Clinical Trial: Hypotonia and Neurofibromatosis Type 1 (NF1) Glioma

Study Status: Recruiting
Recruit Status: Recruiting
Study Type: Observational

Official Title: Hypotonia as a Clinical Predictor of Optic Pathway Glioma in Children With Neurofibromatosis Type 1

Brief Summary: Currently, optic pathway gliomas (OPG) are detected based on abnormal findings made during annual ophthalmologic exams. However, because these exams are annual, it is possible for healthcare providers to miss the point at which a child's vision begins to decline (potentially indicating an OPG). If at-risk children are screened for hypotonia early in life, those children who are hypotonic may undergo magnetic resonance imaging (MRI) to evaluate for OPG before they are showing ophthalmologic symptoms. This would enable healthcare providers to discover vision loss earlier and treat symptomatic OPGs earlier, thereby allowing us a better chance of preventing further vision loss in children with OPGs.

Detailed Summary:
Sponsor: Washington University School of Medicine

Current Primary Outcome: Predictive accuracy of clinical diagnosis of hypotonia as an indicator of OPG in children with NF1 [ Time Frame: At the time of MRI (1 day) ]

  • A pediatric nurse practitioner (PNP) or a pediatric physician specializing in NF1, and physical therapist will screen the children for hypotonia.
  • The MRI scan will show hypotonia if the children have thickening or enlargement of any portion of the optic nerve, optic chiasm, or optic tracts.
  • The data analysis for this will be descriptive in nature.


Original Primary Outcome: Same as current

Current Secondary Outcome:

  • Other features that may be indicatory of OPG in children with NF1 [ Time Frame: At the time of MRI (1 day) ]
    • MRI findings may include other brain tumors or T2 hyperintensities.
    • The data analysis will be descriptive in nature.
  • Determine if a physical therapist (PT) can train another clinical professional to accurately diagnose hypotonia [ Time Frame: 1 day ]
    -PTs use subjective muscle tone, a pull-to-sit test, and the presence or absence of head lag to determine hypotonia.


Original Secondary Outcome: Same as current

Information By: Washington University School of Medicine

Dates:
Date Received: October 20, 2015
Date Started: April 16, 2013
Date Completion: April 30, 2023
Last Updated: March 16, 2017
Last Verified: March 2017