Type 2 Neurofibromatosis(NF2) is one of the neurocutaneous syndromes and it had been formally separated from the more common type 1 neurofibromatosis(von-Recklinghausen's disease), after the National Institute of Health(NIH) Consensus Development
Conference Statement of on neurofibromatosis in 1987.
The diagnositc criteria for type 2 neurofibromatosis(NF2) are met if a person has(1) bilateral eighth nerve masses seen with computed tomographic scanning or magnetic resonance imaging (MRI), or (2) a first-degree relative(parent or sibling) with
type 2
neurofibromatosis(NF2) and either unilateral eighth nerve masses of one of the following : neurofibroma, meningioma, glioma, schwannoma. or juvenile posterior subcapsular lenticular opacity.
We have recently experienced a case of typical type 2 neurofibromatosis(NF2). The patient was 1a 31 years old female who had noticed a bilateral vestibular schwannomas, multiple intra cranial meningioma, bilateral trigeminal neurinomas, large
spinal
schwannoma in sacrum and multiple small spinal schwannomas, and Inultiple cutaneous nodules. (Korean J Otolaryngo1 39 : 6, 1996)
|