早发型和成人起病型强直性肌营养不良的认知功能
Cognitive function in early adult and adult onset myotonic dystrophy.
作者信息
Van Spaendonck K P, Ter Bruggen J P, Weyn Banningh E W, Maassen B A, Van de Biezenbos J B, Gabreëls F J
机构信息
Department of Medical Psychology, University of Njimegen, The Netherlands.
出版信息
Acta Neurol Scand. 1995 Jun;91(6):456-61. doi: 10.1111/j.1600-0404.1995.tb00446.x.
INTRODUCTION
Most studies revealing intellectual deficits in myotonic dystrophy (MyD) involved heterogeneous groups of patients with respect to intelligence and onset of disease. The present study was undertaken to investigate whether patients with early adult and adult MyD show subtle cognitive deficits despite a normal intelligence.
MATERIALS AND METHODS
We compared 26 MyD patients of normal intelligence with mild symptoms and early adult and adult onset to 25 matched control subjects (CS) on a range of neuropsychological tests and a number of motor tasks of increasing complexity, which required increasing cognitive control.
RESULTS
The groups did not differ as far as the neuropsychological tests were concerned, with the exception of the Stroop Color Word Test. With respect to motor performance, the MyD patients were poorer scorers on simple and automatic motor tasks than CS, but the difference disappeared as the complexity of the tasks increased and required correspondingly more cognitive control.
CONCLUSION
We found hardly any evidence of cognitive dysfunction in our group of MyD patients with early adult and adult onset.
引言
大多数揭示强直性肌营养不良（MyD）患者存在智力缺陷的研究涉及智力和发病情况各异的患者群体。本研究旨在调查成年早期和成年期MyD患者在智力正常的情况下是否存在细微的认知缺陷。
材料与方法
我们将26名智力正常、症状轻微且为成年早期和成年期发病的MyD患者与25名匹配的对照受试者（CS）进行了一系列神经心理学测试以及一些难度逐渐增加的运动任务比较，这些任务需要越来越多的认知控制。
结果
就神经心理学测试而言，除了斯特鲁普颜色词测试外，两组之间没有差异。在运动表现方面，MyD患者在简单和自动运动任务上的得分比CS低，但随着任务复杂性增加并相应需要更多认知控制时，这种差异消失了。
结论
我们几乎没有发现成年早期和成年期发病的MyD患者组存在认知功能障碍的证据。
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