Hypotonia and Neurofibromatosis Type 1 (NF1) Glioma

The Washington University

Status: Terminated

Conditions: Neurofibromatosis Type 1

Treatments: Device: Magnetic resonance imaging; Drug: Gadolinium contrast

Study type: Interventional

Funder types: Other

Identifiers

NCT02584413

201303074

Details and patient eligibility

About

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.

Enrollment: 29 patients

Sex: All

Ages: 1 to 7 years old

Volunteers: No Healthy Volunteers

Inclusion criteria

• Patient must be seen at the St. Louis Children's Hospital NF Clinic
• Diagnosis of NF1
• Between 1 and 7 years of age, inclusive
• Diagnosed with hypotonia
• Legally authorized representative/guardian must be able to understand and willing to sign an IRB-approved informed consent document
• Must have an MRI scan ordered by a treating physician

Exclusion criteria

• Normal tone on clinical exam
• Known allergy to gadolinium or the sedative, propofol, used during MRI
• Poor kidney function defined as a known renal disease or elevated BUN and creatine
• Requiring intubation for anesthesia

Trial design

Primary purpose: Screening

Allocation: N/A

Interventional model: Single Group Assignment

Masking: None (Open label)

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29 participants in 1 patient group

Arm 1: MRI of brain with gadolinium contrast

Experimental group

Description:

-Eligible children whose guardians have consented to their participation will undergo routine clinical brain MRI with gadolinium contrast. The MRI scan will last no more than 45 minutes

Treatment:

Drug: Gadolinium contrast

Device: Magnetic resonance imaging