1. Neurofibromatosis
The neurofibromatoses (NF) are genetic disorders of the nervous system.
distinct forms are known: NF1 and NF2. One baby in every four thousand is born in the .
United States with NF1. It is one of the most common genetic disorders, affecting some .
one hundred thousand americans. NF2 is less common and occurs in only one and every .
forty thousand births. In both forms of NF severity of symptoms vary greatly. .
The effects can be severely disabling, mildly disfiguring or can even go undetected.
Several other names for this disorder have been used in the past but recent advancements .
in understanding the disorder made some of those terms obsolete. .
NF1 was previously known as peripheral neurofibromatosis, or von Recklinghouasen's .
disease after the doctor who first described it in 1882. NF2 was called bilateral acoustic .
nuerofibromatosis, central neurofibromatosis, or vestibular schwannoma. .
NF is found in every racial and ethnic group throughout the world and .
affects both sexes equally. Both neurofibromatosis1 and neurofibromitosis2 are caused.
by abnormal genes. This disease cannot be "caught" from someone who has it already. .
A common early sign of NF1 is six or more tan spots on the skin. They are .
called "cafe-au-lait" (French for "coffee with milk") spots. These spots are .
often present at birth, and may increase in size and number with age. They .
also may become darker. More than half of affected children have some signs .
of NF1 by 2 years of age. .
Benign tumors (lumps), under the skin or deeper, may appear at any age but .
especially during adolescence. The tumors, which grow on nerves, are made .
up of cells that surround nerves and other cell types, and are called .
neurofibromas. An affected person may have any number of neurofibromas, .
from none to hundreds. The tumors, which vary in size, may or may not be .
painful. A person also may have a single neurofibroma without having NF.
Some tumors may grow on the optic (eye) nerves and in rare cases may .
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