Billard C, Gillet P, Signoret J L, Uicaut E, Bertrand P, Fardeau M, Barthez-Carpentier M A, Santini J J
Unité de Neuro-chirurgie Neurologie infantile, Hopital Clocheville, Tours, France.
Neuromuscul Disord. 1992;2(5-6):371-8. doi: 10.1016/s0960-8966(06)80008-8.
In order to clarify cognitive functions in Duchenne muscular dystrophy (DMD), we performed a new controlled neuropsychological study. IQ (WISC-R), verbal skills (fluency, confrontation naming and syntax comprehension) and memory abilities (BEM) were studied in two matched groups; 24 DMD children and 17 spinal muscular atrophy (SMA) children aged 12-16 yr. A significant difference appeared between the DMD and SMA patients: only in the DMD group were there significant disabilities in certain specific functions and normal scores in others. Despite similar education, the DMD children more often had significantly greater learning disabilities. There were more DMD left-handers. Verbal IQ was significantly low whereas performance IQ was at a normal level. DMD children also performed poorly in reading tasks and in some memory functions such as story recall and verbal recognition. Specific cognitive disabilities in certain DMD children, not seen in SMA children, suggest a relationship with a DMD genetic disorder.
为了阐明杜氏肌营养不良症（DMD）的认知功能，我们进行了一项新的对照神经心理学研究。对两个匹配组进行了智商（WISC-R）、语言技能（流畅性、对物命名和句法理解）以及记忆能力（BEM）的研究；24名12至16岁的DMD儿童和17名脊髓性肌萎缩症（SMA）儿童。DMD患者和SMA患者之间出现了显著差异：仅在DMD组中，某些特定功能存在明显缺陷，而其他功能得分正常。尽管接受了相似的教育，但DMD儿童更常出现明显更严重的学习障碍。DMD左利手儿童更多。言语智商显著偏低，而操作智商处于正常水平。DMD儿童在阅读任务以及某些记忆功能（如故事回忆和言语识别）方面也表现不佳。某些DMD儿童存在的特定认知缺陷在SMA儿童中未出现，这表明其与DMD遗传疾病有关。
