Wessel Lauren E, Albers Anne C, Gutmann David H, Dunn Courtney M
1Department of Neurology, Washington University School of Medicine, St. Louis, MO, USA.
J Child Neurol. 2013 Dec;28(12):1664-7. doi: 10.1177/0883073812460918. Epub 2012 Oct 15.
Children with the neurofibromatosis type 1 (NF1) inherited tumor predisposition syndrome are at risk for the development of brain tumors. In addition, children with neurofibromatosis type 1 often exhibit low tone (hypotonia). In this study, the authors explored the hypothesis that hypotonia could be a clinical indicator of glioma in children with neurofibromatosis type 1. A total of 56 children between 1 and 7 years of age with a confirmed diagnosis of neurofibromatosis type 1 were evaluated. Brain magnetic resonance imaging (MRI) was available for 19 of these children. Chi-square analysis demonstrated a statistically significant correlation between hypotonia and glioma in children with neurofibromatosis type 1 (90% sensitivity and 78% specificity). These results suggest that hypotonia might be a clinically useful indicator of brain tumor in this at-risk population.
患有1型神经纤维瘤病（NF1）这种遗传性肿瘤易感性综合征的儿童有患脑肿瘤的风险。此外，1型神经纤维瘤病患儿常表现出肌张力低下（肌张力减退）。在本研究中，作者探讨了肌张力低下可能是1型神经纤维瘤病患儿患胶质瘤的临床指标这一假说。对总共56名年龄在1至7岁之间确诊为1型神经纤维瘤病的儿童进行了评估。其中19名儿童进行了脑磁共振成像（MRI）检查。卡方分析表明，1型神经纤维瘤病患儿的肌张力低下与胶质瘤之间存在统计学上的显著相关性（敏感性为90%，特异性为78%）。这些结果表明，肌张力低下可能是这一高危人群中脑肿瘤的一个临床有用指标。
