雷特综合征女性的听觉感觉记忆持续时间严重缩短。
Auditory sensory memory span for duration is severely curtailed in females with Rett syndrome.
机构信息
The Cognitive Neurophysiology Laboratory, Ernest J. Del Monte Institute for Neuroscience, Department of Neuroscience, University of Rochester School of Medicine and Dentistry, Rochester, NY, USA.
The Cognitive Neurophysiology Laboratory, Department of Pediatrics, Albert Einstein College of Medicine & Montefiore Medical Center, Bronx, NY, USA.
出版信息
Transl Psychiatry. 2019 Apr 9;9(1):130. doi: 10.1038/s41398-019-0463-0.
Rett syndrome (RTT), a rare neurodevelopmental disorder caused by mutations in the MECP2 gene, is typified by profound cognitive impairment and severe language impairment, rendering it very difficult to accurately measure auditory processing capabilities behaviorally in this population. Here we leverage the mismatch negativity (MMN) component of the event-related potential to measure the ability of RTT patients to decode and store occasional duration deviations in a stream of auditory stimuli. Sensory memory for duration, crucial for speech comprehension, has not been studied in RTT.High-density electroencephalography was successfully recorded in 18 females with RTT and 27 age-matched typically developing (TD) controls (aged 6-22 years). Data from seven RTT and three TD participants were excluded for excessive noise. Stimuli were 1 kHz tones with a standard duration of 100 ms and deviant duration of 180 ms. To assess the sustainability of sensory memory, stimulus presentation rate was varied with stimulus onset asynchronies (SOAs) of 450, 900, and 1800 ms. MMNs with maximum negativity over fronto-central scalp and a latency of 220-230 ms were clearly evident for each presentation rate in the TD group, but only for the shortest SOA in the RTT group. Repeated-measures ANOVA revealed a significant group by SOA interaction. MMN amplitude correlated with age in the TD group only. MMN amplitude was not correlated with the Rett Syndrome Severity Scale. This study indicates that while RTT patients can decode deviations in auditory duration, the span of this sensory memory system is severely foreshortened, with likely implications for speech decoding abilities.
雷特综合征(RTT)是一种由 MECP2 基因突变引起的罕见神经发育障碍,其特征为严重的认知和语言障碍,使得在该人群中很难通过行为准确测量听觉处理能力。在这里,我们利用事件相关电位中的失匹配负波(MMN)成分来衡量 RTT 患者解码和存储听觉刺激流中偶尔出现的时长偏差的能力。时长的感觉记忆对于言语理解至关重要,但尚未在 RTT 中进行研究。我们成功地在 18 名患有 RTT 的女性和 27 名年龄匹配的典型发育(TD)对照组(年龄 6-22 岁)中记录了高密度脑电图。有 7 名 RTT 和 3 名 TD 参与者的数据因噪声过大而被排除在外。刺激为 1 kHz 音调,标准持续时间为 100 ms,偏差持续时间为 180 ms。为了评估感觉记忆的可持续性,我们改变了刺激呈现率,刺激呈现的时间间隔(SOA)分别为 450、900 和 1800 ms。在 TD 组中,每个呈现率的额中央头皮上都有明显的最大负性的 MMN,潜伏期为 220-230 ms,但在 RTT 组中只有最短的 SOA 才有。重复测量方差分析显示,组间存在显著的 SOA 交互作用。MMN 振幅仅与 TD 组的年龄相关。MMN 振幅与 Rett 综合征严重程度量表无相关性。这项研究表明,尽管 RTT 患者可以解码听觉时长的偏差,但这种感觉记忆系统的跨度严重缩短,这可能对言语解码能力产生影响。
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