老年1型神经纤维瘤病患者的神经心理学损害

Neuropsychological impairments in elderly Neurofibromatosis type 1 patients.

作者信息

Costa Danielle de Souza, de Paula Jonas Jardim, de Rezende Nilton Alves, Rodrigues Luiz Oswaldo Carneiro, Malloy-Diniz Leandro Fernandes, Romano-Silva Marco Aurélio, Miranda Débora Marques de

机构信息

INCT de Medicina Molecular, Faculdade de Medicina, Universidade Federal de Minas Gerais, Av. Alfredo Balena, 190, Belo Horizonte, MG, CEP 30130-100, Brazil; Laboratório de Investigações Neuropsicológicas (LIN) do Instituto Nacional de Ciência e Tecnologia em Medicina Molecular, Faculdade de Medicina, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brazil.

出版信息

Eur J Med Genet. 2014 Apr;57(5):216-9. doi: 10.1016/j.ejmg.2014.02.004. Epub 2014 Feb 18.

DOI:10.1016/j.ejmg.2014.02.004

PMID:24556498

Abstract

Cognitive performance is compromised in Neurofibromatosis type 1 (NF1) patients, but neuropsychological data including elderly NF1 are extremely sparse. We compared the cognitive performance of a small elderly NF1 group (n = 5) with an age-matched healthy control group (n = 49). NF1 group performed worse than control group on a global cognitive impairment task, verbal working memory, and visuospatial functioning. The results suggest that cognitive impairment is an important feature of NF1 across lifespan, including elderly individuals. Future studies approaching the NF1 cognitive profile might benefit from looking at the mechanisms linked to the age-related aspects of cognitive decline.

摘要

1型神经纤维瘤病（NF1）患者的认知功能受损，但包括老年NF1患者在内的神经心理学数据极为稀少。我们将一小群老年NF1患者（n = 5）的认知表现与年龄匹配的健康对照组（n = 49）进行了比较。在整体认知障碍任务、言语工作记忆和视觉空间功能方面，NF1组的表现比对照组差。结果表明，认知障碍是NF1患者整个生命周期的一个重要特征，包括老年人。未来关于NF1认知特征的研究可能会受益于研究与认知衰退的年龄相关方面相关的机制。