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Thursday, March 22, 2018

Neurofibroma - neurofibromatosis (NF) - Von Recklinghausen's disease

 neurofibroma homeopathy treatment in chennai

Neurofibroma or neurofibromatosis (NF) or Von Recklinghausen's disease

A neurofibroma or neurofibromatosis or Von Recklinghausen's Disease is a benign nerve sheath tumor in the peripheral nervous system. Usually found in individuals with neurofibromatosis type 1 (NF1), a genetically-inherited disease.

It is a genetic disease in which patients develop multiple soft tumors (neurofibromas). These tumors occur under the skin and throughout the nervous system.

In this disease, a genetic defect causes these neural crest cells (neural crest cells are primitive cells which exist during fetal development) to develop abnormally. This results in numerous tumors and malformations of the nerves, bones, and skin.

Neurofibromatosis occurs in about one of every 4,000 births. Two types of NF exist, NF-1 (90% of all cases), and NF-2 (10% of all cases).

Both forms of neurofibroma or neurofibromatosis are caused by a defective gene. NF-1 is due to a defect on chromosome 17; NF-2 results from a defect on chromosome 22. Both of these disorders are inherited in a dominant fashion. This means that anybody who receives just one defective gene will have the disease.

It is interesting to note that only about half of all cases show a family history of the disease. The other cases occur because a specific gene spontaneously changes its structure (mutates). A person with this type of spontaneous mutation can then pass that gene onto his or her offspring. There is a 50% chance that a parent with neurofibroma or neurofibromatosis will pass the gene on to a child.

Symptoms & diagnosis
To be given the diagnosis of neurofibromatosis type 1 (NF1), an individual must have at least two of the following features. Some people with NF1 have only two, while others can have several of these features:

Ø  Six or more cafe-au-lait spots, or coffee-colored birthmarks, each measuring over an inch in adults (1/4 inch in children).
Ø  Two of more benign skin tumors called neurofibromas or one diffuse tumor of the soft tissue called a plexiform neurofibroma.
Ø  Freckles under the arm or in the groin region.
Ø  A tumor of the nerve to the eye called an optic glioma.
Ø  Two or more spots on the iris of the eye called Lisch nodules.
Ø  A problem of one of the bones such as bowing of a leg, with or without a fracture.
Ø  A parent, brother, sister, or child with NF1.

Individuals with (NF2) have fewer outward signs of the condition than individuals with NF1. To be given the diagnosis of NF2, an individual must have the following features:

Benign tumors on both sides of the head involving the nerves for hearing and balance, called acoustic neuromas or vestibular schwannomas
Family history of NF2 (a first-degree relative, i.e. mother, father, brother, sister, or child, with NF2) AND one of the following:

An acoustic neuroma or vestibular schwannoma on one side of the head before the age of 30,
Any two of the following : meningioma, glioma, schwannoma, juvenile posterior subcapsular lenticular opacities / juvenile cortical cataract.

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Disclaimer: These articles is for information only and should not be used for the diagnosis or treatment of medical conditions. we used all reasonable care in compiling the information but make no warranty as to its accuracy. Consult a doctor or other health care professional for diagnosis and treatment of medical conditions.