Woodward J B, Heaton R K, Simon D B, Ringel S P
J Clin Neuropsychol. 1982 Dec;4(4):335-42. doi: 10.1080/01688638208401141.
Although the literature contains several references to clinically apparent cognitive deficits in patients with myotonic dystrophy (MYD), efforts to support these observations with formal testing have been lacking. The current study compared 17 MYD patients with 25 normal controls on an expanded Halstead-Reitan Battery. The MYD group scored worse than the controls on nearly every neuropsychological measure. Significant neuropsychological impairment was present even when tests of motor skills were excluded. There was no relationship between general neuropsychological impairment and degree of weakness, myotonia, or muscle atrophy in the MYD patients. These findings suggest that cognitive impairment can be an important and relatively independent component of the disability in MYD, which should be considered in the clinical evaluation and counselling of persons with this disease.
虽然文献中有几处提到强直性肌营养不良（MYD）患者存在临床上明显的认知缺陷，但一直缺乏通过正式测试来支持这些观察结果的努力。当前研究使用扩展的Halstead-Reitan成套神经心理测验对17名MYD患者和25名正常对照进行了比较。MYD组在几乎每一项神经心理学测量上的得分都低于对照组。即使排除运动技能测试，仍存在显著的神经心理学损害。MYD患者的一般神经心理学损害与虚弱程度、肌强直或肌肉萎缩之间没有关系。这些发现表明，认知障碍可能是MYD患者残疾的一个重要且相对独立的组成部分，在对该病患者进行临床评估和咨询时应予以考虑。
