Hinton V J, De Vivo D C, Nereo N E, Goldstein E, Stern Y
Department of Neurology, the College of Physicians and Surgeons, Columbia University, New York, NY 10032, USA.
Neurology. 2000 Jun 13;54(11):2127-32. doi: 10.1212/wnl.54.11.2127.
To determine whether all boys with Duchenne muscular dystrophy (DMD) have a similar verbal and memory profile of skills, or whether only a subset is affected, and to determine whether the weak areas in their profile are substantially different from a control group.
Performance of patients with DMD on neuropsychological tests of verbal and memory skills was examined in two ways. Standardized test scores for 80 boys with DMD (estimated IQ range, 70 to 160) were ranked individually from worst to best, and the individual rankings were compared across the group using Friedman rank analysis. Additionally, performance of 41 boys with DMD was compared with that of their sibling control subjects of similar age and estimated IQ using multivariate analysis of variance.
Individual cognitive profiles were significantly similar among the subjects with DMD, such that for most subjects digit span, story recall, and comprehension were the tests on which each performed most poorly. This finding remained true regardless of whether they were of high or low intellectual function. In contrast, no significant cognitive profile was found among their sibling control subjects, and when compared with their siblings, the DMD group scored significantly more poorly on digit span, comprehension, and story recall, but not on other verbal and memory measures.
Boys with DMD have a specific cognitive profile, regardless of their general level of cognitive function. Specifically, boys with DMD performed more poorly on tests requiring attention to complex verbal information than they did on other verbal or memory measures. The possibility that the missing dystrophin brain products may contribute to selective cognitive processing is considered.
确定所有杜氏肌营养不良症（DMD）男孩是否具有相似的语言和记忆技能特征，还是只有一部分受到影响，并确定他们特征中的薄弱领域与对照组是否存在显著差异。
通过两种方式检查DMD患者在语言和记忆技能神经心理学测试中的表现。对80名DMD男孩（估计智商范围为70至160）的标准化测试分数从最差到最佳进行个体排名，并使用弗里德曼秩和检验对整个组的个体排名进行比较。此外，使用多变量方差分析将41名DMD男孩的表现与其年龄和估计智商相似的同胞对照受试者的表现进行比较。
DMD受试者之间的个体认知特征显著相似，以至于对于大多数受试者来说，数字广度、故事回忆和理解是他们表现最差的测试项目。无论他们的智力功能高低，这一发现都成立。相比之下，在他们的同胞对照受试者中未发现显著的认知特征，并且与他们的同胞相比，DMD组在数字广度、理解和故事回忆方面的得分明显更低，但在其他语言和记忆测量方面并非如此。
患有DMD的男孩具有特定的认知特征，无论其总体认知功能水平如何。具体而言，患有DMD的男孩在需要关注复杂语言信息的测试中表现比在其他语言或记忆测量中更差。考虑了缺失的肌营养不良蛋白脑产物可能有助于选择性认知加工的可能性。
