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成年莫比乌斯综合征患者的认知评估。

Cognitive evaluation in adult patients with Möbius syndrome.

作者信息

Verzijl H T F M, van Es N, Berger H J C, Padberg G W, van Spaendonck K P M

机构信息

Department of Neurology, University Medical Center Nijmegen, P.O. Box 9101, 6500 HB Nijmegen, The Netherlands.

出版信息

J Neurol. 2005 Feb;252(2):202-7. doi: 10.1007/s00415-005-0637-y.

Abstract

OBJECTIVE

To establish the occurrence of mental retardation in a group of patients with Möbius syndrome and subsequently, if mental retardation is absent, to screen major aspects of memory and attention, in order to assess possible pervasive dysfunction in these cognitive domains which might be responsible for the current view that mental retardation occurs frequently in Möbius syndrome.

METHODS

In a group of 12 Dutch Möbius patients, intellectual performance, memory function and attention were assessed using a number of standardized neuropsychological tests.

RESULTS

The mean general intellectual performance did not differ significantly from that of the Dutch population. Screening of selective attention and memory did not provide indications of pervasive dysfunctions in these domains.

CONCLUSION

The rate of occurrence of mental retardation in our group of Möbius patients did not differ from that in the normal Dutch population. Furthermore, there was no evidence of attention and memory dysfunction in our group of Möbius patients.

摘要

目的

确定一组莫比乌斯综合征患者中智力发育迟缓的发生率;若不存在智力发育迟缓,则对记忆和注意力的主要方面进行筛查,以评估这些认知领域可能存在的普遍功能障碍,这种功能障碍可能导致目前认为莫比乌斯综合征患者经常出现智力发育迟缓的观点。

方法

对12名荷兰莫比乌斯综合征患者,使用多种标准化神经心理学测试评估其智力表现、记忆功能和注意力。

结果

平均总体智力表现与荷兰人群无显著差异。对选择性注意力和记忆的筛查未显示这些领域存在普遍功能障碍。

结论

我们这组莫比乌斯综合征患者中智力发育迟缓的发生率与正常荷兰人群无异。此外,我们这组莫比乌斯综合征患者没有注意力和记忆功能障碍的证据。

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