Cogonitive function in Duchenne muscular dystrophy

杜氏肌营养不良症的认知功能

• 批准号: 05451044
• 负责人: FUJITA Kazuhiro
• 金额: $ 2.5万
• 依托单位: University of Tsukuba
• 依托单位国家: 日本
• 项目类别: Grant-in-Aid for General Scientific Research (B)
• 财政年份: 1993
• 资助国家: 日本
• 起止时间: 1993 至 1994
• 项目状态: 已结题
• 来源: https://kaken.nii.ac.jp/en/grant/KAKENHI-PROJECT-05451044/
• 关键词: Duchenne muscular dystrophy; cognitive function; K-ABC; CAI

Duchenne muscular dystrophy (DMD) is a chromosome X-linked inherited disease. DMDchildren have moderate cognitive impairment, but the underlying mechanisms are unknown. Many studies raised the possibility that cognitive abnormalities in DMD are attributable to synaptic dysfunction associated with deficits in brain dystrophin molecules. In order to clarify cognitive function in DMD,we performed new cognitive studies.In the first study, Twenty-two male patients with DMD aged 5-9 yeaes were tested with the Japanese WISC-R and the Japanese K-ABC mental processing subtests. male patients with DMD showed a discrepancy between verbal and performance IQ (VIQ<PIQ) on the WISC-R and a significant difference between Simultaneous Processing and Sequential Processing standard score (Simultaneous>Sequential) on the K-ABC.In the second study, a female patient with DMD and a female carrier were tested with the Japanese K-ABC.They also showed significant difference between Simultaneous. Processing and Sequential Processing standard score (Simultaneous>Sequential) on the K-ABC.In the third study, we developed the CAI program which emphasized visual cues for DMD children.

杜氏肌营养不良症 (DMD) 是一种 X 染色体连锁遗传病。 DMD儿童存在中度认知障碍，但其潜在机制尚不清楚。许多研究提出了 DMD 认知异常可能归因于与脑肌营养不良蛋白分子缺陷相关的突触功能障碍。为了阐明DMD的认知功能，我们进行了新的认知研究。在第一项研究中，22名5-9岁的男性DMD患者接受了日本WISC-R和日本K-ABC心理加工子测试。男性DMD患者在WISC-R上表现出言语智商和表现智商（VIQ<PIQ）之间的差异，在K-ABC上同时处理和顺序处理标准得分（Simultaneous>Sequential）之间存在显着差异。在第二项研究中，一名女性DMD患者和一名女性携带者在日本K-ABC上进行了测试，他们在同步处理之间也表现出显着差异。 K-ABC 上的处理和顺序处理标准分数（同时>顺序）。在第三项研究中，我们开发了强调 DMD 儿童视觉线索的 CAI 程序。