Grant J, Karmiloff-Smith A, Gathercole S A, Paterson S, Howlin P, Davies M, Udwin O
Cogn Neuropsychiatry. 1997 May 1;2(2):81-99. doi: 10.1080/135468097396342.
Williams syndrome (WS), a rare neurodevelopmental disorder of genetic origin, is characterised by a relative advantage of language over more serious deficits in other cognitive domains. In this study the relationship of phonological short-term memory to WS language, in particular vocabulary, was explored. Using Gathercole and Baddeley's Children's Test of Nonword Repetition (CNRep) (Gathercole & Baddeley, 1996), we examined the pattern of performance by WS participants on that task and compared it to performance on measures of receptive language, visuospatial ability, and digit span. In addition, a comparison was made of WS nonword repetition score with those of two groups of individually matched normally developing 5-year-olds, one on the basis of nonverbal test age and the other on the basis of verbal test age. As expected WS repetition scores showed an effect of nonword length but not of phonological complexity. Nonwords that were relatively wordlike were better repeated than nonwords that were less wordlike. CNRep scores were correlated with test ages on TROG, Ravens, and digit span but not with chronological age. Test age on the CNRep was at a similar level to that on TROG, Ravens, and digit span but significantly lower than on BPVS. The results lend support to the view that phonology in WS is a relative strength. In contrast, despite relatively good productive and receptive vocabulary, certain aspects of the processes of word learning in WS do not seem to develop beyond that of normal 4year-olds. The good vocabulary scores of older children and adults with WS may be simply due to their relatively good phonological short-term memory.
威廉姆斯综合征（WS）是一种罕见的遗传性神经发育障碍，其特征是语言能力相对较强，而在其他认知领域存在更严重的缺陷。在本研究中，探讨了语音短期记忆与WS语言，特别是词汇之间的关系。使用加瑟科尔和巴德利的儿童非词重复测试（CNRep）（加瑟科尔和巴德利，1996），我们研究了WS参与者在该任务上的表现模式，并将其与接受性语言、视觉空间能力和数字广度测量的表现进行了比较。此外，还将WS非词重复分数与两组个体匹配的正常发育5岁儿童的分数进行了比较，一组基于非语言测试年龄，另一组基于语言测试年龄。正如预期的那样，WS重复分数显示出非词长度的影响，但没有语音复杂性的影响。相对像单词的非词比不太像单词的非词更容易被重复。CNRep分数与TROG、瑞文推理测验和数字广度的测试年龄相关，但与实际年龄无关。CNRep的测试年龄与TROG、瑞文推理测验和数字广度的测试年龄相似，但明显低于BPVS的测试年龄。结果支持了WS中的语音是一种相对优势的观点。相比之下，尽管WS在产出性和接受性词汇方面相对较好，但WS中单词学习过程的某些方面似乎没有发展到超过正常4岁儿童的水平。年龄较大的WS儿童和成人的良好词汇分数可能仅仅是由于他们相对较好的语音短期记忆。
