PDF(Pubmed)
Abstract：
We interrogated auditory sensory memory capabilities in individuals with CLN3 disease (juvenile neuronal ceroid lipofuscinosis), specifically for the feature of \"duration\" processing. Given decrements in auditory processing abilities associated with later-stage CLN3 disease, we hypothesized that the duration-evoked mismatch negativity (MMN) of the event related potential (ERP) would be a marker of progressively atypical cortical processing in this population, with potential applicability as a brain-based biomarker in clinical trials.
We employed three stimulation rates (fast: 450 ms, medium: 900 ms, slow: 1800 ms), allowing for assessment of the sustainability of the auditory sensory memory trace. The robustness of MMN directly relates to the rate at which the regularly occurring stimulus stream is presented. As presentation rate slows, robustness of the sensory memory trace diminishes. By manipulating presentation rate, the strength of the sensory memory trace is parametrically varied, providing greater sensitivity to detect auditory cortical dysfunction. A secondary hypothesis was that duration-evoked MMN abnormalities in CLN3 disease would be more severe at slower presentation rates, resulting from greater demand on the sensory memory system.
Data from individuals with CLN3 disease (N = 21; range 6-28 years of age) showed robust MMN responses (i.e., intact auditory sensory memory processes) at the medium stimulation rate. However, at the fastest rate, MMN was significantly reduced, and at the slowest rate, MMN was not detectable in CLN3 disease relative to neurotypical controls (N = 41; ages 6-26 years).
Results reveal emerging insufficiencies in this critical auditory perceptual system in individuals with CLN3 disease.
We employed three stimulation rates (fast: 450 ms, medium: 900 ms, slow: 1800 ms), allowing for assessment of the sustainability of the auditory sensory memory trace. The robustness of MMN directly relates to the rate at which the regularly occurring stimulus stream is presented. As presentation rate slows, robustness of the sensory memory trace diminishes. By manipulating presentation rate, the strength of the sensory memory trace is parametrically varied, providing greater sensitivity to detect auditory cortical dysfunction. A secondary hypothesis was that duration-evoked MMN abnormalities in CLN3 disease would be more severe at slower presentation rates, resulting from greater demand on the sensory memory system.
Data from individuals with CLN3 disease (N = 21; range 6-28 years of age) showed robust MMN responses (i.e., intact auditory sensory memory processes) at the medium stimulation rate. However, at the fastest rate, MMN was significantly reduced, and at the slowest rate, MMN was not detectable in CLN3 disease relative to neurotypical controls (N = 41; ages 6-26 years).
Results reveal emerging insufficiencies in this critical auditory perceptual system in individuals with CLN3 disease.
摘要：
背景：我们询问了患有CLN3疾病（青少年神经元类脂褐菌病）的个体的听觉感觉记忆能力，专门针对“持续时间”处理的功能。考虑到与晚期CLN3疾病相关的听觉处理能力下降，我们假设事件相关电位（ERP）的持续时间诱发的失配负性（MMN）将是该人群中逐渐非典型皮质处理的标志，在临床试验中作为基于大脑的生物标志物的潜在适用性。
方法：我们采用了三种刺激速率（快速：450毫秒，medium:900ms,慢：1800毫秒），允许评估听觉感觉记忆痕迹的可持续性。MMN的鲁棒性直接与定期发生的刺激流被呈现的速率相关。随着演示率的降低，感觉记忆痕迹的鲁棒性减弱。通过操纵演示率，感觉记忆痕迹的强度是参数变化的，提供更高的灵敏度来检测听觉皮质功能障碍。次要假设是CLN3疾病中持续时间诱发的MMN异常在较慢的表现速率下会更严重，由于对感官记忆系统的需求增加。
结果：来自患有CLN3疾病的个体（N=21；范围6-28岁）的数据显示出强大的MMN反应（即，完整的听觉感觉记忆过程）在中等刺激速率下。然而,以最快的速度,MMN显著降低,以最慢的速度，相对于神经典型对照（N=41；年龄6-26岁），在CLN3疾病中未检测到MMN。
结论：结果显示，在患有CLN3疾病的个体中，这种关键的听觉感知系统出现了不足。
方法：我们采用了三种刺激速率（快速：450毫秒，medium:900ms,慢：1800毫秒），允许评估听觉感觉记忆痕迹的可持续性。MMN的鲁棒性直接与定期发生的刺激流被呈现的速率相关。随着演示率的降低，感觉记忆痕迹的鲁棒性减弱。通过操纵演示率，感觉记忆痕迹的强度是参数变化的，提供更高的灵敏度来检测听觉皮质功能障碍。次要假设是CLN3疾病中持续时间诱发的MMN异常在较慢的表现速率下会更严重，由于对感官记忆系统的需求增加。
结果：来自患有CLN3疾病的个体（N=21；范围6-28岁）的数据显示出强大的MMN反应（即，完整的听觉感觉记忆过程）在中等刺激速率下。然而,以最快的速度,MMN显著降低,以最慢的速度，相对于神经典型对照（N=41；年龄6-26岁），在CLN3疾病中未检测到MMN。
结论：结果显示，在患有CLN3疾病的个体中，这种关键的听觉感知系统出现了不足。
