Neurofibromatosis encompasses a set of distinct genetic disorders that causes tumors to grow along various types of nerves. NF can also affect the development of non-nervous tissues such as bones and skin. Neurofibromatosis causes tumors to grow anywhere on or in your body.
Types Of Neurofibromatosis
Neurofibromatosis (NF) has been classified into three distinct types: NF1, NF2 and Schwannomatosis.
Neurofibromatosis 1 (NF1): also known as von Recklinghausen NF or Peripheral NF. Occurring in 1:2,500 births, web characterized by multiple cafe-au-lait spots and neurofibromas on or under the skin. NF1 is the most common neurological disorder caused by a single gene. Enlargement and deformation of bones and curvature of the spine (scoliosis) may also occur. Occasionally, tumors may develop in the brain, on cranial nerves, or on the spinal cord. About 50% of people with NF also have learning disabilities.
Neurofibromatosis 2 (NF2): also known as Bilateral Acoustic NF (BAN), is much rarer occurring in 1:25,000 births. NF2 is characterized by multiple tumors on the cranial and spinal nerves, and by other lesions of the brain and spinal cord. Tumors affecting both of the auditory nerves are the hallmark. Hearing loss beginning in the teens or early twenties is generally the first symptom.
Schwannomatosis: a rare form of NF that has only recently been recognized and appears to affect around 1:40,000 individuals. It is less well understood than NF1 and NF2, and features may vary greatly between patients.
Unlike NF1 and NF2, the inheritance patterns of Schwannomatosis are not understood due largely to lack of cases to study. However it is estimated that 85% of cases are in fact sporadic (no previous family history) and 15% are inherited.
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