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Neurofibromatosis



             interfere with vision. Tiny tan or brown "Lisch nodules" (small tumors) often .
             appear on the iris (colored part of the eye), but these cause no trouble. They .
             usually show up around age 6 to 10 years and may help a doctor diagnose .
             NF1. NF2 is characterized by tumors that grow on what is referred to as the .
             8th cranial nerve (one of 12 pairs of nerves that enter or emerge from the brain). These .
             tumors often cause pressure on the acoustic (hearing) nerves, resulting in hearing loss. .
             Persons with NF2 also may develop other tumors of the brain or spinal cord, and .
             sometimes develop cataracts at an early age. Persons with NF2 have few, if any, cafe-au-.
             lait spots or tumors under the skin. Symptoms of NF2 may not begin until after puberty.
             NF1 and NF2 are caused by two separate abnormal genes. The abnormal gene in each .
             form of NF can either be inherited from one parent who has the disorder or can result .
             from a new mutation (change) in the gene. Thus, NF can occur in a person who has no .
             family history of the condition. About 50 percent of NF1 and NF2 cases are caused by .
             new mutations. An individual with NF caused by a new mutation can have the same .
             variety of signs and symptoms as a person who inherited it from an affected parent.
             Inheritance of the gene is "autosomal dominant", so that the child of a parent with NF has .
             a 50-50 chance of inheriting the NF gene and showing at least some signs of .
             neurofibromatosis sooner or later. The child also has a 50-50 chance of inheriting the NF .
             gene's normal partner (since genes come in pairs) and remaining free of NF.
             In most cases of NF1, symptoms are mild and affected individuals live a normal life. .
             Some individuals have many skin neurofibromas (lumps on the nerves) on the face and .
             body. Neurofibromas also can grow inside the body. Neurofibromas may increase during .
             puberty and pregnancy because of hormonal changes. Scoliosis (a progressive curvature .
             of the spine) is common in NF and can begin at an early age.


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