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...

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Bibliographic Details
Published in:Journal of child neurology 2013-12, Vol.28 (12), p.1664-1667
Main Authors: Wessel, Lauren E., Albers, Anne C., Gutmann, David H., Dunn, Courtney M.
Format: Article
Language:English
Subjects:
Brain Neoplasms - complications
Child
Child, Preschool
Female
Glioma - complications
Humans
Infant
Magnetic Resonance Imaging
Male
Muscle Hypotonia - complications
Neurofibromatosis 1 - complications
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Summary: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.
ISSN:0883-0738
1708-8283
DOI:10.1177/0883073812460918