神经纤维瘤病1型患儿认知功能缺陷的性质和发生率
The nature and frequency of cognitive deficits in children with neurofibromatosis type 1作者机构：Children's Hospital at Westmead Clinical Sciences Building Locked Bag 4001 Westmead NSW 2145 Australia
出 版 物：《世界核心医学期刊文摘（神经病学分册）》 (Digest of the World Core Medical Journals:Clinical Neurology)
年 卷 期：2006年第2卷第3期
页 面：27-27页
学科分类：1002[医学-临床医学] 100205[医学-精神病与精神卫生学] 10[医学]
主 题：神经纤维瘤病 认知功能 执行功能 神经心理 视知觉 重度损伤 全面认知 功能评估 数学能力 空间感觉
摘 要：Objective: To assess the frequency and severity of specific cognitive deficits in children with neurofibromatosis type 1 (NF1) in a large unbiased cohort. Methods: Extensive cognitive assessments were performed in 81 children with NF1 ages 8 to 16 years and their performance was compared with that of 49 unaffected sibling controls. Results: Eighty-one percent of the children with NF1 had moderate to severe impairment in one or more areas of cognitive functioning. Although 51%of children with NF1 performed poorly on tasks of reading, spelling, and mathematics, specific learning disabilities (as defined by IQ achievement discrepancies) were present in only 20%of the children. Sustained attention difficulties were present in 63%of children with NF1, with 38%of children with NF1 fulfilling the diagnostic criteria for attention deficit-hyperact- ivity disorder. The NF1 neuropsychological profile is characterized by deficits in perceptual skills (visuospatial and visuoperceptual), executive functioning (planning and abstract concept formation), and attention (sustained and switching). Interestingly, both verbal and visual memory was unaffected in NF1 children, and their memory skills were in general stronger than their level of general intellectual function. Although both expressive and receptive language skills were significantly impaired in NF1 children, they appeared to be relatively better preserved than visuospatial abilities once IQ is taken into account. Conclusion: There is an extremely high frequency of cognitive problems in children with neurofibromatosis type 1, making cognitive dysfunction the most common complication to affect quality of life in these children.
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