摘要
目的探讨2型神经纤维瘤病（NF2）的临床特征。方法回顾性分析69例NF2的临床特征。结果本组15例有家族史，平均发病年龄为27．3岁，发病年龄无性别差异，67例（97％）有听神经瘤，37例（54％）伴发脑膜瘤，31例（45％）伴发椎管肿瘤，33例（48％）以听神经瘤症状起病，13例（19％）以椎管病变起病。57例病人在我院行78次手术，1例死亡（死亡率1．4％）。随访到40例，KPS60分以上21例，7例病人死亡。结论NF2往往累及全身多个系统，需要由多学科协作进行个性化治疗。
Objective To study the clinical characteristics of Neurofibromatosis 2. Methods We evaluated 69 affected patients from Jan 2001 to Aug 2008 in the department of neurosurgery in Beijing Tiantan Hospital. Results The mean age at onset in 69 cases was 27. 3. There was no statistical difference between male and female. The vestibular schwannoma was documented in 97% individuals. The other findings included meningiomas （54%） and spinal tumors （45%）. The initial symptoms resulted from Vestibular schwannomas（48% ） and spinal tumors（ 19% ）. 78 operations were performed for 57 cases. The mortality was 1.4%. 40 cases were followed, and the KPS of 21 patients were above 60. 7 cases died. Conclusions Patients with NF2 always have diseases involved multiple systems, and individualized treatment strategy needs the cooperation of muhidisciplinary.
出处
《中华神经外科杂志》
CSCD
北大核心
2009年第4期330-332,共3页
Chinese Journal of Neurosurgery
基金
首都医学发展科研基金（2007-2064）.
关键词
2型神经纤维瘤病
临床特征
听神经瘤
Neurofibromatosis 2
Clinical characteristics
Vestibular schwannoma
