使用了来自国际注册HD数据集的简短形式的问题行为评估心理健康症状数据来表征八个HD组的心理健康症状：1-5阶段，入列前和基因型阴性个体，和家庭对照(n=8567)使用卡方分析和事后比较。
结果：我们发现晚期HD（2-5期）患者的冷漠程度明显更高，强迫症,并且（从第3阶段开始）比其余组具有中等效果大小的迷失方向，随着时间的推移，这些发现在三个措施管理部门中基本保持不变。
结论：这些发现强调了从第2阶段开始，表现为HD的危重症状，但也证明了抑郁症等关键症状，焦虑,和烦躁不安存在于受HD影响的人群中(包括基因扩增的非携带者)。结果强调需要对晚期HD心理症状进行特定的临床管理，以及受影响家庭的系统性支持。
