Neurofibromatosis
See Also:
Neurofibromatosis:
Overview & Types
Neurofibromatosis:
Causes & Risk Factors
Neurofibromatosis:
Signs & Symptoms
Neurofibromatosis:
Medical Tests & Diagnosis Methods
Neurofibromatosis:
Treatment Options
Introduction, Overview & Types:
| Neurofibromatoses
(plural) are a group of three genetically
distinct nervous related, non-contagious,
progressive disorders affecting people of all
races and ethnic groups, irrespective of sex,
whereby tumors develop in cells that make up the
myelin sheath. (A myelin sheath envelops and
protects nerve fibers.) “The most
common tumors are neurofibromas, which develop in
the tissue surrounding peripheral nerves.”
Generally, these tumors are not malignant and may
turn cancerous over a period of time (1) &
(2).
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The exact causes for occurrence of these tumors
are unknown but gene mutations to the genes NF1 and NF2
are supposed to suppress production of specific proteins
that control cell production leading to uncontrolled
multiplication of cells and thus form tumors (1).
Types of Neurofibromatoses
Neurofibromatoses are categorized into the following
three types:
Neurofibromatosis 1 (NF1):
Neurofibromatosis 1 is an autosomal dominant genetic
disorder (i.e., 50% of the cases inherited from the
parent). This type is characterized by multiple
café-au-lait spots (six or more flat, pigmented spots
called [by the French term for coffee (café) with milk
(lait) due to their light tan color] and neurofibromas on
or under the skin. Characterists of NF1 include
enlargement and deformation of bones and curvature of the
spine (scoliosis) may also occur. Tumors may develop in
the brain, on cranial nerves and/or on the spinal cord.
This is a more common form of NF occurring in 1:3,000
births globally (3), (4) & (5).
Neurofibromatosis 1 is also called as Peripheral
Neurofibromatosis, Recklinghausen Disease and von
Recklinghausen Disease (7).
Neurofibromatosis 2 (NF2):
NF2 is also an autosomal dominant genetic disorder
(inherited) and is characterized by multiple tumors on
cranial and spinal nerves. Usually, tumors affect both
the auditory nerves with loss of hearing as the first
symptom. NF2 affects 1 in 25,000 people (3), (4) &
(5).
Neurofibromatosis 2 is also called as Bilateral
Acoustic Neurofibromatosis, Vestibular Schwannomas,
Central Neurofibromatosis, Familial Acoustic Neuromas and
Neurofibromatosis Type II (8).
Schwannomatosis:
Multiple benign tumors (i.e., Schwannomas) form in
peripheral nerve fiber cells (called Schwann cells).
Chronic pain is a hallmark in this type of NF but no
neurological disabilities or malignant tumors occur. Pain
may occur in any part of the body depending on peripheral
nerves that are affected. Schwannomatosis is a rare form
of NF affecting 1 in 40,000 people (3), (4) & (5).
See Also:
Neurofibromatosis:
Overview & Types
Neurofibromatosis:
Causes & Risk Factors
Neurofibromatosis:
Signs & Symptoms
Neurofibromatosis:
Medical Tests & Diagnosis Methods
Neurofibromatosis:
Treatment Options
Article by Kona Vishnu, MS
Medical Writer,
OmniMedicalSearch.com
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