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1例伴基底脑膜膨出及异常血管的Ⅰ型神经纤维瘤病

[A case of neurofibromatosis type I associated with basal meningocele and abnormal vessels].

作者信息

Yoshioka H, Sakoda K, Kohno H, Hada H, Hanaya R, Arita K, Kurisu K

机构信息

Department of Neurosurgery, Mazda Motor Corporation, Mazda Hospital.

出版信息

No To Shinkei. 1998 Mar;50(3):279-83.

PMID:9566005
Abstract

A 21-year-old man with neurofibromatosis type 1 (NF 1) had many widespread cutaneous neurofibroma on his right face. Magnetic resonance imaging (MRI) revealed basal meningocele due to dysplasia of the skull base. Carotid and vertebral angiograms revealed occlusion of the right internal carotid artery, persistent primitive trigeminal artery. We have reviewed the clinical and radiographic features of this case of neurofibromatosis, meningocele and cerebral arterial abnormalities. NF associated with both intracranial vascular malformation and meningocele is very rare, and in our case both were thought to arise congenitally as a manifestation of mesodermal dysplasia. Careful follow up using MRI and MR angiography should be performed for such patients.

摘要

一名患有1型神经纤维瘤病(NF 1)的21岁男性,其右脸有许多广泛分布的皮肤神经纤维瘤。磁共振成像(MRI)显示由于颅底发育异常导致基底脑膜膨出。颈动脉和椎动脉血管造影显示右颈内动脉闭塞、永存原始三叉动脉。我们回顾了该例神经纤维瘤病、脑膜膨出和脑动脉异常病例的临床及影像学特征。与颅内血管畸形和脑膜膨出相关的NF非常罕见,在我们的病例中,两者被认为均先天性发生,是中胚层发育异常的表现。对于此类患者,应使用MRI和磁共振血管造影进行仔细的随访。

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