Department of Neurology, Vanderbilt University Medical Center, Nashville, Tennessee.
Cogn Behav Neurol. 2022 Mar 3;35(1):40-48. doi: 10.1097/WNN.0000000000000293.
Anosognosia can manifest as an unawareness of neurobehavioral symptoms in individuals with Huntington disease (HD). Measurement of anosognosia is challenging, but the Anosognosia Scale (AS) represents a brief option with promising findings in small samples.
To replicate application of the AS in a larger HD sample than previous studies in order to assess psychometrics and demographic correlates and to investigate the genetic, motor, and neuropsychological correlates of the AS in individuals with HD.
We retrospectively reviewed the AS ratings of 74 genetically confirmed Huntington gene carriers, nearly all early motor manifest, who had been referred for clinical neuropsychological assessment. Concurrent clinical neurologic examination and neuropsychometric assessment data were compiled, where available (ns = 35-74). The severity of the anosognosia per AS ratings was characterized for the HD sample.
The AS ratings did not correlate with demographic variables, genetic markers, or motor dysfunction severity. Correlation analyses revealed that higher AS ratings correlated with worse recognition-discrimination memory performance (r = 0.38, P < 0.05) but not cognitive control on executive functioning performance or on collateral-reported frontal-behavioral symptoms. Higher AS ratings also correlated with fewer patient-reported depressive symptoms (r = -0.38, P < 0.01) and diurnal hypersomnia symptoms (r = -0.44, P < 0.01).
Anosognosia (per AS) is associated with recognition-discrimination deficits and fewer self-reported neuropsychiatric symptoms in individuals with pre-to-early manifest HD, though not with HD severity per genetic or motor markers, nor to executive dysfunction or collateral-reported frontal-behavioral symptoms.
认知障碍可能表现为亨廷顿病（HD）患者对神经行为症状无意识。认知障碍的测量具有挑战性，但认知障碍量表（AS）是一种简短的选择，在小样本中具有有前途的发现。
在比以前的研究更大的 HD 样本中复制 AS 的应用，以评估心理计量学和人口统计学相关性，并研究 HD 个体中 AS 的遗传、运动和神经心理学相关性。
我们回顾性地审查了 74 名经基因确认的亨廷顿基因携带者的 AS 评分，他们几乎都是早期运动表现，已经被转介进行临床神经心理学评估。在可用的情况下（ns = 35-74），我们汇编了同时进行的临床神经病学检查和神经心理评估数据。为 HD 样本描述了 AS 评分的认知障碍严重程度。
AS 评分与人口统计学变量、遗传标志物或运动功能障碍严重程度无关。相关分析表明，AS 评分越高，识别-辨别记忆表现越差（r = 0.38，P < 0.05），但执行功能表现或间接报告的额叶行为症状的认知控制能力没有差异。AS 评分越高，患者报告的抑郁症状（r = -0.38，P < 0.01）和日间过度嗜睡症状（r = -0.44，P < 0.01）越少。
在预至早期表现的 HD 个体中，认知障碍（根据 AS 评估）与识别-辨别缺陷以及较少的自我报告神经精神症状相关，而与 HD 严重程度无关，无论遗传或运动标志物如何，也与执行功能障碍或间接报告的额叶行为症状无关。
