For a diagnosis of NF-1, at least 2 of the diagnostic criteria should be present3
NF-1 Diagnostic Criteria | Patient 1 | Patient 2 | Patient 3 | Patient 4 | Patient 5 |
---|---|---|---|---|---|
Six or more café au lait macules >5 mm in greatest diameter in prepubertal individuals and >15 mm in greatest diameter in postpubertal individuals | Yes | Yes | Yes | Yes | Yes |
Two or more neurofibromas of any type or 1 plexiform neurofibroma | Yes | ||||
Freckling in the axillary or inguinal regions | Yes | Yes | Yes | Yes | Yes |
Optic glioma | Yes | Yes | |||
Two or more Lisch nodules (iris hamartomas) | Yes | Yes | Yes | ||
A distinctive osseous lesion such as sphenoid dysplasia or tibial pseudarthrosis | |||||
A first-degree relative (parent, sibling, or offspring) with NF-1 as defined by the above criteria | Yes | Yes | Unknown (adopted) | ||
MRI findings: focal abnormal signal intensities | Yes | Yes | Yes | Yes | Yes |