Neurofibromatosis
Neurofibromatosis
Neurofibromatosis (commonly abbreviated NF) is genetically-inherited disorder in which the nerve tissue grows tumors that may be harmless or may cause serious damage by compressing nerves and other tissues.
Neurofibromatosis Type 1 (85%): 2 or more meets diagnositic criteria
· Two or more neurofibromas on the skin or under the skin or one plexiform neurofibroma (a large cluster of tumors involving multiple nerves).
· Freckling of the groin or the axilla.
· Six or more measuring 5mm in greatest diameter in prepubertal individuals and over 15mm in greatest diameter in post pubertal individuals.
· Skeletal abnormalities, such as sphenoid dysplasia or thinning of the cortex of the long bones of the body.
· Lisch nodules (hamartomas of iris), freckling in the iris.
· Optic glioma
· First degree relative (parent, sibling, or offspring) with NF1 by above criteria
Neurofibromatosis Type 2 (10%)
· Bilateral tumors, acoustic neuromas on the vestibulocochlear nerve (the eighth cranial nerve) leading to hearing loss
· In fact, the hallmark of NF2 is hearing loss due to acoustic neuromas around the age of twenty
· Tumors may cause:
1. Headache
2. Balance problems, and Vertigo
3. Facial weakness/paralysis
4. Patients with NF2 may also develop other brain tumors, as well as spinal tumors