The association between hypotonia and brain tumors in children with neurofibromatosis type 1.
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.
1Department of Neurology, Washington University School of Medicine, St. Louis, MO, USA.
Lauren E
LE
Wessel
Anne C
AC
Albers
David H
DH
Gutmann
Courtney M
CM
Dunn
D001932:Brain Neoplasms / Q000150:complications*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005910:Glioma / Q000150:complications*; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D009123:Muscle Hypotonia / Q000150:complications*; D009456:Neurofibromatosis 1 / Q000150:complications*