Huntington's disease is associated with motor, cognitive and behavioral dysfunction. Behavioral symptoms may present before, after, or simultaneously with clinical disease manifestation. The relationship between age of onset and behavioral symptom presentation and severity was explored using the Enroll‐HD database. Manifest individuals (n = 4469) were initially divided into three groups for preliminary analysis: early onset (<30 years; n = 479); mid‐adult onset (30‐59 years; n = 3478); and late onset (>59 years; n = 512). Incidence of behavioral symptoms reported at onset was highest in those with early onset symptoms at 26% (n = 126), compared with 19% (n = 678) for mid‐adult onset and 11% (n = 56) for late onset (P < 0.0001). Refined analysis, looking across the continuum of ages rather than between categorical subgroups found that a one‐year increase in age of onset was associated with a 5.6% decrease in the odds of behavioral symptoms being retrospectively reported as the presenting symptom (P < 0.0001). By the time of study enrollment, the odds of reporting severe behavioral symptoms decreased by 5.5% for each one‐year increase in reported age of onset. Exploring environmental, genetic and epigenetic factors that affect age of onset and further characterizing types and severity of behavioral symptoms may improve treatment and understanding of Huntington's disease's impact on affected individuals.

中文翻译：

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亨廷顿病的发病年龄和行为表现：一项 Enroll-HD 队列分析

亨廷顿病与运动、认知和行为功能障碍有关。行为症状可能在临床疾病表现之前、之后或同时出现。使用 Enroll-HD 数据库探讨了发病年龄与行为症状表现和严重程度之间的关系。显性个体 (n = 4469) 最初分为三组进行初步分析：早发（<30 岁；n = 479）；中成人发病（30-59 岁；n = 3478）；和晚发型（> 59 岁；n = 512）。行为症状的发生率在早发症状者中最高，为 26% (n = 126)，相比之下，中成人发病率为 19% (n = 678)，晚发病为 11% (n = 56)。磷 < 0.0001)。细化分析，跨年龄连续体而不是分类亚组之间发现，发病年龄增加一年与行为症状被回顾性报告为表现症状的几率降低 5.6% 相关（P  < 0.0001） . 到研究登记时，报告的发病年龄每增加一年，报告严重行为症状的几率就会降低 5.5%。探索影响发病年龄的环境、遗传和表观遗传因素并进一步表征行为症状的类型和严重程度，可能会改善治疗和了解亨廷顿病对受影响个体的影响。