神经纤维瘤病1型（Neurofibromatosis type 1,NF1）也称Von Recklinghausen病,是一种很常见的常染色体显性遗传病[1],起源于神经上皮组织,主要累及皮肤、周围神经和中枢神经系统,而发生在腹膜后及盆腔的报道少见。本文就CT发现1例弥漫...神经纤维瘤病1型（Neurofibromatosis type 1,NF1）也称Von Recklinghausen病,是一种很常见的常染色体显性遗传病[1],起源于神经上皮组织,主要累及皮肤、周围神经和中枢神经系统,而发生在腹膜后及盆腔的报道少见。本文就CT发现1例弥漫性腹膜后及盆腔巨大神经纤维瘤病1型报道如下,并结合文献复习,以提高对神经纤维瘤病1型的认识。展开更多
Objective:To determine the prevalence of optic nerve sheath meningiomas(ONSMs)in patients with neurofibromatosis type 2(NF2).Methods:An observational retrospective case series of 30 consecutive patients with NF2 refer...Objective:To determine the prevalence of optic nerve sheath meningiomas(ONSMs)in patients with neurofibromatosis type 2(NF2).Methods:An observational retrospective case series of 30 consecutive patients with NF2 referred to an academic ophthalmology unit from November 1,1991,through August 31,2003.Twenty-six patients were followed up for a mean of 93 months(range,3-150 months).One individual was lost to follow-up,and 3 had been referred recently.Diagnosis of ONSM was made based on typical neuroradiologic and clinical features in 7 patients and on histologic criteria in 1.Results:Eight of 30 patients harbored unilateral(n=6)or bilateral(n=2)-ONSMs.Six ONSMs were diagnosed at initial examination,and 4 during follow-up.Conclusions:There is a strong association between ONSMs and NF2 that parallels the well-known association of optic nerve gliomas with NF1.Physicians should be aware of the possibility that patients with ONSMs may also have NF2.展开更多
文摘神经纤维瘤病1型（Neurofibromatosis type 1,NF1）也称Von Recklinghausen病,是一种很常见的常染色体显性遗传病[1],起源于神经上皮组织,主要累及皮肤、周围神经和中枢神经系统,而发生在腹膜后及盆腔的报道少见。本文就CT发现1例弥漫性腹膜后及盆腔巨大神经纤维瘤病1型报道如下,并结合文献复习,以提高对神经纤维瘤病1型的认识。
文摘Objective:To determine the prevalence of optic nerve sheath meningiomas(ONSMs)in patients with neurofibromatosis type 2(NF2).Methods:An observational retrospective case series of 30 consecutive patients with NF2 referred to an academic ophthalmology unit from November 1,1991,through August 31,2003.Twenty-six patients were followed up for a mean of 93 months(range,3-150 months).One individual was lost to follow-up,and 3 had been referred recently.Diagnosis of ONSM was made based on typical neuroradiologic and clinical features in 7 patients and on histologic criteria in 1.Results:Eight of 30 patients harbored unilateral(n=6)or bilateral(n=2)-ONSMs.Six ONSMs were diagnosed at initial examination,and 4 during follow-up.Conclusions:There is a strong association between ONSMs and NF2 that parallels the well-known association of optic nerve gliomas with NF1.Physicians should be aware of the possibility that patients with ONSMs may also have NF2.
