School of Psychology, The University of Queensland, Brisbane, Queensland, Australia.
Queensland Brain Institute, The University of Queensland, Brisbane, Queensland, Australia.
J Neuropsychol. 2024 Jun;18(2):265-285. doi: 10.1111/jnp.12354. Epub 2023 Nov 23.
Amyotrophic lateral sclerosis (ALS) is a multi-system disorder that commonly affects cognition and behaviour. Verbal fluency impairments are consistently reported in ALS patients, and we aimed to investigate whether this deficit extends beyond the verbal domain. We further aimed to determine whether deficits are underpinned by a primary intrinsic response generation impairment (i.e., a global reduction across tasks), potentially related to apathy, or an inability to maintain responding over time (i.e., a 'drop off' pattern). Twenty-two ALS patients and 21 demographically-matched controls completed verbal and nonverbal fluency tasks (phonemic/semantic word fluency, design fluency, gesture fluency and ideational fluency), requiring the generation of responses over a specified time period. Fluency performance was analysed in terms of the overall number of novel items produced, as well as the number of items produced in the first 'initiation' and the remaining 'maintenance' time periods. ALS patients' overall performance was not globally reduced across tasks. Patients were impaired only on meaningful gesture fluency, which requires the generation of gestures that communicate meaning (e.g., waving). On phonemic fluency, ALS patients showed a 'drop off' pattern of performance, where they had difficulty maintaining responding over time, but this pattern was not evident on the other fluency tasks. Apathy did not appear to be related to fluency performance. The selective meaningful gesture fluency deficit, in the context of preserved meaningless gesture fluency, highlights that the retrieval of action knowledge may be weakened in early ALS.
肌萎缩性侧索硬化症（ALS）是一种多系统疾病，通常会影响认知和行为。ALS 患者普遍存在言语流畅性障碍，我们旨在研究这种缺陷是否超出言语领域。我们还旨在确定缺陷是否是由原发性内在反应生成障碍（即，跨任务的整体减少）引起的，这种障碍可能与淡漠有关，或者是无法随着时间的推移维持反应（即“下降”模式）。22 名 ALS 患者和 21 名年龄匹配的对照组完成了言语和非言语流畅性任务（语音/语义词汇流畅性、设计流畅性、手势流畅性和意念流畅性），需要在规定的时间内生成反应。流畅性表现的分析包括生成的新颖项目总数，以及在第一个“启动”和剩余“维持”时间段内生成的项目数量。ALS 患者的整体表现并没有在所有任务中都降低。患者仅在手语流畅性上存在障碍，这需要生成传达意义的手势（例如挥手）。在语音流畅性方面，ALS 患者表现出“下降”的反应模式，他们随着时间的推移难以维持反应，但在其他流畅性任务中，这种模式并不明显。淡漠似乎与流畅性表现无关。在保留无意义手势流畅性的情况下，选择性的有意义手势流畅性缺陷表明，在早期 ALS 中，动作知识的检索可能会减弱。
