丘脑纹状体T2加权高信号（不明亮物体）与1型神经纤维瘤病患儿的认知障碍相关。

Thalamo-striatal T2-weighted hyperintensities (unidentified bright objects) correlate with cognitive impairments in neurofibromatosis type 1 during childhood.

作者信息

Chabernaud Camille, Sirinelli Dominique, Barbier Charlotte, Cottier Jean-Philippe, Sembely Catherine, Giraudeau Bruno, Deseille-Turlotte Geraldine, Lorette Gerard, Barthez Marie-Anne, Castelnau Pierre

机构信息

Pediatric Neurology Unit, Clocheville Hospital, Tours, France.

出版信息

Dev Neuropsychol. 2009;34(6):736-48. doi: 10.1080/87565640903265137.

DOI:10.1080/87565640903265137

PMID:20183730

Abstract

Learning disabilities represent the main childhood complication in neurofibromatosis type 1 (NF1). Patients frequently exhibit T2-weighted hyperintensities called unidentified bright objects (UBOs) on brain magnetic resonance imaging (MRI), with unclear relationship to such cognitive disabilities. This study aimed to determine whether thalamo-striatal UBOs correlate with cognitive disturbances. Thirty-seven NF1 children were studied: 24 with UBOs (18 of which were thalamo-striatal UBOs), and 13 without UBOs. NF1 subjects carrying thalamo-striatal UBOs had significantly lower IQs and visuospatial performances than those without UBOs in this location. These results suggest that UBOs may contribute to NF1 cognitive impairments through thalamo-cortical dysfunction.

摘要

学习障碍是1型神经纤维瘤病（NF1）主要的儿童期并发症。患者在脑部磁共振成像（MRI）上常表现为T2加权高信号，称为不明亮物体（UBOs），其与这类认知障碍的关系尚不清楚。本研究旨在确定丘脑纹状体UBOs是否与认知障碍相关。对37名NF1儿童进行了研究：24名有UBOs（其中18名是丘脑纹状体UBOs），13名没有UBOs。携带丘脑纹状体UBOs的NF1受试者在智商和视觉空间表现方面显著低于该部位没有UBOs的受试者。这些结果表明，UBOs可能通过丘脑皮质功能障碍导致NF1认知障碍。