Maes B, Fryns J P, Van Walleghem M, Van den Berghe H
Center for Human Genetics, University Hospital Gasthuisberg, Leuven, Belgium.
Am J Med Genet. 1994 Apr 1;50(2):190-200. doi: 10.1002/ajmg.1320500211.
The present study describes cognitive skills and information processing strategies of mentally retarded fra(X) men. Fifty-eight fra(X) positive and 58 fra(X) negative adults, matched on sex, chronological age, length of institutionalisation, general cognitive level, and living conditions, were evaluated with the Bayley or McCarthy Scales of Mental Abilities. Mental ages were mostly situated in the severe mental handicap category and were found to be negatively influenced by chronological age. A relative strength in perceptual performance and non-verbal reasoning and a deficit in sequential information processing turned out to be typical of all mentally retarded subjects, irrespective of fra(X) or control status. Fra(X) adults could be significantly differentiated from control persons on the ground of a higher level of acquired knowledge because of better vocabulary and verbal-expressive skills. On the other hand, they were less able to imitate non-verbal patterns, had more difficulty with visual-motor integration and co-ordination, and applied less efficient general mental processing skills in solving new problems. The memory profile of fra(X) adults was strongly determined by the meaning and the complexity of the information that has to be reproduced. In this article the profile of cognitive strengths and weaknesses in the fra(X) group will be discussed and some general advice for training is suggested.
本研究描述了脆性X综合征男性智力障碍者的认知技能和信息处理策略。选取了58名脆性X综合征阳性和58名脆性X综合征阴性的成年人，这些人在性别、实际年龄、机构收容时长、一般认知水平和生活条件方面相匹配，采用贝利或麦卡锡智力量表对他们进行评估。心理年龄大多处于重度智力障碍范畴，且发现受实际年龄的负面影响。所有智力障碍受试者，无论是否患有脆性X综合征或作为对照，其典型特征都是在感知表现和非言语推理方面相对较强，而在序列信息处理方面存在缺陷。脆性X综合征成年人由于词汇量和言语表达能力较好，在已掌握知识水平方面显著高于对照人群。另一方面，他们模仿非言语模式的能力较差，在视觉运动整合与协调方面存在更多困难，并且在解决新问题时运用的一般心理处理技能效率较低。脆性X综合征成年人的记忆特征很大程度上取决于需要再现的信息的意义和复杂性。本文将讨论脆性X综合征组的认知优势和劣势特征，并提出一些一般性的训练建议。
