Quest Kelsey M, Byiers Breanne J, Payen Ameante, Symons Frank J
Department of Educational Psychology, University of Minnesota, United States.
Department of Educational Psychology, University of Minnesota, United States.
Res Dev Disabil. 2014 May;35(5):1191-7. doi: 10.1016/j.ridd.2014.01.011. Epub 2014 Feb 25.
Rett syndrome (RTT) is a neurodevelopmental disorder primarily affecting females. It is characterized by apparently normative development of motor and communicative abilities followed by deterioration in these domains. Stereotypic hand movements are one of the core diagnostic criteria for RTT. There is some anecdotal but limited scientific evidence that changes in hand stereotypy may be a sign of increased anxiety or arousal (i.e., a 'stress response') in RTT. Understanding stress responsivity is difficult in RTT because almost all individuals are nonverbal or otherwise severely communicatively impaired. This study used direct behavioral observation to quantify and compare the frequency of hand stereotypy and signs of negative affect during presumed periods of high and low stress associated with functional analysis conditions (negative reinforcement ['escape'] and control ['free play'], respectively) for 5 females with RTT (mean age=17.8; range 4-47). Negative affect was more likely to occur during negative reinforcement ('stress') conditions for each participant whereas hand stereotypies did not differ across conditions for any of the participants. Although preliminary, the results suggest that hand stereotypy may not be a valid behavioral 'stress-response' indicator in females with RTT. Alternatively, the approach we used may have been limited and not sufficient to evoke a stress response. Either way, more work with direct relevance to improving our understanding of hand stereotypy and anxiety in RTT in relation to social context appears warranted.
雷特综合征(RTT)是一种主要影响女性的神经发育障碍。其特征是运动和沟通能力在早期发育正常,随后在这些方面出现退化。刻板手部动作是雷特综合征的核心诊断标准之一。有一些传闻但有限的科学证据表明,手部刻板动作的变化可能是雷特综合征患者焦虑或觉醒增加(即“应激反应”)的迹象。在雷特综合征中,理解应激反应性很困难,因为几乎所有患者都无法言语交流或存在严重的沟通障碍。本研究采用直接行为观察法,对5名雷特综合征女性患者(平均年龄 = 17.8岁;年龄范围4 - 47岁)在与功能分析条件(分别为负强化['逃避']和对照['自由玩耍'])相关的假定高应激和低应激期间的手部刻板动作频率和消极情绪迹象进行量化和比较。对于每位参与者,消极情绪在负强化(“应激”)条件下更有可能出现,而手部刻板动作在任何参与者的不同条件下并无差异。尽管这只是初步结果,但表明手部刻板动作可能不是雷特综合征女性患者有效的行为“应激反应”指标。或者,我们使用的方法可能存在局限性,不足以引发应激反应。无论哪种情况,开展更多与改善我们对雷特综合征中手部刻板动作及焦虑与社会背景关系的理解直接相关的研究似乎很有必要。