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伴有胼胝体发育不全的FG综合征中的语言与发育

Language and development in FG syndrome with callosal agenesis.

作者信息

McCardle P, Wilson B

机构信息

Department of Pediatrics, Uniformed Services University of Health Sciences, Bethesda, Maryland.

出版信息

J Commun Disord. 1993 Jun;26(2):83-100. doi: 10.1016/0021-9924(93)90002-r.

Abstract

The FG syndrome is characterized by unusual facies, sudden infant death, developmental delay, and abnormalities of the cardiac, gastrointestinal, and central nervous systems. No longitudinal data on development in surviving patients are currently available. Serial evaluations of a patient with FG syndrome, whose sole central nervous system anomaly was agenesis of the corpus callosum, showed a consistent pattern over time. Specific language impairments in syntactic and pragmatic-semantic areas are emerging. These findings represent the first detailed data on which expectations for children with the FG syndrome can be based. The findings also fit theoretical constructs on the function of the corpus callosum and may therefore be generalized to provide expectations for other patients with isolated agenesis of the corpus callosum. Given the information gained from this case, it is clear that language intervention/consultation should be a pivotal service for such children, and that the speech-language pathologist should play a role in the development of an integrated educational services plan.

摘要

FG综合征的特征为面容异常、婴儿猝死、发育迟缓以及心脏、胃肠道和中枢神经系统异常。目前尚无关于存活患者发育情况的纵向数据。对一名FG综合征患者进行的系列评估显示,随着时间推移呈现出一致的模式,该患者唯一的中枢神经系统异常是胼胝体发育不全。句法和语用-语义领域的特定语言障碍正在显现。这些发现是首次能据此对FG综合征患儿的预期情况做出判断的详细数据。这些发现也符合关于胼胝体功能的理论架构,因此或许可以推广,为其他孤立性胼胝体发育不全的患者提供预期情况参考。鉴于从该病例中获得的信息,很明显语言干预/咨询对于这类儿童应是一项关键服务,并且言语语言病理学家应在制定综合教育服务计划中发挥作用。

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