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Abstract：
Huntington\'s disease (HD) is an autosomal dominant inherited neurodegenerative disorder caused by CAG repeats expansion. Cognitive decline contributes to the loss of daily activity in manifest HD. We aimed to examine the cognition status in a Chinese HD cohort and explore factors influencing the diverse cognitive domains.
A total of 205 participants were recruited in the study with the assessment by neuropsychological batteries, including the mini-mental state examination (MMSE), Stroop test, symbol digit modalities test (SDMT), trail making test (TMT), verbal fluency test (VFT), and Hopkins verbal learning test-revised, as well as motor and psychiatric assessment. Pearson correlation and multiple linear regression models were applied to investigate the correlation.
Only 41.46% of patients had normal global function first come to our center. There was a significantly difference in MMSE, Stroop test, SDMT, TMT, and VFT across each stage of HD patients (p < .05). Apathy of PBA-s was correlated to MMSE, animal VFT and Stroop-interference tests performance. Severity of motor symptoms, functional capacity, age, and age of motor symptom onset were correlated to all neuropsychological scores, whereas education attainment and diagnostic delay were correlated to most neuropsychological scores except TMT. Severity of motor symptoms, functional capacity, and education attainment showed independent predicting effect (p < .05) in diverse cognitive domains.
Cognitive impairment was very common in Chinese HD patients at the first visit and worse in the patients in advanced phase. The severity of motor symptoms and functional capacity were correlated to the diverse cognitive domains.
A total of 205 participants were recruited in the study with the assessment by neuropsychological batteries, including the mini-mental state examination (MMSE), Stroop test, symbol digit modalities test (SDMT), trail making test (TMT), verbal fluency test (VFT), and Hopkins verbal learning test-revised, as well as motor and psychiatric assessment. Pearson correlation and multiple linear regression models were applied to investigate the correlation.
Only 41.46% of patients had normal global function first come to our center. There was a significantly difference in MMSE, Stroop test, SDMT, TMT, and VFT across each stage of HD patients (p < .05). Apathy of PBA-s was correlated to MMSE, animal VFT and Stroop-interference tests performance. Severity of motor symptoms, functional capacity, age, and age of motor symptom onset were correlated to all neuropsychological scores, whereas education attainment and diagnostic delay were correlated to most neuropsychological scores except TMT. Severity of motor symptoms, functional capacity, and education attainment showed independent predicting effect (p < .05) in diverse cognitive domains.
Cognitive impairment was very common in Chinese HD patients at the first visit and worse in the patients in advanced phase. The severity of motor symptoms and functional capacity were correlated to the diverse cognitive domains.
摘要：
目的：亨廷顿病（HD）是一种由CAG重复序列扩增引起的常染色体显性遗传性神经退行性疾病。认知能力下降会导致明显HD的日常活动丧失。我们旨在研究中国HD队列中的认知状况，并探索影响不同认知领域的因素。
方法：共招募了205名参与者，通过神经心理学电池进行评估，包括小型精神状态检查(MMSE)，Stroop测试,符号数字模态测试(SDMT)，跟踪测试(TMT)，口语流利度测试(VFT)和霍普金斯口头学习测试修订，以及运动和精神评估。采用Pearson相关和多元线性回归模型进行相关性研究。
结果：只有41.46%的患者总体功能正常。MMSE有显著差异，Stroop测试,SDMT,TMT,各阶段HD患者的VFT（p<0.05）。PBA-s的冷漠与MMSE相关，动物VFT和Stroop干扰测试性能。运动症状的严重程度,功能能力，年龄,运动症状发作的年龄与所有神经心理学评分相关，而受教育程度和诊断延迟与除TMT外的大多数神经心理学评分相关。运动症状的严重程度,功能能力，教育程度在不同的认知领域表现出独立的预测效应(p<.05)。
结论：认知障碍在中国HD患者首次就诊时非常常见，在晚期患者中更为严重。运动症状的严重程度和功能能力与不同的认知领域相关。
方法：共招募了205名参与者，通过神经心理学电池进行评估，包括小型精神状态检查(MMSE)，Stroop测试,符号数字模态测试(SDMT)，跟踪测试(TMT)，口语流利度测试(VFT)和霍普金斯口头学习测试修订，以及运动和精神评估。采用Pearson相关和多元线性回归模型进行相关性研究。
结果：只有41.46%的患者总体功能正常。MMSE有显著差异，Stroop测试,SDMT,TMT,各阶段HD患者的VFT（p<0.05）。PBA-s的冷漠与MMSE相关，动物VFT和Stroop干扰测试性能。运动症状的严重程度,功能能力，年龄,运动症状发作的年龄与所有神经心理学评分相关，而受教育程度和诊断延迟与除TMT外的大多数神经心理学评分相关。运动症状的严重程度,功能能力，教育程度在不同的认知领域表现出独立的预测效应(p<.05)。
结论：认知障碍在中国HD患者首次就诊时非常常见，在晚期患者中更为严重。运动症状的严重程度和功能能力与不同的认知领域相关。
