The main feature of NF2 is bilateral acoustic neuromas
(vestibular schwannomas). Spinal tumours and intracranial
meningiomas occur in over 40% of cases. Surgical removal of
VIIIth nerve tumours is difficult and prognosis for this disorder
is often poor. The NF2 gene on chromosome 22 has been
cloned and various mutations, deletions and translocations
have been identified, allowing presymptomatic screening and
prenatal diagnosis within affected families.
Saturday, April 11, 2009
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