Diagnostic criteria for neurofibromatosis type 1

At least two major criteria makes the diagnosis of neurofibromatosis type 1

Major criteria

  • cafe au lait spots
  • freckling in inguinal or axillary areas
  • one plexiform neurofibroma or two neurofibromas of any type
  • visual pathway glioma
  • two or more Lisch nodules of iris
  • distinctive osseous lesion (sphenoid dysplasia or thinning of cortex)
  • first-degree relative with neurofibromatosis-1

Minor criteria

  • small stature
  • macrocephaly
  • scoliosis
  • pectus excavatus
  • hamartomatous lesion of NF-1
  • neuropsychological abnormalities