CAJ | 학술논문

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,KtX560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.
목적 탐토2형신경섬유류병(NF2)적림상특정.방법 회고성분석69례NF2적림상특정.결과 본조15례유가족사,평균발병년령위27.3세.발병년령무성별차이,67례(97%)유은신경류,37례(54%)반발뇌막류,31례(45%)반발추관종류,33례(48%)이은신경류증상기병.13례(19%)이추관병변기병.57례병인재아원행78차수술,1례사망(사망솔1.4%).수방도40례,KtX560분이상21례,7례병인사망.결론 NF2왕왕루급전신다개계통,수요유다학과협작진행개성화치료.
Objective To study the clinical characteristics of Neurofibromatosis 2.Methods Weevaluated 69 affected patients from Jan 2001 to Aug 2008 in the department of neuroburgery in Beijing Tiantan Hospital. Results The merit age at onset in 69 cases wag 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 symproms resulted from Vestibular sehwannomas(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.