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 χ² 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.

中文翻译：

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了解亨廷顿病的坚持、合并行为症状、运动衰退、功能衰退和自我报告准确性之间的关系有助于为临床实践提供信息

背景： 

顽固是亨廷顿病（HD）最令人衰弱的症状之一。

客观的： 

通过分析跟踪患有 HD 或有 HD 风险的个体和健康对照组 (HC) 的横截面数据，研究坚持不懈及其与共病行为症状、运动衰退、功能衰退和受试者自我报告准确性的关系。

方法： 

我们研究了来自 HD 家庭的 96 名个体和 35 名 HC，他们要么是家庭对照，要么是基因阴性。我们使用 χ2^检验来比较患者人口统计和调查结果数据，并分析与坚持相关的强迫观念和强迫行为(OC)、抑郁和冷漠的存在情况。

结果： 

与患有相同阶段的 HD 但没有坚持的个体相比，患有 HD 并坚持不懈的个体出现 OC、抑郁和冷漠的情况更高（分别为 19%、47.6% 和 47.6% vs 15%、40% 和 25%） 。此外，处于 HD 1-3 阶段且运动评分较高（显示疾病晚期）的个体表现出比 HC 显着更高的坚持率（分别为P = 0.0476；P = 0.0499）。知情人的存在导致 HD 第 1 阶段和第 2 阶段的个人的坚持报告率显着较高（有知情人的情况下，分别为 41.2% 和 53.8%，而没有知情人的情况下，分别为 23.5% 和 11.1%）。

结论： 

HD 患者的所有运动和功能阶段都表现出坚持，不同阶段之间没有显着差异。此外，信息提供者有利于获得准确的患者坚持情况报告。这些发现应该对医生评估和治疗考虑有用。