Neurofibromatosis type 1 natural history, complications and prognosis
Neurofibromatosis type 1 Microchapters |
Differentiating Neurofibromatosis type 1 from other Diseases |
---|
Diagnosis |
Treatment |
Case Studies |
Neurofibromatosis type 1 natural history, complications and prognosis On the Web |
American Roentgen Ray Society Images of Neurofibromatosis type 1 natural history, complications and prognosis |
FDA on Neurofibromatosis type 1 natural history, complications and prognosis |
CDC on Neurofibromatosis type 1 natural history, complications and prognosis |
Neurofibromatosis type 1 natural history, complications and prognosis in the news |
Blogs on Neurofibromatosis type 1 natural history, complications and prognosis |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Moises Romo M.D.
Overview
If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
OR
Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
OR
Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.
Natural History, Complications, and Prognosis
Natural History
- The symptoms of (disease name) usually develop in the first/ second/ third decade of life, and start with symptoms such as ___.
- The symptoms of (disease name) typically develop ___ years after exposure to ___.
- If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
Complications
- Common complications of [disease name] include:
- [Complication 1]
- [Complication 2]
- [Complication 3]
Prognosis
- Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [--]%.
- Depending on the extent of the [tumor/disease progression] at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as poor/good/excellent.
- The presence of [characteristic of disease] is associated with a particularly [good/poor] prognosis among patients with [disease/malignancy].
- [Subtype of disease/malignancy] is associated with the most favorable prognosis.
- The prognosis varies with the [characteristic] of tumor; [subtype of disease/malignancy] have the most favorable prognosis.
There is wide variability in how different individuals with the NF-1 gene manifest the disorder. Some individuals may have no symptoms, while others may have rapidly progressive disorder.
The primary problem of NF-1 is the disfigurement due to the cutaneous neurofibromas, pigmented lesions, and occasional limb abnormalities.
Several more severe complications of NF-1 are listed in the following section.
- Chronic pain, numbness, Pritchetts face, and/or paralysis due to the peripheral nerve sheath tumors
- Blindness due to optic nerve gliomas
- Brain tumors
- Neurologic impairment due to severe spinal scoliosis and/or kyphosis
- Amputation due to a tibial pseudarthrosis
- Malignant degeneration of a plexiform neurofibroma into malignant periphreal nerve sheath tumor (MPNST), occurring in 10-12%
- Depression, It is very common of NF sufferers to suffer severe depression because of the disfigurement it can cause to the body and face.
- Social Anxiety is also common among NF sufferers because of the reaction of others to the condition.
The life expectancy of individuals with neurofibromatosis type 1 is reduced by ~8–21 years,[1] and an excess of deaths occurs in younger individuals (<40 years of age), compared with the general population; the most common cause of early death is malignant neoplasm,,[1][2][3][4][5][6] Individuals have an increased risk for malignant and non-malignant conditions compared with the general population (BOX 1).