高强度
认知功能衰退
神经心理学
心理学
医学
认知
队列
神经心理评估
儿科
听力学
磁共振成像
内科学
神经科学
放射科
痴呆
疾病
作者
James V. Pottala,Kristine Yaffe,Jennifer G. Robinson,Mark A. Espeland,Robert B. Wallace,William S. Harris
出处
期刊:Neurology
[Lippincott Williams & Wilkins]
日期:2014-01-23
卷期号:82 (5): 435-442
被引量:150
标识
DOI:10.1212/wnl.0000000000000080
摘要
Background: Cognitive impairment is the most common complication of neurofibromatosis type 1 (NF1) in childhood. Current research suggests a strong relationship between cognitive deficits and brain T2-hyperintensities. The majority of these lesions disappear as the child ages. Cross-sectional data suggest that there also are improvements in intellect. Objective: To determine the natural history of cognitive functioning and MRI T2-hyperintensities from childhood into adulthood, and whether changes in MRI T2-hyperintensities over time are predictive of changes in cognitive functioning. Methods: The authors conducted a prospective longitudinal study of a cohort of 32 patients with NF1 and 11 unaffected sibling controls. All patients underwent neuropsychological assessments and 27 children underwent MRI examinations. The patients were then reassessed after an 8-year period. Results and Conclusions: There was no improvement in cognitive ability as the children with NF1 developed into adulthood compared with controls. Despite significant decreases in the number, size, and intensity of the T2-hyperintensities over the 8-year period, these changes were not associated with changes in cognitive ability. T2-hyperintensities in the cortex or subcortical or deep white matter are more frequent with age and these lesions are likely to have a different pathology than basal ganglia lesions. The best predictor of cognitive dysfunction in adulthood was the presence of T2-hyperintensities in childhood, rather than current lesion status. There is a limited time window (<18 years) in which the presence of T2-hyperintensities can be used as biologic markers of cognitive dysfunction.
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