All::Neurology::Diseases::Neurofibromatosis

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Intro

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What is Neurofibromatosis?

a group of conditions in which tumors grow in the nervous system

Neurofibromatosis

NF1

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How common is Neurofibromatosis type I?

affects around 1 in 4,000

Neurofibromatosis

What are the main clinical features of Neurofibromatosis type I?

  • Café-au-lait spots (>= 6, 15 mm in diameter)
  • Axillary/groin freckles
  • Peripheral neurofibromas
  • Iris hamatomas (Lisch nodules) in > 90%
  • Scoliosis
  • Pheochromocytomas

Neurofibromatosis

What is the cause of Neurofibromatosis type I?

a gene mutation on chromosome 17 which encodes neurofibromin

Neurofibromatosis

NF2

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How common is Neurofibromatosis type II?

1 in 100,000

Neurofibromatosis

What are the main clinical features of Neurofibromatosis type II?

  • Bilateral vestibular schwannomas
  • Multiple intracranial schwannomas, mengiomas and ependymomas

Neurofibromatosis

What are the main causes of Neurofibromatosis type II?

gene mutation on chromosome 22

Neurofibromatosis