Institute of Physiology (Research Unit iDN-interdisciplinary Developmental Neuroscience; IN:spired), Center for Physiological Medicine, Medical University of Graz, Austria.
Res Dev Disabil. 2013 Oct;34(10):3133-8. doi: 10.1016/j.ridd.2013.06.040. Epub 2013 Jul 24.
Rett syndrome (RTT) is a severe neurological disorder characterized by a developmental regression in motor and speech-language domains. There is, however, limited research on socio-communicative development of affected children before the onset of regression. We analyzed audio-video recordings made by parents of six 9- to 12-month old girls later diagnosed with typical RTT, applying the Inventory of Potential Communicative Acts (IPCA) to identify early communicative forms and functions. Each girl used at least one communicative form (e.g., body movement, eye gaze, or vocalizations) to gain attention and answer, but none were observed to make choices or request information. Varying numbers of children were observed to perform other communicative functions according to the IPCA including social convention, rejecting or requesting an object. Non-verbal forms (e.g., reaching, moving closer, eye contact, smiling) were more common than non-linguistic verbal forms (e.g., unspecified vocalizations, pleasure vocalizations, crying). (Pre-)linguistic verbal forms (e.g., canonical or variegated babbling, proto-words) were not used for communicative purposes. These data suggest that atypical developmental patterns in the socio-communicative domain are evident prior to regression in young individuals later diagnosed with RTT.
雷特综合征(RTT)是一种严重的神经发育障碍,其特征是运动和言语语言领域的发育倒退。然而,对于在回归前受影响儿童的社会交际发展,相关研究有限。我们分析了后来被诊断为典型 RTT 的 6 名 9 至 12 个月大女孩的父母录制的音频视频,应用潜在交际行为量表(IPCA)识别早期交际形式和功能。每个女孩都至少使用一种交际形式(例如,身体动作、眼神注视或发声)来吸引注意力并回答问题,但没有观察到她们做出选择或请求信息。根据 IPCA,不同数量的孩子被观察到执行其他交际功能,包括社会惯例、拒绝或请求物品。非言语形式(例如,伸手、靠近、眼神接触、微笑)比非语言言语形式(例如,未指定的发声、愉悦发声、哭泣)更常见。(前)语言言语形式(例如,规范或杂化的咿呀学语、原词)没有用于交际目的。这些数据表明,在后来被诊断为 RTT 的年轻个体出现回归之前,其社会交际领域就已经出现了异常的发展模式。