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.

Original languageEnglish
Pages (from-to)1664-1667
Number of pages4
JournalJournal of Child Neurology
Issue number12
StatePublished - Dec 2013


  • brain tumor
  • hypotonia
  • neurofibromatosis type 1
  • optic glioma


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