Summers Jane
Hospital for Sick Children , Toronto , Ontario , Canada.
Department of Psychiatry, University of Toronto , Toronto , ON , Canada.
Dev Neurorehabil. 2019 Nov;22(8):516-526. doi: 10.1080/17518423.2019.1619857. Epub 2019 May 22.
This study was designed to assess memory, imitation of motor actions and motor performance by 12 children (age range 40-151 months) with Angelman syndrome (AS), a rare neurogenetic disorder associated with learning and memory impairments. : Children's functioning was assessed at several time points over a 3-month period. : Memory and motor performance tests had acceptable test-retest and inter-rater reliability whereas the motor imitation test did not. Children were able to recall action sequences after a 24-h delay. Memory and motor performance scores were correlated with children's chronological age and raw scores on subdomains of the Vineland-II. : These behavioral tests require further development and evaluation but may show promise to accompany standardized assessments that are currently in use with children with AS.
本研究旨在评估12名患有天使综合征(AS)的儿童(年龄范围为40 - 151个月)的记忆、运动动作模仿和运动表现。天使综合征是一种罕见的神经遗传疾病,与学习和记忆障碍有关。在3个月的时间里,在多个时间点对儿童的功能进行了评估。记忆和运动表现测试具有可接受的重测信度和评分者间信度,而运动模仿测试则没有。儿童能够在24小时延迟后回忆动作序列。记忆和运动表现得分与儿童的实际年龄以及温兰德社会适应量表第二版(Vineland-II)子领域的原始得分相关。这些行为测试需要进一步开发和评估,但可能有望辅助目前用于评估天使综合征儿童的标准化评估。