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神经轴索钙化性假肿瘤(CaPNoN):青少年动眼神经麻痹的罕见病因。

Calcifying pseudoneoplasm of the neuraxis (CaPNoN): an unusual cause of third nerve palsy in a teenager.

作者信息

Ghaemi Joseph, Wasimi Mohammed, Siripurapu Rekha, McKee David, Pal Piyali, du Plessis Daniel, Rutherford Scott, Laitt Roger, Potter Gillian

机构信息

Department of Neuroradiology, Greater Manchester Neurosciences Centre, Salford Royal NHS Foundation Trust, UK.

Department of Neurology, Greater Manchester Neurosciences Centre, Salford Royal NHS Foundation Trust, UK.

出版信息

BJR Case Rep. 2016 Jul 28;2(3):20150494. doi: 10.1259/bjrcr.20150494. eCollection 2016.

Abstract

An 18-year-old part-time teacher presented with headache and diplopia. Physical examination showed partial left oculomotor palsy. Neurology examination was otherwise unremarkable. Cross-sectional imaging was arranged for investigation of third nerve palsy. On CT scan, the lesion was calcified, and on MRI, hypointense on and weightedimages with thin rim enhancement, resembling an atypical meningioma. CT angiogram showed no vascular connection. Following worsening diplopia and a slight increase in lesion size on follow-up MRI, the patient was re-reviewed in our regional skull base multidisciplinary team meeting, where a decision for excision was made. Pre-operatively, the absence of a vascular connection was confirmed on catheter angiogram. Histopathological examination demonstrated features typical of calcified pseudoneoplasm of the neuraxis, with extensive metaplastic calcification with stroma containing variable fibrovascular tissue and focal inflammatory cell infiltrates, spindle and epithelioid cells, and psammoma bodies at the rim of the lesion. Following surgery, the patient had persisting diplopia. He remains under clinical review. As surgical resection is considered curative, no further imaging follow-up is planned.

摘要

一名18岁的兼职教师出现头痛和复视症状。体格检查发现左侧动眼神经部分麻痹。神经系统检查其他方面无异常。安排了横断面成像以调查动眼神经麻痹。CT扫描显示病变钙化,MRI上,T1加权像和T2加权像呈低信号,有薄环状强化,类似非典型脑膜瘤。CT血管造影显示无血管连接。在随访MRI上复视加重且病变大小略有增加后,患者在我们地区颅底多学科团队会议上接受了再次评估,会上做出了切除的决定。术前,导管血管造影证实无血管连接。组织病理学检查显示为神经轴钙化假瘤的典型特征,有广泛的化生钙化,间质含有可变的纤维血管组织和局灶性炎性细胞浸润、梭形细胞和上皮样细胞,病变边缘有砂粒体。手术后,患者仍有持续性复视。他仍在接受临床复查。由于手术切除被认为是治愈性的,因此未计划进一步的影像学随访。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d718/6243365/6d1bf28a5325/bjrcr.20150494.g001.jpg

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