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Korean Journal of Otorhinolaryngology-Head and Neck Surgery > Volume 39(6); 1996 > Article
Korean Journal of Otorhinolaryngology-Head and Neck Surgery 1996;39(6): 1031-9.
Type 2 Neurofibromatosis : A Case Report
Nam Pyo Hong, MD1, Sang Seung Shin, MD1, Min Song, MD1, Chang Il Cha, MD1, and Won Suk Choi, MD2
1;Department of Otolaryngology, 2;Diagnostic Radiology, College of Medicine, Kyung-Hee University, Seoul, Korea
제2형 다발성 신경 섬유종증 증례
홍남표1 · 차창일1 · 신상승1 · 송 민1 · 최우석2
경희대학교 의과대학 이비인후과학교실1;진단방사선과학교실2;
ABSTRACT

Type 2 Neurofibromatosis(NF2) is one of the neurocutaneous syndromes and it had been formaly 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 diagnostic 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 or 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 a 31 years old female who had noticed a bilateral vestibular schwannomas, multiple intra cranial meningioma, bilateral trigeminal neurinomas, large spinal schwannoma in sacrum and mutiple small spinal schwannomas, and multiple cutaneous nodules.

Keywords: Bilateral vestibular schwannomasType 2 neurofibromatosis.
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